Professional Documents
Culture Documents
Cardiovascular Medicine
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1c p American College of Physicians®
Leading Internal Medicine, Improving Lives
Welcome to the Cardiovascular
Medicine Section of MKSAP 17!
In these pages, you will find updated information on risk assessment in cardiovascular disease, diagnostic testing, coronary
artery disease, heart failure, arrhythmias, pericardia! and myocardial disease, valvular heart disease, and other clinical chal
lenges. All of these topics are uniquely focused on the needs of generalists and subspecialists outside of cardiovascular medicine.
The publication of the 17th edition of Medical Knowledge Self-Assessment Program (MKSAP) represents nearly a half-century
of serving as the gold-standard resource for internal medicine education. It also marks its evolution into an innovative learn
ing system to better meet the changing educational needs and learning styles of all internists.
The core content of MKSAP has been developed as in previous editions-newly generated, essential information in 11 topic
areas of internal medicine created by dozens of leading generalists and subspecialists and guided by certification and recer
tification requirements, emerging knowledge in the field, and user feedback. MKSAP 17 also contains 1200 all-new, psycho
metrically validated, and peer-reviewed multiple-choice questions (MCQs) for self-assessment and study, including 120 in
Cardiovascular Medicine. MKSAP 17 continues to include High Value Care (HVC) recommendations, based on the concept
of balancing clinical benefit with costs and harms, with links to MCQs that illustrate these principles. In addition, HVC Key
Points are highlighted in the text. Also highlighted, with blue text, are Hospitalist-focused content and MCQs that directly
address the learning needs of internists who work in the hospital setting.
MKSAP 17 Digital provides access to additional tools allowing you to customize your learning experience, including regular
text updates with practice-changing, new information and 200 new self-assessment questions; a board-style pretest to help
direct your learning; and enhanced custom-quiz options. And, with MKSAP Complete, learners can access 1200 electronic
flashcards for quick review of important concepts or review the updated and enhanced version of Virtual Dx, an image-based
self-assessment tool.
As before, MKSAP 17 is optimized for use on your mobile devices, with iOS- and Android-based apps allowing you to sync
your work between your apps and online account and submit for CME credits and MOC points online.
Please visit us at the MKSAP Resource Site (rnksap.acponline.org) to find out how we can help you study, earn CME credit
and MOC points, and stay up to date.
Whether you prefer to use the traditional print version or take advantage of the features available through the digital version,
we hope you enjoy MKSAP 17 and that it meets and exceeds your personal learning needs.
On behalf of the many internists who have offered their time and expertise to create the content for MKSAP 17 and the editorial staff
who work to bring this material to you in the best possible way, we are honored that you have chosen to use MKSAP 17 and appreci
ate any feedback about the program you may have. Please feel free to send us any comments to mksap_editors@acponline.org.
Sincerely,
ii
Cardiovascular Medicine
iii
Jeffrey S. Berger, MD, MS
ACP Principal Staff
Research Grants/Contracts
Patrick C. Alguire, MD, FACP2
AstraZeneca, American Heart Association,
Senior Vice President, Medical Education
Doris Duke Charitable Foundation,
Sean McKinney1 National Institutes of Health
Vice President, Medical Education Consultantship
Bristol-Myers Squibb, Takeda Pharmaceuticals
Margaret Wells1
Other
Director, Self-Assessment and Educational Programs
Maintenance of Certification: Pri-Med; Planning
Becky Krumm1 Committee: American College of Cardiology
Managing Editor
W. Schuyler Jones, MD
Valerie A. Dangovetsky1 Other
Administrator American Physician Institute
Research Grants/Contracts
Ellen McDonald, PhD1
AstraZeneca, Bristol-Myers Squibb, American Heart
Senior Staff Editor
Association, Boston Scientific
Katie ldell1
Andrew M. Kates, MD
Digital Content Associate/Editor
Speakers Bureau
Megan Zborowski1 Pfizer, American College of Cardiology, MCE Medical
Senior Staff Editor
Jonathan P. Piccini, MD, MHS
Randy Hendrickson1 Consultantship
Production Administrator/Editor Medtronic, Forest Laboratories, Pfizer /Bristol-Myers Squibb,
Linnea Donnarumma1
Spectranetics, Johnson & Johnson
Staff Editor Research Grants/Contracts
Janssen Pharmaceuticals, GE Healthcare, Boston Scientific,
Susan Galeone1 ARCA Biopharma, ResMed
Staff Editor
Ileana L. Piiia, MD, MPH
Jackie 1\vomey1
Employment
Staff Editor
Montefiore-Einstein Medical Center
Kimberly Kems1 Research Grants/Contracts
Administrative Coordinator Duke University/National Institutes of Health
Royalties
iv
Andrew Wang, MD used to apply to the American Board of Internal Medicine
Research Grants/Contracts for Maintenance of Certification (MOC) points.
Abbott Vascular, Edwards Lifesciences, Gilead Sciences,
American Heart Association
Consultantship Royal College Maintenance
American College of Cardiology Foundation of Certification
Other
In Canada, MKSAP 17 is an Accredited Self-Assessment
Expert reviewer for legal case of infective endocarditis
Program (Section 3) as defined by the Maintenance of
Certification (MOC) Program of The Royal College of
Acknowledgments Physicians and Surgeons of Canada and approved by
the Canadian Society of Internal Medicine on December
The American College of Physicians (ACP) gratefully
9, 2014. Approval extends from July 31, 2015 until July
acknowledges the special contributions to the develop
31, 2018 for the Part A sections. Approval extends from
ment and production of the 17th edition of the Medical
December 31, 2015 to December 31, 2018 for the Part B
Knowledge Self-Assessment Program® (MKSAP® 17) made
sections.
by the following people:
Fellows of the Royal College may earn three credits per
Graphic Design: Michael Ripca (Graphics Technical
hour for participating in MKSAP 17 under Section 3.
Administrator) and WFGD Studio (Graphic Designers).
MKSAP 17 also meets multiple CanMEDS Roles, includ
Production/Systems: Dan Hoffmann (Director, Web ing that of Medical Expert, Communicator, Collaborator,
Services & Systems Development), Neil Kohl (Senior Manager, Health Advocate, Scholar, and Professional.
Architect), Chris Patterson (Senior Architect), and Scott For information on how to apply MKSAP 17 Continuing
Hurd (Manager, Web Projects & CMS Services). Medical Education (CME) credits to the Royal College
MOC Program, visit the MKSAP Resource Site at
MKSAP 17 Digital: Under the direction of Steven Spadt,
mksap.acponline.org.
V ice President, Digital Products & Services, the digital ver
sion of MKSAP 17 was developed within the ACP's Digital
Product Development Department, led by Brian Sweigard
(Director). Other members of the team included Dan The Royal Australasian College
Barron (Senior Web Application Developer/Architect), of Physicians CPD Program
Chris Forrest (Senior Software Developer/Design Lead), In Australia, MKSAP 17 is a Category 3 program that may
Kara Kronenwetter (Senior Web Developer), Brad Lord be used by Fellows of The Royal Australasian College
(Senior Web Application Developer), John McKnight of Physicians (RACP) to meet mandatory Continuing
(Senior Web Developer), and Nate Pershall (Senior Web Professional Development (CPD) points. Two CPD cred
Developer). its are awarded for each of the 200 AMA PRA Category 1
Credits"' available in MKSAP 17. More information about
The College also wishes to acknowledge that many other
using MKSAP 17 for this purpose is available at the MKSAP
persons, too numerous to mention, have contributed to
Resource Site at mksap.acponline.org and at www.racp.
the production of this program. Without their dedicated
edu.au. CPD credits earned through MKSAP 17 should be
efforts, this program would not have been possible.
reported at the MyCPD site at www.racp.edu.au/mycpd.
v
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Hospital-Based Medicine Publisher's Information
For the convenience of subscribers who provide care in Copyright© 2015 American College of Physicians. All
hospital settings, content that is specific to the hospital rights reserved.
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tronic or mechanical, including photocopy, without the
express consent of the American College of Physicians.
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MKSAP 17 is for individual use only. Only one account
Key Points in the text that relate to High Value Care
per subscription will be permitted for the purpose of
concepts (that is, concepts that discuss balancing clinical
earning Continuing Medical Education (CME) credits and
benefit with costs and harms) are designated by the
Maintenance of Certification (MOC) points/credits and for
HVC icon (HVC).
other authorized uses of MKSAP 17.
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persist in print. Drug dosage schedules are, we believe, ful.The American College of Physicians (ACP) prohibits
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Readers are advised, however, to ensure that the recom form either for individual use or for distribution.
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(Cardiovascular Medicine) ISBN: 978-1-938245-19-0
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vii
Table of Contents
ix
Giant Cell Myocarditis . . . . . . . . . . . . . . . . . . . . . . . . 41 Pericardia! Disease
Tachycardia-Mediated Cardiomyopathy . . . . . . . . . 42 Acute Pericarditis . . . . . . .. . . . . . . . . . . . . . . . . . . . . . . . 64
Clinical Presentation . . . . . . . . . . . . . . . . . . . . . . . . . 64
Myocardial Disease Evaluation . . . . . . . . . . . . . . .. . . . . . . . . . . . . . . . . . 64
Hypertrophic Cardiomyopathy . . . . . . . . . . . . . . . . . . . . 42 Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6S
Clinical Presentation and Evaluation . . . . . . . . . . . 42 Constrictive Pericarditis . . . . . . . . . . . . . . . . . . . . . . . . . . 66
Clinical Course and Risk Stratification . . . . . . . . . . 43 Clinical Presentation and Evaluation . . . . . . . . . . . 66
Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 44 Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 68
x
Ventricular Septal Defect . . . . . . . . . . . . . . . . . . . . . . . . . 89 Aortic Atheroma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 99
Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . 89 Abdominal Aortic Aneurysm . . . . . . . . . . . . . . . . . . . . . . 99
Clinical Presentation . . . . . . . . . . . . . . . . . . . . . . . . . 90 Screening and Surveillance . . . . . . . . . . . . . . . . . . . 99
Diagnostic Evaluation . . . . . . . . . . . . . . . . . . . . . . . . 90 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 100
Treatment. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 90
Follow-up After Ventricular Septal Peripheral Arterial Disease
Defect Closure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 90
Epidemiology and Screening . . . . . . . . . . . . . . . . . . . . . 100
Patent Ductus Arteriosus . . . . . . . . . . . . . . . . . . . . . . . . . 91
Evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 101
Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . 91
History and Physical Examination . . . . . . . . . . . . 101
Clinical Presentation . . . . . . . . . . . . . . . . . . . . . . . . . 91
Diagnostic Testing . . . . . . . . . . . . . . . . . . . . . . . . . 101
.
Diagnostic Evaluation . . . . . . . . . . . . . . . . . . . . . . . . 91
Medical Therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 102
Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 91
Cardiovascular Risk Reduction . . . . . . . . . . . . . . . 103
Pulmonary Valve Stenosis . . . . . . . . . . . . . . . . . . . . . . . . 91
Symptom Relief . . . . . . . . . . . . . . . . . . . . . . . . . . . . 103
Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . 91 Interventional Therapy . . . . . . . . . . . . . . . . . . . . . . . . . . 104
Clinical Presentation . . . . . . . . . . . . . . . . . . . . . . . . . 91 Acute Limb Ischemia . . . . . . . . . . . . . . . . . . . . . . . . . . . 104
Diagnostic Evaluation . . . . . . . . . . . . . . . . . . . . . . . . 91
Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 92
Cardiovascular Disease in Cancer Survivors
Aortic Coarctation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 92
Cardiotoxicity of Radiation Therapy
Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . 92
to the Thorax . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 105
Clinical Presentation . . . . . . . . . . . . . . . . . . . . . . . . . 92
Cardiotoxicity of Chemotherapy . . . . . . . . . . . . . . . . . . 106
Diagnostic Evaluation . . . . . . . . . . . . . . . . . . . . . . . . 92
Treatment. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 92
Follow-up After Aortic Coarctation Repair. . . . . . . 93 Pregnancy and Cardiovascular Disease
Tetralogy of Fallot . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 93 Cardiovascular Changes During Pregnancy . . . . . . . . . 108
Diagnostic Evaluation After Repair Prepregnancy Evaluation . . . . . . . . . . . . . . . . . . . . . . . . 108
of Tetralogy of Fallot . . . . . . . . . . . . . . . . . . . . . . . . . 93 Management of Cardiovascular Disease
Treatment of Tetralogy of Fallot Residua . . . . . . . . 94 During Pregnancy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 109
Adults with Cyanotic Congenital Heart Disease . . . . . . 94 Peripartum Cardiomyopathy . . . . . . . . . . . . . . . . . 110
General Management . . . . . . . . . . . . . . . . . . . . . . . . 94 Other Cardiovascular Disorders . . . . . . . . . . . . . . 110
Eisenmenger Syndrome . . . . . . . . . . . . . . . . . . . . . . 94 Cardiovascular Medication Use
During Pregnancy . . . . . . . . . . . . . . . . . . . . . . . . . . 110
xi
Cardiovascular Medicine High Value
Care Recommendations
The American College of Physicians, in collaboration with without other major risk factors such as hypertension or
multiple other organizations, is engaged in a worldwide tobacco use) (see Item 88).
initiative to promote the practice of High Value Care • Stress testing is most efficacious in patients with an
(HVC). The goals of the HVC initiative are to improve intermediate pretest probability of coronary artery dis
health care outcomes by providing care of proven benefit ease (10%-90%), because it is these patients who, by the
and reducing costs by avoiding unnecessary and even result of their stress test, can be reclassified into higher
harmful interventions. The initiative comprises several or lower risk categories.
programs that integrate the important concept of health • Exercise electrocardiographic testing is recommended
care value (balancing clinical benefit with costs and as the initial test of choice in patients who are able to
harms) for a given intervention into a broad range of edu exercise with a normal baseline electrocardiogram and
cational materials to address the needs of trainees, prac an intem1ediate pretest probability of coronary artery
ticing physicians, and patients. disease based on age, sex, and symptoms (see Item 87).
• Measurement of coronary artery calcium should be lim
HVC content has been integrated into MKSAP 17 in several
ited to a select group of asymptomatic patients with an
important ways. MKSAP 17 now includes HVC-identified
intermediate Framingham risk score (10%-20%) in whom
key points in the text, HVC-focused multiple choice
results will influence treatment strategy because of its
questions, and, for subscribers to MKSAP Digital, an HVC
associated cost and radiation exposure.
custom quiz. From the text and questions, we have gen
• Routine yearly imaging evaluation of structural heart dis
erated the following list of HVC recommendations that
ease in asymptomatic patients is usually not indicated.
meet the definition below of high value care and bring us
• Patients with grade 1 or 2 midsystolic murmurs who are
closer to our goal of improving patient outcomes while
asymptomatic with no associated findings and those with
conserving finite resources.
continuous murmurs suggestive of a venous hum or mam
High Value Care Recommendation: A recommendation to mary souffle do not warrant echocardiographic evaluation.
choose diagnostic and management strategies for patients • For stable angina pectoris, percutaneous coronary inter
in specific clinical situations that balance clinical benefit vention is reserved for patients with refractory symptoms
with cost and harms with the goal of improving patient while on optimal medical therapy, those who are unable
outcomes. to tolerate optimal medical therapy owing to side effects,
or those with high-risk features on noninvasive imaging.
Below are the High Value Care Recommendations for the
• Clinical practice guidelines do not recommend the rou
Cardiovascular Medicine section of MKSAP 17.
tine use of ECG monitoring, stress testing, or anatomic
• Current guidelines do not support the use of high-sensi testing (coronary CT angiography or invasive angiogra
tivity C-reactive protein (hsCRP) evaluation in the general phy) in asymptomatic patients after percutaneous coro
population, but hsCRP testing may be used in interme nary intervention or coronary artery bypass graft surgery.
diate-risk patients in whom choice of therapy may be • Routine stress testing is not currently recommended
affected by reclassification of risk. for asymptomatic patients following an acute coronary
• There is currently no role for the routine measurement of syndrome who are not entering a cardiac rehabilitation
Lp(a) lipoprotein levels, homocysteine levels, or evalua program.
tion of lipid particle size as these tests are expensive and • Evaluation of unusual causes of heart failure should not
no studies to date have shown that treatment targeted to be performed routinely but should be performed when
these levels affects outcomes. there are suggestions of specific diseases by history or
• The evaluation of subclinical disease with coronary physical examination findings (see Item 116).
artery calcium scoring may be appropriate to further risk • In patients with chronic heart failure who are clinically
stratify intermediate-risk patients but is not a component stable, annual or more frequent follow-up echocardiogra
of routine risk assessment. phy rarely provides therapeutic or diagnostic benefit and
• Aspirin should not routinely be given to patients with is not recommended.
diabetes mellitus who are at low cardiovascular risk (men • Patients hospitalized for heai1 failure who are scheduled
younger than 50 years and women younger than 60 years for a follow-up appointment within 1 week after discharge
xiii
have a reduced risk of future heart failure hospitalization • Screening of asymptomatic patients for abnormalities
(see Item 34). of the thoracic aorta should be reserved for patients
• Patients with asymptomatic, mild aortic stenosis or with underlying vascular pathology (such as Marfan or
regurgitation require echocardiography every 3 to Ehlers-Danlos syndrome), a bicuspid aortic valve, or a
5 years. family history of aortic disease.
• Asymptomatic patients with moderate aortic regurgi • Invasive imaging of the aorta by angiography is rarely
tation should be evaluated clinically on a yearly basis necessary for the diagnosis of acute disease; it should
and have echocardiography performed every 1 to be reserved for patients in whom a percutaneous inter
2 years, but they do not require medical or surgical vention is planned.
intervention (see Item 59). • Diuretics, particularly in high doses, will exacerbate
• Patients with asymptomatic, severe aortic stenosis with the propensity towards dynamic left ventricular out
preserved left ventricular function may be managed flow tract obstruction and, therefore, should be
with close clinical follow-up and echocardiography avoided in patients with hypertrophic cardiomyopathy
every 6 to 12 months and should not undergo valve (see Item 5).
replacement (see Item 20). • When an atrial septal aneurysm is identified inciden
• Patients with an asymptomatic bicuspid aortic valve tally, no further evaluation, medical treatment, or inter
should undergo surveillance transthoracic echocardiog vention is needed (see Item 10).
raphy yearly if the aortic root or ascending aortic diam • Influenza vaccine should be administered to patients
eter is greater than 4 cm (see Item 97). with established cardiovascular disease to reduce the
• Infective endocarditis prophylaxis should be limited risk of future cardiovascular events (see Item 13).
to those with a prosthetic cardiac valve; a history of • Accelerated idioventricular rhythm is a common com
infective endocarditis; unrepaired cyanotic congen plication following coronary reperfusion and does not
ital heart disease or repaired congenital heart defect require intervention when it occurs within 24 hours of
with prosthesis or shunt (::;6 months post-procedure) reperfusion (see Item 42).
or residual defect; or valvulopathy following cardiac • Routine screening for coronary artery disease in
transplantation. asymptomatic patients with diabetes mellitus does not
• Antibiotic prophylaxis to prevent bacterial endocarditis reduce mortality (see Item 65).
is not recommended for nondental procedures, includ • Supervised exercise therapy can effectively treat clau
ing transesophageal echocardiography and genitouri dication, with increases in pain-free walking time and
nary or gastrointestinal procedures, in the absence of maximal walking time, and is recommended as part of
active infection (see Item 25). the initial treatment regimen for intermittent claudica
• There is no indication for patent foramen ovate closure tion (see Item 84).
or for antiplatelet therapy in asymptomatic patients, and • Asymptomatic first-degree atrioventricular block with
randomized trials do not support patent foramen ovale bifascicular block does not require pacemaker implan
closure to reduce risk of recurrent stroke or migraine. tation (see Item 109).
• A small atrial septa! defect (pulmonary-to-systemic blood • Patients with acute pericarditis who do not have
flow ratio [Qp:Qs] <l.5:1) with no associated symptoms or high-risk features (fever, leukocytosis, acute trauma,
right heart enlargement can be followed clinically. abnormal cardiac biomarkers, immunocompromise,
• A small membranous ventricular septal defect with oral anticoagulant use, large pericardial effusions, or
out left heart enlargement, pulmonary hypertension, evidence of cardiac tamponade) can be managed medi
recurrent endocarditis, or valve regurgitation can be cally on an outpatient basis with close clinical
observed clinically (see Item 45). follow-up (see Item 114).
xiv
Cardiovascular Medicine
Epidemiology and Women have a higher prevalence of risk factors for CVD,
including elevated cholesterol levels, diabetes mellitus,
Risk Factors hypertension, and inactivity. Only tobacco use is higher
among men.
Overview More women present with angina than men, but women
In the United States, the mortality rate from cardiovascular dis often have other symptoms in addition to chest pain. Women
ease (CVD), including heart disease, stroke, peripheral vascular have "atypical" symptoms more frequently than men, includ
disease, hypertension, and heart failure has steadily declined ing nausea, shortness of breath, and unusual fatigue. More
over the past decade-33% from 1999 to 2009, likely as a result than two thirds of women who die suddenly from coronary
of better prevention and acute care efforts. Nonetheless, CVD is heart disease either did not recognize the symptoms or had no
the leading killer of both men and women, and although mortal previous symptoms. Women undergo fewer revascularization
ity of CVD is decreasing, CVD prevalence is increasing. By 2030, procedures than men, with 25% of coronary artery bypass
according to the American Heart Association's Heart Disease surgeries and nearly 33% of percutaneous coronary interven
and Stroke Statistics, more than 40% of the U.S. population is tions occurring in women.
projected to have some form of CVD. More than one in three
American adults currently have some form of CVD, and the
prevalence increases from more than 10% in those aged 20 to
Eth n icity a n d
39 years to more than 70% in those aged 60 to 79 years. Based on Ca rd iovascu lar Disease
data from the Framingham Heart Study, two out of three men and The prevalence of CVD and risk factors in the United States
one out of two women will develop CVD in their lifetime. Despite vary by ethnicity. American Indians and Alaska Natives
the decreasing mortality, hospitalizations for cardiovascular have the highest rate of heart disease (12.7%), followed by
related diseases have steadily continued to rise. There were whites (11.1%), blacks or African Americans (10.7%),
nearly 6 million hospital discharges for cardiovascular-related Hispanics or Latinos (8. 6%), and Asians (7.4%). Peripheral
diseases in 2009, with an estimated cost of $312.6 billion. arterial disease affects nearly 8.5% of Americans older than
The prevalence of heart failure continues to rise, with a 40 years, and prevalence is highest among older persons,
predicted prevalence in the United States of 25% by 2030. It is non-Hispanic blacks, and women. The population most
estimated that 5.1 million Americans older than 20 years have affected by heart failure is African Americans, at a rate of
a diagnosis of heart failure. Currently, the incidence is 1/100 4.6/1000 person-years, followed by Hispanic, white, and
annually in those older than 65 years. Most of these patients Chinese Americans.
have a history of hypertension. Both systolic dysfunction and Cardiovascular risk factors also vary among ethnicities.
diastolic dysfunction are associated with the development of Blacks have the highest rate of hypertension, at 33.4%
symptomatic heart failure, and the prevalence of heart failure (higher in black women), followed by American Indians or
with preserved ejection fraction (diastolic dysfunction) is Alaska Natives (25.8%), whites (23.3%), Hispanics or Latinos
increasing. Mortality in heart failure is quite high-nearly 50% (22. 2%), and Asians (18.7%). Blacks have the highest preva
mortality at 5 years. lence of two or more cardiovascular risk factors (48.7%).
The prevalence of risk factors is increased with decreasing
levels of education and income. Obesity and lack of physical
Cardiovascu lar D isease i n Women activity are highest among Hispanic/Latino adults and non
Since 1984, the number o f deaths from CVD has been greater Hispanic blacks.
for women than men and highest among black women. More Environmental influences on cardiovascular risk factors
than 400,000 women died of CVD in 2009, 51% of all CVD are changing the prevalence of CVD in certain populations. In
deaths. Women have a higher mortality rate after myocardial countries with previously low rates of CVD, rates of disease are
infarction: 26% in women versus 19% in men older than increasing with the adoption of Western eating habits and
45 years. The death rate for women with heart failure is higher increasing tobacco use. With declining rates of infant mortality
than among men, although women are often older. Incidence and death from infectious diseases, the influence of urbaniza
of and mortality from stroke is highest among women, with tion and change in traditional lifestyles are resulting in increas
the highest among black women. ing rates of CVD.
1
Epidemio logy a n d Risk Factors
Lifestyle Risk Factors a prevalence greater than 70% in persons older than 65 years.
As much as 90% of the risk for myocardial infarction has been Treatment of hypertension reduces risk for cardiovascular events,
attributed to modifiable risk factors, with elevated cholesterol including stroke, and reduces end-organ damage such as heart
levels, smoking, and psychosocial stressors accounting for a failure and kidney disease. Although the prevalence of blood
significant portion of the attributable risk. The attributable risk pressure control (that is, blood pressure within recommended
for myocardial infarction is highest for cholesterol levels, fol ranges) has improved in the United States from less than 30% two
lowed by current smoking, psychosocial stressors, diabetes, decades ago (1988-1994) , it still is only 50% (2007-2008) .
hypertension, abdominal obesity, no alcohol intake, inadequate Sedentary lifestyle, poor diet, and obesity contribute to
exercise, and irregular consumption of fruits and vegetables. increased cardiovascular risk and increased risk for diabetes.
Elevated cholesterol levels increase the risk of CVD, and Nearly one third of all U.S. adults report no leisure time activity,
multiple studies have shown that reductions in cholesterol and less than 30% of high school students engaged in 60 min
levels, particularly LDL cholesterol, reduce risk. Nearly 14% of utes of daily physical activity; this rate was lowest among girls.
adults older than 20 years have total cholesterol levels greater Between 1971 and 2004, total energy consumption increased by
than 240 mg/dL (6.21 mmol/L}; approximately 6% of adults are 22% in women and 10% in men. Average fruit and vegetable
estimated to have undiagnosed hypercholesterolemia. Elevated consumption was 2.4 to 4 servings daily (recommended,
LDL cholesterol and low HDL cholesterol levels are independ >5 daily) and was lowest among blacks. The increased caloric
ent risk factors for CVD. For every 1 % decrease in LDL choles intake coupled with decreased physical activity has led to an
terol level, there is a corresponding 1 % decrease in risk for
increased incidence of obesity. More than two thirds of the
coronary artery disease. The risk reduction is even greater with
American population older than 20 years are overweight
changes in HDL cholesterol, with a risk reduction of 2% to 3%
(BM! 25-29.9) vvith more than one third obese (BMl>30). In chil
for every 1% increase in HDL cholesterol level. However, rand
dren and adolescents between the ages of 2 and 19 years, nearly
omized clinical trials evaluating pharmacologic therapies that
33% are obese or overweight and 17% of these children are obese.
raise HDL cholesterol levels in patients with well-treated LDL
Psychosocial stressors are an important contributor to
cholesterol levels have not shown reduction in clinical end
cardiovascular risk. These include depression, anger, and anx
points. Long-standing guidelines (Adult Treatment Panel III
iety, and are associated with worse outcomes. Depression has
[ATP III]) have provided treatment goals for LDL and non-HDL
been associated with higher risk for cardiovascular events,
cholesterol levels based on cardiovascular risk factors and
and psychosocial stressors also affect the course of treatment
Framingham risk score. In 2013, the American College of
and adherence to healthy lifestyles after an event. Awareness
Cardiology and the American Heart Association (ACC/AHA)
of these factors and appropriate therapies may improve out
published revised guidelines that treat lipid blood levels
comes in these individuals.
according to cardiovascular risk, rather than LDL cholesterol
Impaired glucose control is a significant component of
targets (see MKSAP 17 General Internal Medicine, Dyslipidemia).
the metabolic syndrome, which is characterized by elevated
The use of tobacco has declined over the past few decades,
but despite this decline, in 2011, more than 21.3% of men, 16.7% glucose, central obesity, low HDL cholesterol, elevated triglyc
of women, and 18% of high school students were smokers. The erides, and high blood pressure. More than 34 % of adults older
rates were highest among American Indian/ Alaska Natives and than 20 years meet the criteria for metabolic syndrome (three
non-Hispanic black males and lowest among Hispanic females. of the five components). The presence of metabolic syndrome
Tobacco use increases the risk of CVD, including coronary is associated with an increased risk of CVD. This risk increases
heart disease, stroke, and peripheral vascular disease, for which with an increased number of components and also appears to
smoking is a major risk factor, and increases CVD mortality by be higher among women. The National Diabetes Prevention
2 to 3 times. The risk of coronary artery disease is increased by Program found that in persons at high risk for diabetes,
25% in women who smoke. Smoking increases the risk of improved food choices and at least 150 minutes of exercise
stroke by 2 to 4 times. Secondhand smoke is also a risk factor weekly led to 5% to 7% weight loss and reduced the risk of
2
Epidemiology a n d Risk Factors
developing diabetes by 58%, but no interventions have shown burden is likely a result of both the inflammatory process of the
a reduction in CVD events to date. systemic disease, including a prothrombotic state, as well as
KEY POINT the presence of traditional cardiovascular risk factors.
3
Epidemio logy a n d Risk Factors
choice of therapy may be affected by reclassification of risk. Asp i ri n for Pri m a ry Prevention
Elevated hsCRP levels should be rechecked within 2 weeks, and
Aspirin is a powerful agent for both primary and secondary
other potential causes of infection or inflammation should be
prevention of coronary artery disease. Aspirin for second
ruled out. Although statin therapy has been shown to lower
ary prevention is discussed under Coronary Artery Disease.
hsCRP levels, therapy targeting hsCRP alone is not appropriate
For primary prevention of myocardial infarction, data sug
as patients should be treated according to cardiovascular risk.
gest that there is greater benefit in men, particularly those
Although elevated levels of Lp(a) lipoprotein and homo
older than 45 years. For women, benefit outweighs risk of
cysteine have been associated with elevated cardiovascular
aspirin therapy after the age of 65 years. Between the ages
risk, these tests should not be routinely performed.
of 55 and 65 years, the risk of stroke is reduced in women
Interventions to reduce homocysteine levels with folic acid
on aspirin therapy. Guidance for using aspirin for primary
supplementation have not been shown to reduce cardiovascu
prevention of myocardial infarction and stroke is provided
lar events. Although epidemiologic evidence supports the
in Table 1 and Table 2 .
association between elevated levels of Lp(a) lipoprotein and
I t i s important to balance the benefits o f aspirin therapy
cardiovascular events, to date no trials have shown that treat
with the risks of gastrointestinal (GI) bleeding. The risk of seri
ment to lower Lp(a) lipoprotein levels lowers risk. There is
ous bleeding is greatly increased in patients with a history of
currently no role for the evaluation of lipid particle size and
GI ulcers and who use NSAIDs, and these factors should be
number. No studies to date have shown that treatment targeted
considered when assessing the benefits and harms of using
to particle size and number affects outcomes.
aspirin in the individual patient.
The evaluation of subclinical disease with coronary artery
Aspirin should not be routinely given to patients with
calcium (CAC) scoring may be appropriate to further risk
diabetes who are at low risk for CVD (men <50 years and
stratify intermediate-risk patients but is not a component of
women <60 years with no major additional CVD risk fac
routine risk assessment. Evidence of calcification of coronary
tors; 10 -year CVD risk <5%). It is reasonable to give low
vessels is indicative of atherosclerotic disease, but the absence
dose aspirin to adults with diabetes and no previous
of calcification does not rule out the presence of soft plaque.
history of vascular disease who are at increased CVD risk
K EY P O I N T S (10-year Framingham risk >10%) and without increased
HVC • Current guidelines do not support the use of high- risk for bleeding.
sensitivity C-reactive protein (hsCRP) evaluation in the KEY P O I NTS
general population, but hsCRP testing may be used in
• In men ages 45 to 79 years, aspirin for primary preven
intermediate-risk patients in whom choice of therapy
tion of myocardial infarction is recommended if the
may be affected by reclassification of risk.
benefit of treatment outweighs the risk of gastrointesti
HVC • There is currently no role for the routine measure-
nal bleeding.
ment of Lp(a) lipoprotein levels or homocysteine lev
• In women ages 55 to 79 years, aspirin for primary pre
els or evaluation of lipid particle size as these tests
vention of stroke is recommended if the benefit of treat
are expensive and no studies to date have shown that
ment outweighs the risk of gastrointestinal bleeding.
treatment targeted to these levels affects outcomes.
HVC • Aspirin should not be routinely given to patients with HVC
• The evaluation of subclinical disease with coronary
diabetes who are at low risk; that is, men younger than
artery calcium scoring may be appropriate to further
50 years and women younger than 60 years with no
risk stratify intermediate-risk patients but is not a com
major additional cardiovascular risk factors.
ponent of routine risk assessment.
· . · ·
Adapted with permission from U.S. Preventive Services Task Force Aspirin for the prevention of card iovascular disease: U.S. Preventive Services Task Force recommendation
statement. Ann I ntern Med. 2009 Mar 1 7; 1 50(6):396-404. [PMID: i9293072]
4
D i a g n ostic Testing in Cardio lo g y
TABLE 2. Risk Level at Which CHO Events Prevented Exceed GI Harms in Patients Taking Daily Aspirin for Primary Prevention
Men Women
1 0-Year CHO Risk• 1 0-Year Stroke Riskb
'Risk factors for CHO include age, diabetes mellitus, total cholesterol level, HOL cholesterol level, blood pressure, and smoking. CHO risk estimation tool: http://cvdrisk.nhlbi.nih.
gov/calculator.asp. (Note: This is the Framingham risk score.)
bRisk factors for ischemic stroke include age, high blood pressure, diabetes mellitus, smoking, history of cardiovascular disease, atrial fibrillation, and left ventricular hypertrophy.
NOTE: This table applies to adults who are not taking NSAIOs and who do not have upper GI pain or a history of GI ulcers.
Adapted with permission from U.S. Preventive Services Task Force. Aspirin for the prevention of cardiovascular disease: U.S. Preventive Services Task Force recommendation
statement.Ann Intern Med. 2009 Mar 1 7; 1 S0(6):396-404. [PMIO: 1 9293072]
5
D i a g nostic Testi n g i n Cardiology
Exercise ECG I n itial diagnostic test in m ost Data acq u i red on exercise Not useful when baseline ECG is
patients with suspected CAD capacity, blood pressure and abnormal (LVH, LBBB, paced
heart rate response, and rhythm, WPW syndrome, >1 mm
p rovoked symptoms ST-segment depression)
Stress echocard iography Recommended when baseline Exercise data acq u i red along Image quality is suboptimal i n
ECG is abnormal or when with i mag ing for wal l motion some patients but can be
informatio n on a particular area abnormalities to i ndicate improved with microbubble
of myocard i u m at risk is ischemia transpu l monary co ntrast
needed
Allows evaluation of valve Image interpretation is difficult
function and pulmonary when basel ine wall motion
pressures abnormalities are present
Nuclear SPECT perfusion Recommended when basel i ne Gating ( i mage acq uisition Attenuation artifacts can be
ECG i s abnormal o r when coordinated with the cardiac caused by breast tissue or
information on a particular area cycle), use of higher energy diaphragm i nterference
of myocard i u m at risk is agents such as technetium,
Radiatio n exposure
needed and tech niques used to correct
f o r attenuation provide
With LBBB, conduction delay i n
improved specificity
the septum may cause false-
positive abnormal ity; this can Late reperfusion imaging
be i m proved with the use of allows evaluation of myocardial
vasodilator stress viability if thal l i u m is used
Dobutami n e Recommended i n patients w h o Because t h e patient is supi ne, Dobutamine contraindications are
echocardiography cannot exercise i mages are acquired severe baseline hypertension,
continuously, allowing the test unstable angina, and arrhythmias
Recommended when
to be stopped as soon as
i nformatio n on an area of �-Blockers m ust be withheld
ischemia is evident
myocardi u m at risk i s needed before the test
Vasod ilator nuclear Recommended in patients who Vasodi lator stress testing may Contraind ications are
perfusion (adenosine, cannot exercise m i n i mize effect of �-blockade bronchospastic airway disease,
dipyridamole, on perfusion defect size theophyl line use, sick sinus
May minimize septa!
regadenoson) syndrome, and high-degree AV
abnormalities frequently seen Can image sooner after
block
with nuclear perfusion myocardial i nfarction with
scan n i ng in patients with LBBB vasodilator stress Caffeine must be with held 24
hours before the test
Adenosine or d i pyridamole may
cause chest pain, dyspnea, or
fl ushing
Radiation exposure
Dobutami n e nuclear Recommended in patients who Has comparabl e sensitivity and Dobutam ine contraindications are
perfusion cannot exercise and have specificity to exercise or severe baseline hypertension,
contraindications to vasodilators vasodilator perfusion i mag i ng unstable ang i na, and arrhythmias
for diagnosis of myocard ial
Recommended when �-Blockers should be withheld
ischemia
information on an area of before the test
myocard i u m at risk is needed
Radiation exposure
PET/CT Provides best perfusion i mages Study d u ration is shorter and Not widely avai lable
i n larger patients rad iation dose is lower than
More expensive than other
conventional nuclear perfusion
Provides data on myocard ial i mag ing modalities
i magi n g
perfusion, function, and
Used with pharmaco logic stress
viability Absol ute myocardial blood
only (no exercise protocol)
flow can be measured
Radiation exposure
Can be combi ned with CAC
scoring
6
D i a g n ostic Testing i n Cardi o logy
Radiation exposure
CAC testing CAC testi ng is reasonable i n CAC scores a r e predictive of Does not provide data on
asymptomatic patients at cardiovascular risk in selected coronary l u m i n a l na rrowing
intermed iate risk for CAD patients
Radiation exposure
Coronary CT angiogra phy Identifies anomalous coronary Coronary a rtery vessel l u men Req u i res high-resolution (64-slice)
arteries and atherosclerotic lesions can CT instruments
be visualized i n detail
Useful for selected patients Does not provide d eta iled images
with intermed iate risk for CAD of distal vessel anatomy
C M R imaging Gadoliniu m-enha nced images Accu rate test for myocard ial Some patients experience
identify viable and i nfarcted via bility claustrophobia
myocard i u m
M a y b e contra indicated i n patients
Id entifies anomalous coronary with pacemaker, ICD, or other
arteries implanted device
AV = atrioventricular; CAC = coronary artery calcium; CAD = coronary artery disease; CMR = cardiac magnetic resonance; ECG = electrocardiography; ICD = implantable
cardioverter-defibrillator; LBBB = left bundle branch block; LVH = left ventricular hypertrophy; SPECT = single-photon emission CT; WPW = Wolff-Parkinson-White.
Cl capacity and heart rate and blood pressure response, can be of myocardial oxygen demand, patients should continue to
u t i lized in prediction models such as the D u ke t readmill score. exercise until l i m i ted by symptoms. Achiev i ng a rate pressure
CONT
which factors development of symptoms. degree of ST-segment product (heart rate x systol ic blood pressure) of at least
depression, and exercise duration to provide incremental 2 5 , 0 0 0 is also considered an adeq uate workload. as t h is
prognostic i n formation for 5-year mortality risk. Heart rate measure renects left ven t ricular myocardi a l performance. A
recovery is another powerfu l predictor; patients wit h a heart standard Bruce protocol i ncreases t he speed and grade of t he
rate drop of less than 12/m i n in the first m in u te after cessation t rea d m i l l every 3 minutes. and patients who have poor func
of exercise have a h igher mortality rate. tional capacity and ca n not achieve at least the first s tage of
lschemia is iden t i fied on the basis or t he development of the Bruce protocol (5 metabol i c equivalents [ M ETs]) have
1 m m or greater o f horizo n tal o r downsloping ST depression significantly h igher a l l -cause mortal i ty. Stress tests s hould be
with exercise (Figure I) . but the coronary territory i nvolved terminated when the patient has exerted maximal effort and
cam1ot be localized based on the ECG changes alone. Ideally, achieved at least 85'1o PM H R , the patient requests to stop or
patients should exercise for 6 to 12 m i n u tes to provide ade experiences sign i ficant angina! or other physical symptoms,
q uate t i me for development of maximal metabolic demand. or when other adverse markers develop. such as exertional
Although achieving 85% of t h e age-predicted maxi mal heart hypotension, signi ficant hypertension, ST-segment e levation
rate ( P M H R) is considered adequate for d iagnosis of i schemia. o r signifi ca n t ST-segmen t depressi o n . o r ven t ri c u l a r or
as heart rate a n d blood pressure are t he major determina nts supraventricular a rr hy th mias.
7
D i a g n ostic Testing in Card i ol ogy
BASEl.INE 2'-L>..
X ST
L••d Ltnd
EXERCISE STAGE 1 49 bpm ST @ l OmmlmV ST EXERCISE STAGE 4 136 bpm ST @ lOuunlwV ST
00:00 1.0 SOms poslJ Slopt 9:29 10.S SOu1> pa.tJ Slop<
''k
���;,jr-- � ��;.:1r-Jr--
�: I · ·:� l �: I "
�� �·1.1 r--��
•·· ··· ..
;; '1i
���� ��;;:t-�
0.5
0.1
0.4
-0.1
1.1
0. 1
0.1
-0.2
-1.9
-0.S
-2.4
-0.9
-2. 1
-0. l
-2.0
-1.0
F I G U R E 1 . El ectrocard iogram recorded d u ring exercise stress testi ng. The presence of 2-mm downsloping ST-segment depressions i n leads I, II, I l l, and aVF, and leads V3
through v6 ind icates ischemia.
CJ
The decision about whether to keep a patient on card iac t he pat ient is able to exercise because of t h e potential fr)r a
medications during stress tes t i ng should be i ndividual ized false- posit ive test owing to a septa! perfusion abnorma l i ty t hat
CONT.
based on the c l i nical question being addressed. I f the stress test may occur with exercise. The choice o f imaging moda l i ty
is being performed to establish the diagnosis of CAD, medica should be based on local expertise a nd patient characterist ics.
t ions such as �-blockers and n i t rates should be wit hheld for at I n stress tes t i ng w i t h adjunct ive imaging, basel i n e i m ages
least 24 hours before the test. If the stress test is being performed are obt a i ned and compared w i t h i m ages obtained after ei ther
to evaluate symptoms or to define prognosis in a patient with exercise or pharmacologic stress (Table 4) . Exercise invokes
known d isease. the patient should remai n on current therapy to ischemia as t he epicard i a l vessels become unable to m a i n t a i n
determ ine i f ischemia is presen t on the current regimen . adeq u a te flow related t o myocardial oxygen d e m a n d via
There are several indications for stress testing w i t h add i autoregu lation, and ischemia develops d i stal to t he obstruc
ti onal imaging with either echocardi ography, CM R imaging, or t ion . Dobuta m i n e. l i ke exercise, i ncreases myocard i a l oxygen
perfusion imaging w i t h S PECT or PET/CT. These include i nabil demand and e l i c i ts ischemia because of i nsuff1cient perfusion
ity to exercise. base l i ne ECG abnormali ties, and cond it i o n s i n to the a ffected myocardium. Vasodilators. such as regadenoson
which exercise i s contrai nd icated . Patients w i t h abnormal or adenosine, produce hypere m i a and a flow d isparity between
base l i n e ECGs that i n terfere with the i nterpreta t i o n of t he exer myoca rdium suppl ied by the stenotic vessel ( i n which the d i s
cise ECG ( for example, left bundle branch block [LBBB] . left tal vasculature is a l ready max i m a l ly d i lated) as compared w i t h
ventricular hypertrophy w i t h ST-segment abnorma l i ties. or a the myocard i u m supp l i ed b y unobstructed vessels. I n add i t ion
paced rhyt h m ) should u ndergo s t ress i m ag i ng lo iden t i fy to ide n t i fy i ng t h e presence o f d i sease, perfusion imagi ng can
obstruct ive CA D. I n addition, stress testing w i t h imaging may define the loca t ion and extent o f reduced perfusion and pro
be helpful lo elucidate a d i agnosis i n patients w i t h i ndeterm i vide add i t i o nal prognostic i n fo rmat io n compared with ECG
nate resu lts on t readm i l l test i ng. Patients with right bundle s t ress tes t i ng alone. These i m aging mod a li t ies may a lso be
branch block ( RBBB) . b i fascicular block. or who are o n d igoxin used to q ua n t i fy i n farction and assess myocard i a l viab i l i ty. The
can undergo exercise stress test i ng. but ST segments may be additional i n formation and impact on patient care obtai ned
more d i fficu l t to interpret or may produce false-posi tive resu l ts. with i maging must be bala nced with the add i t ional costs,
Patients w i t h severe aort ic stenosis, abdomi na l aortic aneu t i me, and exposure to rad i a t i o n or contrast agents incurred .
rysm, severe hypertension. or uncontrolled a rrhyth m i as should Exercise stress echocardiography is performed w i t h either
not exercise : rather, t hese pat ients should undergo pharmaco supi n e ergometry or t read m i l l test i ng. Supine ergometry allows
logic stress test i ng with vasod i lators. Pa t ients who are unable to for contin uous i maging during exercise. whereas with t read
exercise should u ndergo pharmacologic stress testing w i t h m i l l tes t i ng. i mages need to be obtained i mmediately after
i magi ng. I n addit ion . i n patients with LBBB u ndergoing nuclear exercise. and any delay can reduce t he accuracy of the i n forma
stress testing, a pharmacologic stressor should be used even i f t ion obtai ned. New regional wal l motion abnormalities seen on
8
D i a g n ostic Testing in Card i o logy
Stress Echocardiography
At Rest After Stressor I nterpretation
Normal Normal Normal, no ischemia
Normal Wa l l motion abnorma lity Normal function at rest, ischemia with stress
Abnormal Abnormal I nfarct
Normal LV d i l ation Sma l l or no distinct zone of ischemia, possi ble bala nced isch emia or
m u ltivessel CAD
CAD = coronary artery disease; LV = left ventricle; SPECT = single-photon emission CT.
Cl
t h e echocardiogram fo l l owing exercise i n d i cate areas o f stress echocardi ography a l l ows assessment of wall motion at
ischemia (see Tab l e 4) . Wal l m o t i o n abnorma l i t i es at rest t ha t rest and at peak or i m mediately following imaging to assess for
CONT.
do not change w i t h exercise usually ind icate i n farct ion. obstructive CA D. I f the examination is performed to assess
I mprovement i n regional wall motion w i t h low-dose exercise dyspnea on exertion or valvular function w i t h exercise, t hese
or dobutamine that worsens at higher levels suggests viable but should be specifically requested in order to be sure t ha t ade
h ibernating myocardi u m . A s wit h perfusion i m aging, t h e quate echocardiographic i n formation is o b t a i ned . Rout ine
extent o f w a l l motion abnormali t ies provides prognostic i n for t ranst horacic echoca rdiography (TTE) evaluates left and right
mation regarding risk of future cardiovascul a r events. ventricular size. t h ickness, and function; valvular morphology
The sensitivity of stress echocardiography is reduced with and fu nction; diastolic fu nction ; and t h e pericardium. These
s i ngle-vessel disease and i s dependent on timely i maging. In a re not necessarily rout i nely performed in a st ress echocardio
addition, i n terpretation can be more subjective t h a n with gram so if' t h is i n forma tion is clinica l ly i mportant, it m ay be
o t h e r m od a l i t ies, parti c u l a rly w i t h base l i ne wa l l motion necessary to obtain both a TTE and a s t ress echocardi ograrn.
abnormali ties o r systolic dysfunction . A major advantage of Dobutam i n e stress echocardiography is used for patients who
s t ress echocard i ography i s t h e abi l i ty to obtain add i t ional cannot exercise and can be particularly useful for evaluation of
i n format i o n , such as changes i n p u l monary pressures o r myocardial viab i l ity (Table 5) and to evaluate aortic stenosis i n
cha nges i n valvular fu nction w i t h exercise. At m i n i m u m . patients w i t h a low ejection frac tion.
9
D i a g nostic Testing i n Card i ol ogy
images, it signifies a high degree of stenos is. ease, because it is these patients who, by the result of
their stress test, can be reclassified into higher or lower
risk categories.
�UPlliE • In patients who are able to exercise and have a normal HVC
_t.flT
baseline electrocardiogram, the initial type of stress
SEP l4T \ testing should be exercise stress testing.
STIUSS_fBP(G} • Patients with abnormal baseline electrocardiograms
t) l)
SUPlflE
(ECGs) that interfere with the interpretation of the exer
;.rn
)
cise ECG (for example, left bundle branch block, left
SEP , L.r
' ( ventricular hypertrophy with ST-segment abnormali
HIF
FUST 1-IJP(G} A ties, or a paced rhythm) should undergo stress imaging
to identify obstructive coronary artery disease.
·
. ''"H<E
AllT
......
f.;:.5E • ll..F' EX
CJ
Visualization of the Coronary Anatomy
l�JF
STnFSS FRP(G) Coronary angiography and coronary CT angiography (CTA)
,,.
·;.1.w·11;i::;
AtlT provide anatomic information regarding the coronary vessels
.... ....
. ..,
�·
(Figure 3) . Both procedures require iodinated contrast and
f:&.SE
.., ,,, J.:. - expose the patient to radiation. Coronary angiography pro
tiff
R£ST FBP(GJ B vides a two-dimensional image of the lumen of the vessel filled
with contrast. Assessment of the stenotic lesions is made from
F I G U R E 2 . Selected images from a nuclear perfusion single-photon emission
multiple views of the vessel. Coronary CTA can provide addi
CT (SPECT) stress study. Short axis views (panel A ) of the heart with stress (top row)
and at rest (bottom row) show a radiotracer defect in the septum and inferior wall tional information about some of the characteristics of the
that is filled on the rest images. Long axis views (panel 8) demonstrate an apical plaque. I f. however. culprit lesions are visualized on coronary
filling defect with stress ( top row) that is perfused on rest images (bottom row). CTA. the patient typically requires coronary angiography for
10
Diag nostic Testing i n Cardiology
KEY POINT
• Measurement of coronary artery calcium should be lim- HVC
ited to a select group of asymptomatic patients with an
intermediate Framingham risk score (10%-20%) in
whom results will influence treatment strategy because
of its associated cost and radiation exposure.
c::J
stress test and additional downstream testing. Obtaining a
better definition of the degree of coronary stenosis. Coronary CAC score in a low- risk pat ient may lead to additional tests or
angiography is also required if coronary revascularization is to procedures for an incidental finding on CT.
CONT.
be performed. If percutaneous coronary i n terven tion is indi- Exercise testing is associated with a small risk of myocar
cated. it may be performed a t the t i me of a patient's diagnostic dial infarction or death (1 1 2500 tests) . Exercise stress testing is
catheterization. Coronary a ngiography may be useful as a contraindicated in patients with unstable cardiac conditions,
diagnostic test in patients who, despite maximal medical ther such as uncontrolled cardiac arrhythmias, severe symptomatic
apy, have intolerable ischemic symptoms as long as they are aortic stenosis. uncontrolled heart failure. and unstable angina.
candidates for coronary revascularization. Coronary CTA may Pharmacologic stress agents. including dipyridamole, adeno
be used to rule o u t CAD in symptomatic patients with a n sine, and regadenoson. are associated with development o f
intermediate r i s k of coronary disease. O t her l i m i tations of high-degree atrioventricular b l o c k a n d bronchospasm .
coronary CTA include poor visualization of distal vessels and Nuclear stress testing, CAC scoring. coronary CTA. and
a rtifact from calcification that may l i mit interpretation. coronary angiography expose the patient to radiat ion . The
Suspected coronary anomalies, such as anomalous coro amount of exposure is dependent on factors such as the radi
nary origins, can be evaluated by coronary CTA. CMR imaging. otracer used. equipment, operator technique, complexity of
or coronary angiography. These imaging modali t ies can help procedure performed. and patient characteristics (such as
identify those abnormalities that are associated with a higher body size) .
risk of sudden cardiac death. Cl Various contrast agents are used for CMR imaging (gado
l i n i u m ) . echocardiography (microbubble contrast agents used
Coronary Artery Calcium Scoring for enhancement of endocardial borders ) . coronary CTA. a nd
Coronary artery calcium (CAC) scoring provides information coronary a ngiography (nonionic contrast) . Nonionic contrast
regarding the burden of atherosclerotic disease but does not may be associated with hypersensitivity reactions and acute
provide information regarding the degree of obstruction it kid ney i nj u ry whereas gadolinium is associated w i t h the
may be causing. CAC scoring can be performed with either deve lopment of nephrogenic fibrosing dermatopathy in
electron beam or multi-detector CT, and newer technologic patients with chronic kidney disease.
advances limit radiation exposure to the patient. It detects the Cardiac catheterization can resu l t in complications from
presence of calcification in the walls of the coronary arteries, vascular access, injury to the coronary arteries. dissection of
which is directly proportional to the degree of plaque burden the aorta. or disruption o f plaque res u l t i ng in peripheral
present. CAC scores are categorized as follows: 0, no disease; embo l i and possible stroke. Vascular access complications
1-99, mild disease; 100-399, moderate disease; and above 400, include retroperitoneal hematoma from bleeding at the groin
severe disease. Coronary calcium scores greater than 400 are access site as well as pseudoaneurysm at the arterial puncture
11
D i a g n ostic Testing in Card i o logy
HVC • Routine yearly imaging evaluation of structural h eart quent events, an implanted loop recorder may be wananted.
disease in asymptomatic patients is usually not indi Exercise testing is also frequently employed in patients with
cated; benign murmurs, su c h as gra de 1/6 or 2/6 a suspected or known arrhythmja. Treadm i l l exercise testing is
midsystolic murmurs, are conm1on with pregnancy, an important tool for evaluating chrnnotropic response. ischenua,
anemia, and other high-flow states and do no t ro u t i n ely and exercise-i nduced or adrenergically induced arrhytlU1Ua.
need echocardiographic evaluation . O nce a p a t i e n t is d i agnosed w i t h an arrhy t h m i a or
arrhy t h m ia- prone cardiovascu lar condition. d iagnostic elec
• Evaluation of structural heart disease typically begins
trophysio logy testing can be helpful for both risk strati fication
with transthoracic echocardiography, which provides
a n d treatment (such as catheter ablat io n ) . Selection o f t hese
information about ventricular cavity size, thickness, and
d iagnostic tests is dependent upon the particular pat ient and
function, as well as quantitative information regarding
the d iagnostic concerns, and most patients w i t h arrhy t h m ias
valvular function, diastolic function, and filling pressures.
do not requ i re a n electrophysiology study. CJ
12
D i a g n ostic Testi n g in Card i o logy
Three-d imensional Mitra! valve disease Improved tomographic imaging Adjunct to two-d imensional
echocardiography i maging
ASD (percutaneous ASD Used d u ring cardiac procedures
closu re) for device placement Limited ava i l a b i l ity a n d expertise
Improved assessment of LV
g l o bal/reg ional systol i c function
Radionuclide a n g iography Evaluation of LV systolic Qua ntitative EF measurem ents Radiation exposure
( M UGA) function
Accu rate for serial LVEF No data on other cardiac
measurements with card iotoxic structures
drugs
CMR imaging Congenital heart disease Hig h-resolution tomogra phic Lim ited avai lability a n d expertise
imaging a n d blood-flow data
Aortic disease Some patients experience
Qua ntitative RV volumes and EF cla ustrophobia
Myocardial disease ( i nfiltrative
disease, myocard itis, No i o n izing radiation o r contrast May be contra i n d icated in
hypertrophic cardiomyopathy) patients with pacemaker, !CD, or
Enab les three-dimensional
other i mplanted devices
RV cardiomyopathy (ARVC) reconstruction of aortic and
coronary anatomy Gadolinium is contra i n d icated i n
Qua ntitation of LV mass a n d
kidney fa i l u re
fu n ction
Sinus rhyth m and slower heart
rate are needed for improved
image qual ity
Chest CT Aortic disease High-resolution tomogra phic Radiation and radiocontrast
images exposure
Coronary disease
Enab les th ree-d imensional
Cardiac masses
reconstructi on of vascu l a r
Perica rd ia! disease structures
ARVC = arrhythmogenic right ventricular cardiomyopathy; ASD = atrial septal defect; C MR = cardiac magnetic resonance; EF = ejection fraction; ICD = implantable cardioverter
defibrillator; LV = left ventricle; LVEF = left ventricular ejection fraction; MUGA = multi-gated acquisition; RV = right ventricle.
13
Coro n a ry Artery D i sease
Resting ECG I n itial d i agnostic test i n a l l 1 2-lead ECG recorded d u ring Most arrhythm ias are
patients the arrhyth mia often identifies interm ittent and not recorded
the specific arrhyth m ia on a resting ECG
Ambulatory ECG ( H o lter Frequent (at least dai ly) Records every heart beat Not helpful when arrhyth mia
monitor) asymptomatic or symptomatic d u ring a 24- or 48-hour period occurs less frequently
arrhythmias for later analysis
ECG leads l i mit patient
Patient log al lows correlation a ctivities
with symptoms
Exercise ECG Arrhyth mias p rovoked by A llows d ia gnosis of exercise- Physician supervision needed
exercise related a rrhyth mias i n case a serious a rrhyth m ia
occurs
Al lows assessment of i m pact of
arrhyth mia on blood pressure Most arrhythmias are not
exercise related
Patient-triggered event I nfrequent symptomatic S m a l l , pocket-sized recorder is Only useful for symptomatic
recorder a rrhyth mias that last more than held to the chest when arrhyth mias that persist long
1 -2 min utes symptoms are present enough for patient to activate
the device
Recorded data are transm itted
to central monitoring service Arrhythmia on set not recorded
ECG = electrocardiogram.
KEY POINT
Corona ry Artery Disease
• Patients with a suspected arrhythmia who experience
daily symptoms can be evaluated with a 24- or 48-hour Sta ble Ang i n a Pectoris
ambulatory electrocardiographic monitor (Holter moni
Diagnosis and Evaluation
tor) , whereas patients with less frequent episodes
The most common manifestation of coronary artery disease
require other monitoring strategies, including various
(CAD) is stable angina pectoris: chest pain, pressure, or dis
types of longer-term event recorders.
comfort that develops with exertion and is relieved with rest.
14
Coronary Artery D isease
Symptoms often occur when the burden of atheromatous optimal medical therapy, measure exercise capacity, and
plaque results in fixed coronary stenosis and limitation of evaluate the extent and severity of ischemia.
blood flow, leading to an imbalance between myocardial The development of coronary CTA is an emerging
oxygen supply and demand. When patients with cardiovascu alternative to stress testing, but coronary CTA does not
lar risk factors present with chest pain, the location of the provide important functional information, such as extent of
pain, quality of symptoms (sharp/dull, transient/persistent, ischemia, reproduction of symptoms, or exercise capacity.
occurring at rest/with exertion) , and the age and sex of the Coronary CTA is useful for diagnostic purposes in patients at
patient can help to differentiate stable angina pectoris from intermediate risk for CAD if stress testing is contraindicated
other causes of chest pain, such as gastrointestinal, musculo or revascularization is unlikely to be performed or change
skeletal, or pulmonary causes. These factors are also used to management.
determine a patient's pretest likelihood of CAD (Table 8) . The use of invasive coronary angiography in patients with
The decision to perform exercise or pharmacologic stable angina pectoris is generally limited to those with persis
stress testing or coronary CT angiography (CTA) is based on tent or progressive life-limiting symptoms while on optimal
the pretest likelihood of CAD, the patient's baseline electro medical therapy or those with high-risk criteria on noninva
cardiogram (ECG) , the patient's ability to exercise, and the sive stress testing or coronary CTA (see Figure 5) .
patient's comorbid illnesses, such as asthma or emphysema,
KEY POINTS
that would limit pharmacologic testing (Figure 5) .The selec
tion of tests for evaluating patients with chest pain is dis • When patients with cardiovascular risk factors present
cussed in Diagnostic Testing in Cardiology. Stress testing is with chest pain, the quality of symptoms, the age, and
most useful in patients at intermediate pretest likelihood of the sex of the patient can help to differentiate stable
CAD (10% to 90%) . In patients with low pretest probability, a angina pectoris from other causes of chest pain.
normal test result only confirms that the patient is low risk, • Stress testing is most useful in patients at intermediate HVC
and an abnormal stress test result is most likely a false pretest likelihood of coronary artery disease (10% to 90%) .
positive, possibly leading to more testing (additional stress
testing or invasive angiography) . In patients with a high pre General Approach to Treatment
test likelihood, the use of stress testing for diagnostic pur of Stable Angina Pectoris
poses is not indicated, as an abnormal test result only All patients with ischemic heart disease should be counseled
confirms the presence of disease and a normal test result is on the importance of risk factor modification, including life
most likely to indicate a false-negative result. style changes, such as smoking cessation, weight manage
Stress testing in patients with high pretest likelihood can ment, daily physical activity, and diet modification; as well as
be used to obtain prognostic information, but the results control of modifiable risk factors, such as diabetes mellitus,
should not affect the initiation of optimal medical therapy. In hype1iension, and hyperlipidemia. Medical therapy should be
patients who have been started on medical therapy for CAD, initiated in all patients with ischemic heart disease. The com
stress testing can be used to determine a patient's response to bination of risk factor modification and medical therapy is
TABLE 8. Pretest Likelihoods of Coronary Artery Disease in Low-Risk and High-Risk Symptomatic Patients
Pretest Likelihood
Nonanginal Chest Pain• Atypical Anginab Typical Angina<
Age (y) Men Women Men Women Men Women
aNonanginal chest pain has one or none of the components for typical chest pain.
bAtypical angina has two of the three components for typical angina.
C"fypical angina has three components: ( 1 ) substernal chest pain or discomfort, (2) provoked by exertion or emotional stress, (3) reli eved by rest and/or n itrog l yce r i n .
NOTE: Each value represents the percentage with significant coronary artery disease. The first is the percentage for a low-risk, mid-decade patient without diabetes mellitus,
smoking, or hyperlipidemia. The second is that of the same-age patient with diabetes mellitus, smoking, and hyperlipidemia. Both high- and low-risk patients have normal results
on resting electrocardiography. If ST-T wave changes or 0 waves had been present, the likelihood of coronary artery disease would be higher in each entry of the table.
Adapted with permission of Elsevier Science and Technology Journals, from Gibbons RJ, Abrams J, Chatterjee K. ACC/AHA 2002 guideline update for the management of
patients with chronic stable angina--summary article: a report of the American College of Cardiology/American Heart Association Task Force on practice guidelines (Committee
on the Management of Patients With Chronic Stable Angina). J Am Coll Cardiel. 2003 Jan 1 ;4 1 ( 1 ): 1 S9-68. [PMID: 1 2S 70960]; permission conveyed through Copyright Clearance
Center, Inc.
15
Coro n a ry Artery Disease
Cardiovascular symptoms
I
Intermediate
Yes
Coronary angiography
F I G U R E 5 . Diagnosis of coronary artery d isease. CAD = coronary artery d isease; ECG = electrocard iogram; EF = ejection fraction; T I D = transient ischemic d i lation.
referred to as guideline-directed medical therapy (Figure 6) . currently recommended only following percutaneous coro
Medical therapy is divided into two categories: cardioprotec nary intervention (PCI) or an acute coronary event.
tive medications and antianginal medications. Cardioprotective Owing to the protective effects of p-blockers, these agents
medications improve survival, reduce the occurrence of car are considered first-line therapy in patients with stable angina
diovascular events, and reduce the progression of systemic pectoris. Dose titration of P-blockers is recommended until
atherosclerosis. Antianginal medications vasodilate the coro the resting heart rate is between SS/min and 6 0 /min.
nary vasculature or decrease myocardial oxygen demand, thus P-Blockers have been associated with fatigue, reduced exercise
reducing the frequency and severity of angina pectoris and capacity, symptomatic bradycardia, mood disturbance
improving quality of life. (depression) , and erectile dysfunction. P-Blockers are con
traindicated in patients with symptomatic bradycardia, high
Cardioprotective Medications grade atrioventricular block, acute decompensated heart
The use of aspirin is associated with a decreased risk of myo failure, and severe reactive airways disease.
cardial infarction, stroke, and cardiovascular death in patients ACE inhibitors are indicated in the treatment of patients
with CAD. Aspirin doses of 81 mg to 162 mg daily are recom with stable angina pectoris to reduce cardiovascular and all
mended in all patients with established CAD unless contrain cause mortality. This effect on mortality is more profound in
dicated. In patients allergic to aspirin, clopidogrel is recom patients with concomitant diabetes mellitus and left ven
mended as an alternative. The use of newer antiplatelet agents tricular systolic dysfunction. Additionally, ACE inhibitors are
(prasugrel, ticagrelor) as monotherapy has not been tested in indicated in patients with concomitant systemic hyperten
patients with stable angina pectoris. Dual antiplatelet therapy sion and proteinuric chronic kidney disease. ACE inhibitors
(aspirin plus either clopidogrel, prasugrel, or ticagrelor) is are contraindicated in pregnant women and caution is
16
Coro n a ry Artery D isease
Continued symptoms
y
• I ncrease dose of B-blocker
• I ncrease dose of long-acting n itrate
• Add ca lci u m channel bl ocker (if not a l ready receivi ng)
y
Revascu la rize
Recommendations based on Oaseem A, fihn SD, Dallas P, Williams S, Owens DK, Shekel le P; Clinical Guidelines Committee of the American College of Physicians. Management of stable ischemic heart disease: summary of a clinical prac·
tice guideline from the American College of Physicians/American College of Cardiology foundation/American HeartAssociation/American Association for Thoracic Surgery/ Preventive Cardiovascular Nurses Association/Society oflhoracic
Surgeons.Ann Intern Med. 2012 Nov 20;157(1 0):735-43. [PMID: 23165665]
advised in patients with advanced chronic kidney disease. is proportional to the degree of LDL cholesterol reduction;
Angiotensin receptor blockers are considered acceptable however, statins have been proved to be beneficial in patients
alternatives in patients who are allergic to or intolerant of regardless of cholesterol level. New cholesterol management
ACE inhibitors. guidelines recommend the use of moderate- to high-intensity
Statins have been shown to reduce the risk of myocardial statins for all patients with (1) LDL cholesterol level of190 mg/
infarction and death in patients with chronic ischemic heart dL (4.92 mmol/L) or greater; (2) diabetes mellitus; or (3)
disease by 25% to 30%. The reduction in cardiovascular events greater than 7.5% estimated 10-year risk of atherosclerotic
17
Coro n a ry Artery Disease
cardiovascular disease (ASCVD) . Management of statin ther constipation. Ranolazine should be used with caution in
apy is discussed in MKSAP 17 General Internal Medicine. patients with advanced kidney or liver disease and in those
According to Advisory Committee on Immunization taking medications that are potent inhibitors of the CYP3A4
Practices (ACIP) guidelines, all persons aged 6 months or older pathway. Examples of strong inhibitors of the CYP3A4 path
should have an annual influenza vaccination. In addition, way include ketoconazole, clarithromycin, tacrolimus, and
patients at risk for cardiovascular disease warrant influenza cyclosporine.
vaccination as a preventive measure for cardiovascular disease.
KEY POINTS
The use of selenium, chromium, �-carotene, vitamin C,
• Aspirin or clopidogrel (if aspirin-allergic) is recom
vitamin E, and estrogen has not been associated with improved
mended in all patients with established coronary artery
cardiovascular outcomes and is not recommended in patients
disease unless contraindicated; the use of newer anti
with ischemic heart disease.
platelet agents (prasugrel, ticagrelor) as monotherapy has
Antianginal Medications not been tested in patients with stable angina pectoris.
Medications to reduce the frequency and severity of angina • All patients with stable angina pectoris should receive
pectoris comprise �-blockers, nitrates. calcium channel block a statin and a �-blocker.
ers, and ranolazine.
• ACE inhibitors are indicated in the treatment of stable
�-Blockers and nitrates are first-line antianginal agents. In
angina pectoris, particularly in patients with concomitant
addition to their cardioprotective effects, �-blockers improve
diabetes mellitus and left ventricular systolic dysfunction.
angina pectoris by reducing heart rate, myocardial contractility,
and blood pressure, resulting in reduced myocardial oxygen
Coronary Revascularization
demand. Nitrates improve myocardial oxygen supply and
reduce myocardial oxygen demand by their effects on coronary Decision to Revascularize
and systemic vasodilation, respectively. Nitrates have not been In patients with stable angina pectoris whose symptoms are
proved to reduce the frequency of cardiovascular events (myo not improved with optimal medical therapy, invasive angiog
cardial infarction, death) . Two categories of nitrates are indi raphy is warranted to define coronary artery anatomy and
cated for patients with stable angina pectoris: sublingual or prepare for revascularization via PC! or coronary artery bypass
spray nitroglycerin (for emergency use) and topical or oral graft surgery (CABG) . All patients should be counseled on the
nitroglycerin (for chronic, daily use) . The use of daily nitrates risks, benefits, and alternatives to angiography and revascu
requires periodic nitrate-free intervals (typically at night) to larization before diagnostic angiography is pursued.
avoid the development of tolerance. The most frequent adverse In patients found to have significant CAD on angiography
effect of nitrates is headache. The use of either short- or long that would benefit from revascularization, multiple factors are
acting nitrates is contraindicated in patients who take phos considered in deciding which technique (PC! or CABG) would
phodiesterase 5 (PDE-5) inhibitors for erectile dysfunction be best for the patient. These include the degree of left ven
(sildenafil, vardenafil, tadalafil) owing to the potentiation of tricular systolic dysfunction, whether the patient has had a
hypotension when these drugs are used together. prior CABG, and the patient's ability to adhere to a medication
Calcium channel blockers are second-line therapy i n treatment regimen. The SYNTAX score is an anatomic scoring
patients with stable angina pectoris who are intolerant o f system based on the results of angiography that quantifies
�-blockers or who have continued symptoms on �-blockers lesion complexity in patients vvith multi-vessel and/or left
and nitrates. All calcium channel blockers cause systemic main coronary artery disease and is useful in helping predict
and coronary vasodilation, and nondihydropyridine calcium the outcome of different revascularization strategies. The
channel blockers (diltiazem, verapamil) reduce the heart development of appropriate use criteria (AUC), a collection of
rate. Because of their vasodilatory properties, calcium chan clinical scenarios that mimic frequently encountered patient
nel blockers are first-line agents for the management of presentations, has assisted clinicians in making treatment
patients with Prinzmetal (variant) angina pectoris. The most decisions for patients with all forms of ischemic heart disease.
common adverse effects of calcium channel blockers are
peripheral edema, dizziness, constipation, and bradycardia. Percutaneous Coronary Intervention
Calcium channel blockers are contraindicated in patients PC! has not been shown to be superior to optimal medical
with left ventricular systolic dysfunction or advanced atrio therapy in patients with stable angina pectoris for reduction of
ventricular block. cardiovascular endpoints such as m011ality and myocardial
Ranolazine is a selective inhibitor of the late inward infarction. However, PC! has been associated with improve
sodium channel in the myocardium. It is generally reserved for ment in quality of life by reducing the severity and frequency
patients who remain symptomatic with the use of �-blockers, of angina. Current guidelines recommend that diagnostic
nitrates, and calcium channel blockers. Ranolazine is an effec angiography and PCI be reserved for patients with refractory
tive antianginal medication; however, its use is limited by cost symptoms while on optimal medical therapy, those who are
and adverse effects such as dizziness, headache, nausea, and unable to tolerate optimal medical therapy owing to side
18
Coron a ry Artery D isease
effects, or those with high-risk features on noninvasive exercise ischemic risk is high and bleeding risk is low (Table 9) . A major
and imaging tests. risk with premature discontinuation of dual antiplatelet ther
apy is the occurrence of stent thrombosis, a complication with
Coronary Artery Bypass Graft Surgery high morbidity and mortality.
The use of CABG in patients with stable angina is generally In patients who undergo CABG, preoperative cardioprotec
indicated only for those who remain symptomatic with opti tive and antianginal medications should be continued indefinitely.
mal medical therapy and have specific angiographic findings The benefit of cardioprotective medications (aspirin, �-blockers,
(either left main disease or multivessel disease with involve ACE inhibitors, statins) is greatest in patients with high-risk fea
ment of the proximal left anterior descending artery) , con tures such as reduced left ventricular systolic function, prior
comitant reduced systolic function, or diabetes mellitus. CABG myocardial infarction, chronic kidney disease, or diabetes.
is typically performed via median sternotomy incision and
KEY P O I NTS
institution of cardiopulmonary bypass ; however, recent
advances with off-pump CABG allow patients to avoid the • Percutaneous coronary intervention improves angina
need for cardiopulmonary bypass. Off-pump CABG is associ symptoms and quality of life in patients with stable
ated with adverse event and graft patency rates similar to tra angina pectoris but does not increase survival or reduce
ditional CABG. This less invasive procedure may be more suit future cardiovascular events.
able for patients with significant comorbid medical conditions • For stable angina pectoris, percutaneous coronary HVC
as it may reduce operative risk and shorten hospital and recov intervention is reserved for patients with refractory
ery times, but definitive proof is lacking. symptoms while on optimal medical therapy, those who
are unable to tolerate optimal medical therapy owing
After Revascularization to side effects, or those with high-risk features on non
The long-term goals of therapy for ischemic heart disease are invasive imaging.
to maximize quality of life and exercise function and minimize • Clinical practice guidelines do not recommend the HVC
morbidity and mortality. Clinical practice guidelines do not routine use of ECG monitoring, stress testing, or ana-
recommend the routine use of ECG monitoring, stress testing, tomic testing (coronary CT angiography or invasive
or anatomic testing (coronary CTA or invasive angiography) in angiography) in asymptomatic patients after percuta-
asymptomatic patients after PC! or CABG. neous coronary intervention or coronary artery bypass
All patients with stable angina pectoris who undergo PC! graft surgery.
or CABG should be treated with aspirin (81-162 mg/d) indefi
• In patients with stable angina pectoris who undergo
nitely. In patients who undergo PCJ, dual antiplatelet therapy
percutaneous coronary intervention, dual antiplatelet
(aspirin plus clopidogrel} is recommended for at least 1 month
therapy (aspirin plus clopidogrel) is recommended for
after bare metal stent (BMS) implantation and at least 1 year
at least 1 month after bare metal stent implantation and
after drug-eluting stent (DES) implantation, although extended
at least 1 year after drug-eluting stent implantation.
use can be considered on an individual basis if a patient's
CABG = coronary artery bypass grafting; NSTEMI = non-ST-elevation myocardial infarction; STEMI = ST-elevation myocardial infarction; UA = unstable angina.
•Dual anti platelet therapy consists of aspirin and another antiplatelet agent, with aspirin taken indefinitely unless contraindicated.
bPreliminary data suggest clopidogrel improves patency of bypass grafts after CABG.
NOTE: Extended dual anti platelet therapy can be considered if the risk-benefit ratio is favorable.
19
Coro n a ry Artery D i sease
F I G u R E 7 . Diagnosis o f acute coronary syndromes. CK-M B = creatine kinase M B ; ECG = electroca rdiographic; NSTEMI = non-ST-elevation myocardial infarction;
STEMI = ST-elevation myocard ial infarction.
20
Coro n a ry Artery D i sease
T
I n itial medical thera py: aspirin, &blocker, n itrates,
I
heparin (u nfractionated or l ow-mo lecu lar-weight)
F I G U R E 8 . Management of ST-elevation myocardial infarction. EF = ejection fraction; FMC= first medical contact; PCI = percutaneous coronary intervention;
STEMI = ST-elevation myocardial infarction.
�1f 4 or more hours have elapsed since symptom onset, PCI is preferred.
' High-risk features such as cardiogenic shock and heart failure favor PCI.
'FMC-to·device ("door·to·balloon·) goal for patients being transferred for primary PCI is as soon as possible and � 1 20 minutes.
fSTEMI patients presenting to a hospital without PCI capability and who cannot be transferred to a PCI center and undergo PO within 1 20 minutes of first medical contact (·door-to-balloon time·) should be treated with thrombolytic therapy
within 30 minutes of hospital presentation (•door-to-needle time·) as a systems goal unless thrombolytic therapy is contraindicated.
O"Gara PT, Kushner FG, Ascheim DD, et al; American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. 2013 ACCF/AHA guideline for the management of ST·elevation myocardial infarction: a
repon of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Circulation. 201 3 Jan 29;127(4):e362·425. Erratum in: Circulation. 2013 Dec 24;1 28(25):e481. (PMID: 23247304)
21
Coro n a ry Artery Disease
CJ symptoms to presentation. the presence of high -risk features. TAB LE 1 0. Contraindications to Thrombolytic Therapy for
and relative or absolute contraindications to thrombolytic ther ST-Elevation Myocardial Infarction
CONT.
apy (Table 10) . The preferred method of reperfusion is pri mary Absolute Contraindications
PC!, especially for patients presenting to hospitals with onsite
Any previous intracerebral hemorrhage
PC! faci lities. Because many patients with STE M ! present Lo
hospitals without onsite PCI faci lities, a t reatment algorithm is Known cerebrova scu lar lesion (e.g., a rteriovenous
ma lformation)
typically in place to emergen t ly transfer pat ients to a PCI
capable facil ity or admin ister full-dose thrombolytic therapy. lschemic stroke within 3 months
efficacy of transfer for primary PC! versus thrombolytic ther H i story of ischemic stroke (>3 months), dementia, o r known
i ntracra nial pathology
apy: however. observational studies have reported that patients
oflen experience delays in t ransfer for primary PCI that exceed Tra umatic or prolonged (> 1 0 m i n utes) CPR or major surgery
(<3 weeks)
l hour. When PC! cannot be readi ly ach ieved within 120 min
utes. thrornbolytic therapy is recommended i n t hose patients Recent (within 2-4 weeks) internal bleed i ng
without contraindications. Regional and national efforts have Noncompressible vascu lar puncture site
improved t hese t ransfer procedures to include the abil i ty or For streptokinase/a nistreplase: previous exposure (>5 days) o r
emergency medical services (EMS) to perform ECG in the field. previous al lergic reaction t o these agents
rhage. are rare. t hey carry an extremely h jgh mortal i ty rate •Thrombolytic therapy can be considered if SBP can be reduced to < 1 40 mm Hg
and DBP to <90 mm Hg with initial medical therapy.
(50%-60%) . Patients treated with t h rombolytic t herapy should
bTJMI flow grade 213 refers to mildly impaired flow through the coronary artery involved in the myocardial i nfarction. The higher the percentage of TIMI 213 flow, the more effective
the thrombolytic agent.
Adapted from Boden WE, Eagle K. Granger CB. Reperfusion strategies i n acute ST-segment elevation myocardial infarction: a comprehensive review of contemporary manage
ment options. J Am Coll Cardiel. 2007;50( 1 0):91 7·929. IPMID: 1 77651 1 7]
22
Coro n a ry Artery D isease
f1FR be closely observed c l i n ically fo r symptom resol u t ion and rep agents include clopidogrel. t icagrelor. a n d prasugrel. Clopidogrel
W erfusion arrhythm ias. especially an accelerated iclioventricu l a r has been the mosl widely studied. and its use with concomitant
CONT.
rhythm (AIVR) : A f V R is consiclerecl a benign rhythm when it t h rombolytic therapy and primary PC! is associated w i t h
occurs within 24 hours of reperfusion. A repeat ECG should be i mproved outcomes a n d no apparent increase in the r i s k of
obtai ned 60 m i nutes a fter thrombolysis to determine i f bleeding. For patients for whom primary PC! for treatment of
ST-segment resolutio n h a s occurred . STEM! is planned, both ticagrelor and prasugrel demonstrated
Thrombolytic failure occurs in approximately 30% of' superior e fficacy when compared with clopidogre l ; however,
patients. and typically presents with fai l u re to fu l ly resolve very tew patients in these studies were t reated with t h rornbo
chest pain or improve ST- segment elevation by 50%; some lytic therapy, and l i ttle evidence exists to recommend t he use of
patients may show hemoclynamic instabil ity or ventricular either ticagrelor or prasugrel in patients receiving thrombolytic
arrhyt h m ias. I n these circu mstances, rescue PC! is inclicatecl . t herapy. Dual antiplatelet therapy should be continued in STEM!
Rescue PCI is associated with i mproved cardiovascular out patients for a fu l l year, regardless of intervention or stent used;
comes when compared w i t h conservative medical t herapy in however, i f dual antiplatelet t herapy cannot be maintained for a
patients with fa ilure o f t hrombolytic therapy. Faci litated PC! is fu l l year (for example, because of bleeding, need for surgery. or
t h e administrat ion of fu l l - or h a l f-close t h rombolytic therapy problems with adherence) and the patient has a bare metal stent
(with or without a glycoprotein l l b/l l la i n hi bitor) followed by implanted. a minimum of 4 weeks of dual antiplatelet therapy
pla nned. immediate PCI. Adverse events (especi a l ly bleedi ng) is advised.
have l i m ited the safety and overa l l use of fac i l itated PCI . P l atelet glycoprotein l ib/Il la i n h i b itors (abciximab,
tirofiban. eptifibatide) further i nh i b i t platelet aggregation and
Medical Therapy impair platelet activation. They are useful i n patients with
At the t i me of initial presentation. a l l patients w i t h STEM I STE M ! who undergo prima1y PC] ; however. the use of glyco
should be given a 325 - mg loading close of aspirin. supplemen protein l i b / I l l a inhibitors should be reserved for adm ir1istra
tal oxygen . t herapy to improve symptoms (nitrates. analge tion in the catheterization laboratory rather t h a n u p- fro n t in
sics) , therapy to reduce i n farct size ( � - b l ockers, ACE t he emergency department owing to the i ncreased risk of
inh ibitors) . and an tit hrombotic therapy (anti platelet agen ts. bleeding and no clear benefit when administered prior to pri
ant icoagu l a nts) . Aspirin should be administered immediately; mary PCI. Rou t i ne glycoprotein l l b / l l la i n h i bitor use i n
however, the admi nistration of ot her agents should not delay patients who receive thrombolytic treatment without P C I i s
t h e plan to reperfuse t he i n farct-related artery. controversial ancl n o t currently recom mended.
In many patients wi th STEM!, control of' chest pain can be The choice of anticoagu lant for treatment of STEM! is
achieved with sublingual or intravenous ni trates. Morphine and dependent on t he reperfusion st rategy ava ilable for the patient.
other opioid analgesics are also effective for reducing chest pain U n fractionated heparin (UFH) has been t horoughly studied i n
by decreasing the body's sympat hetic response to STEM ! . patients receiving t h rombolytic agents. a n d its use i s associ
Caution should b e used i n patients with inferior STEM! and evi ated w i t h a reduced incidence of reocclusion of the i n farct
dence of right ventricular infarction because nitrates and anal related artery. Low-molecu lar-weight heparin (LMWH) has
gesics can lead to reduced preload and significant hypotension. also been associated with i mproved outcomes in patients who
In the treatment of STEM ! , �-blockers are recommended receive thrnmbolytic t herapy. In patients undergoing primary
at the time of initial presentation except in patients with evi PCI. the use of U F H is favored over LMWH owing to the abil i ty
dence of heart fai l u re, hypotension. bradycardia, advanced to monitor t h e degree of anticoagu lation (by measurement of
atrioventricul a r block. or o t her contraindications to � activated clotting t i mes) . When a heparin -based strategy is
b lockers. I n t ravenous metoprolol is t he most widely used u t i l i zed for pri mary PC!. guidelines recommend the concomi
� - b locker for STEM ! trea t men t : it is dosed in 5 -rng increments tant administration of a glycoprotein l i b / I l la inhibitor. Recent
every 5 minutes. for a total dose of 15 mg. Fol l owing reperfu studies of bivalirudin. a direct t h rombi.Ji i n h ibitor. have shown
sion, an oral �-b l ocker is recommended to reduce myocard ial that its use at the t i me of primary PCI is associated with a
oxygen demand and reduce mortality. similar rate of ischemic events (death, myocardial infarction.
ACE i11hibitors should be admin istered after reperfusion stroke. stent t h rombosis) and fewer b l eeding events when
in all patients w i t hout contraindications (systolic blood pres compared with a heparin p l us glycoprotein J i b / I l la i n h i b itor.
sure <90 mm Hg, advanced k i dney dysfu nc t i o n , hyper In general. t h erapies that reduce bleeding complications may
kalemia) . Angiotensin receptor blockers may be substituted i n improve survival but with concern for greater risk of nonfatal
patients w h o are a l l ergic or i n tolerant to ACE in h ibitors. ischemic events. such as early stent t h rombosis.
The use of antiplatelet agents in the setti11g of STEM ! has In patients with diabetes mell itus who present with
cha nged over the past decade with the ava i l ab i l i ty of several STEM ! , plasma glucose levels should be maintained below
new agents. Aspirin remains a mainstay in the treatment of ACS 1 80 mg/ c! L ( 1 0 . 0 mmol / L) w h i l e avoiding hypoglycem i a .
and should be administered to a l l patients unless a l lergic or I ntravenous insulin has been tested in m u l tiple trials, b u t
intolerant. Platelet P2Y receptor i n h i b i tors impair platelet n e i t h e r i n t ravenous insu l i n nor glucose- insul i n- potassium
12
aggregation, and t h is effect is additive to aspiri n. Available infusions are recommended curren t ly.
23
Co ro n a ry Artery Dise a se
Right ventricular Hypotension, j u g u l a r > 1 m m ST-segment Dilated right ventricle Elevated right atria l
i nfarction venous distention, clear elevation in leads V3 R with reduced systolic and right ventricular
lung fields and V4 R function pressures, low wedg e
pressure
Extensive left ventricular Systolic blood pressure Extensive ST-segment Severe left ventricular Cl <2.0 Umin/m2,
i nfarcti on <90 m m Hg elevation, usua l ly in systolic dysfunction wedge pressure
a nterior leads > 1 8 m m Hg
Ventricular septa I defect Holosysto l ic murmur Nonspecific; H ig h-velocity left-to- Pro m inent, large v
along left sternal border, a pproximately 50% right systol i c jet within waves in wedge
often with thri l l o f ventricular septal ventricular septum, pressure tracing; step-
defects occur in anterior systol i c turbulence on u p i n oxygen saturation
wall M l right ventricle s i d e of from right atri u m to
ventricular septum right ventricle
Pa pillary muscle rupture Holosystolic murmur at Usua l ly associated with Flail mitral valve leaflet Prominent, large v
left sternal border and inferior and inferior- with attached mass waves i n wedge
apex, may radiate to poste rior wall M l ( pa p i l l a ry m uscle pressure tracing
axil lae; pulmonary hea d ), severe mitral
edema regurg itation
Left ventricular free wall Hypotension, j u g u lar Nonspecific; pulseless Diffuse or localized Eq ualization of diastolic
ru pture venous distention, electrical a ctivity pericardia! effusion pressures, C l <2.0 U
distant heart sounds with tamponade; min/m2
d iscrete wall motion
a bnormality; defect in
myocard i u m may be
seen
24
Coron a ry Artery D isease
Cl
gical mortal i ty is h igh . i npatient 111ort a l i ty for p<l l ie n t s Non-ST-Elevation Acute Coronary Syndromes
who d o not undergo surgery i s nearly I O O'Y. . Percuta neous
. The 111ost common pat hophysiology of' NSTE-ACS is nonoc
ven t ri c u l a r septa! defect closu re devices a re so111 e t i 111es clusive coronary atherosclerosis with or without t hrombus
u sed in nonsurgical patients. but t he i r use is l i m ited by formation . The t rearment o l' UA and NSTEM I patients is
a nato111y a n d operator expert i se. focused on improvement i n epicardial blood flow w i t h medi
M i t ra ! regurgit a t i o n occurs co111 m o n ly a f'ler STE M ! . cations and revascu larization . Because the l i n k between revas
M e ch a n isms i nclude severe lef'l ven t ricular dysfu n c t ion cuiarizat ion a nd clin ical outcomes is less clear than i n STE M !
with a n n u l us d i latation. worsening of pre-exist i ng 111 i t ral patient's. NSTE-ACS patients should undergo r i s k stra t i fication
regu rgi tati o n . a n d comprom ise o f t h e m i t ra l apparatus prior to invasive t rea l menl.
( ru p t u re o f pap i l l a ry 111uscle or chordae l e n d i neae) .
Papil lary muscle rupture o f'ten presen t s 3 to 7 clays a fter Risk Stratification
i n i t ia l myocardi a l i n fa rction with hemodyna111ic compro The TI M I risk score is t he most com mo n ly used rool for
m i se. p u i 111onary edema . a n d a loud systolic 111 u r m u r. estimating t he s hort- term risk for dea t h a n d n o n fatal myo
D iagnosis is 111ost of'len made by t ransthoracic echocarci iog card i a l i n fa rction i n pa t i ents w i t h a N STE-ACS (Tab l e 13) .
ra phy. a n d t ra nsesophagea l echocarci i ogra p hy 111ay be The T I M I risk score is most usefu l to assist in dec i d i ng
requ i red to plan surgical reconstruct ion . Trea t ment consists whether p a t i e n t s w i l l benefi t rrom a n early i nvasive
o f the a d m i n is t n.J t io n o f vasod i l ators to reduce a fterload and
d iuretics to decrease prel oad . If patients become hemociy
namicaliy co111pro111 ised . t h e a d m i n i s t ration of vasopres
sors. placement of an i n t ra -aort i c bal l oon pump. a n d /o r
s u rgical i nterven t io n a re req u i red .
Left ven t ricular free wall rupture is the most ominous
mechanica l co111plication of STEM I and has a h igh 111ortality
rate. It often occurs 3 to 7 days after i n i t ial 111yocarciial i n farc
t ion. Risk factors !or left ven t ricular rupture i nclude advanced
age, fe111ale sex. anterior myocard ial infarction . and i nco111-
plete reperfusion of STEM ! . Pat ients most co111111only present
with pericard ia! tamponade (due to hemopericard i u m) . pulse
less electrical activity. and death. Early recogni t ion. emergent
pericardiocen tesis. and subsequent surgical reconstruction
can improve survival.
Left ventricular t h rombus occurs in approximately L O 'Y..
lo 20% of pa t i en ts a fter a n terior 111yoca rcl ic1 I i n fa rct ion F I G U R E 9 . Echoca rd iographic image showing a left ventricular thrombus in a
despite reperfusion a n d aggressive t rea t m e n t . Transt horacic patient with a recent myocardial infa rction. LV = left ventricle; RV = right ventricle.
25
Coro n a ry Artery Disease
TABLE 1 3. TIMI Risk Score for Non-ST-Elevation Acute D u a l antiplatelet t herapy (aspirin p l u s clopidogrel, pras
Coronary Syndromes ugrel. or t icagrelor) is recom mended in all patients w i t h
Prognostic Variables NSTE-ACS. regardless of T I M I risk score. u n less an increased
risk or bleedi ng exists (see Table 9) . The use of clopidogrel. in
( 1 ) Age �65 years addition to aspi rin, is t h e best-studied combination .
(2) �3 Trad itional CAD risk factors• Clopidogrel should be given as a loadi ng dose (300 mg or
(3) Documented CAD with �50% d i a meter stenosis 600 mg) at hospi tal adm ission and ad m i nistered as a 75-mg
(4) ST-segment deviation daily dose for at least l year regard less of t he need for PC! or
CA BG. Pat ients with a bare metal sLent who cannot tolerate
(5) �2 Angina! episodes in the past 24 hours
dual ant iplatelet t herapy for t h e fu l l year ( for example.
(6) Aspirin use in the past week
because of bleeding. need for su rgery. or problems w i t h
(7) Elevated cardiac biomarkers ( creatine kinase MB or troponin)
adherence) should remain on the t herapy for at least 4 weeks.
TIMI Risk Score (Sum of Prognostic Variables) l f CABG is u l t i mately required. clopiclogrel should be d iscon
tinued and CABG should be postponed for 5 to 7 clays in order
0-2 Low risk
to avoid perioperative bleedi ng.
3-4 Intermed iate risk
Two oral P2Y 12 i n h ib i tors . prasugrel and t icagrelor. have
5-7 High risk been developed, a n d when tested . were superior to clopi
CAD = coronary artery disease. clogrel in UA and NSTEl'vl l pat ients. Ticagrelor and prnsugrel
.iHypertension, hypercholesterolemia, diabetes mellitus, being a current smoker, do not require hepati c m etabolism. are more poten t . and
family history of CAD.
have a faster onset of action when compared with clop i
Adapted from Antman EM, Cohen M, Bernink PJ, et al. The TIMI risk score for dogrel. These agents also should be discont inued 5 t o 7 days
unstable angina/non-ST elevation Ml: a method for prognostication and therapeu-
tic decision making. JAMA. 2000;284(7):835-842. (PMID: 1 0938 1 721 or more prior to CABG.
Ad m i n istration of glycoprotein l ib/ I I la i n h i b i tors in
patients with NSTE-ACS does not appear to be of net clin ical
benefi t un less h igh-risk features. such as ongoing angina or
l'PI t rea t rnenl strategy (Figure 10) . The est imated rates of death evidence of ischemia after the i n i t iation of sta nda rel an ti plate
LI.I
CONT.
and non fa Lal myocardial i n farction also a re useful lo cou nsel let and antianginal medications. rein farction . or heart failure.
palients rega rd ing their risk. ln patients at low risk (Tl M I a re present. However. L hese agents, in combinalion wi l h U FI-1
score o f 0 - 2 ) , practice guide l i nes recom mend a n ischem i a or bivalirudin, are indicated at the time of PC! in pat ients with
gu idecl st rategy t ha t u t i l izes invasive t reatment only i f medi NSTE-ACS who u l t imately require revascularizal ion . Because
cal t herapy is ineffec t ive. Patients a t h igher risk (TI M I score of t heir potent antiplatelet activity, t he main adverse effe ct of'
�3) are more l i kely to benefi t f'rom an early i nvasive approach. glycoprotein l l b/l l la i n h ibitors is i ncreased risk of major a nd
m i nor bleeding events.
Medical Therapy
A l l patients who present with ischemic chest pain should be A 11 t icoagu la n t Medica t i o n s
t reated i ni t i a l ly w i t h asp i r i n , �-blockers, and n i t rates. The use of ant icoagulant medications ( U FI-1. LMWH . foncla
However. compared w i t h STE M ! . in which reperfusion is the parinux, and bivalirudin) has been a cornerstone of t herapy
primary goal of therapy, once the diagnosis of UA or NSTEM I for NSTE-ACSs for more than t h ree decades. The choice of a
has been established ( through the ECG and biomarkers). risk panicular agent is based on t he patient"s bleeding risk. TI M I
stra t i fication can be used to guide t he clinical use of additional risk score. comorbid conditions (such as chronic kidney d is
t herapies (Table 14 . on pages 28-29) . All NSTE-ACS patients ease ) . plan for an early invasive versus a conservat ive st rategy.
should receive a stati n and a P2Y 12 i n h ibitor (such as clopi timing of coronary angiography. and physician preference.
dogrel ) . I n patients at i n termediate or h igh risk (TI M I score U F H and L M W H are the most widely used anticoagu
�3) . additional t herapies. such as anticoagu lant agents or a lants for NSTE-ACSs. In patients in whom an early i nvasive
glycoprotein l ib/ I l la inhibitor, should be considered. approach is planned and i n patients vvi t h chronic kid ney
d isease. U FH is preferred over L M W H . I n patients in whom a
A n t ipla te let Medica t io n s conservative strategy is p la n ned . bot h L M W H and fonda
The i n itial aspirin dose should be 325 mg at t h e t i me of pres parinux have been proved w be safe and effect ive. Advantages
entat ion for ischem ic chest pain . Patients who are al lergic to of fondapari nux and LMWI-1 i nclude the abil ity to close once
aspirin should be administered clopidogrel at the time of pres or twice daily rather than con t i nuously and no req u i rement
entation. Alt hough t here remains debate about subsequent to monitor t herapeutic levels. Because of a sign i fi c a n t ly
aspirin closing based on patient risk and whether revasculari i ncreased bleedi ng risk. the use of the a n ticoagu lant bivaliru
zation with PC! or CABG occurs. most patients can be treated din is curre n t ly n o t recom mended by clinical guidelines
with a dose of 81 mg daily inde fi n i tely (especially when dual other than d u ri ng PC! or in patients who a re allergic to
antiplatelet t herapy is being used) . hepari n-based produ cts.
26
Coro n a ry Artery Disease
N on-ST-elevation acute
coronary syndrome
(UA/NST E M I )
1
I n iti ate aspirin, B-blocker, n itrates, stati n
I I
... '
F I G U R E 1 0 . I n itial management of n on-ST-elevation acute coronary syndromes. G P = glycoprotei n ; LMWH = low-molecular-weight heparin; LVEF = left ventricular
ejection fraction; NSTEMI = non-ST-elevation myocard ial infarction; UA = unstable angi na; U FH = unfractionated heparin.
aclopidogrel o r ticagrelor m a y b e dosed at t h e time of hospital admission a n d acute coronary syndrome diagnosis.
'If coronary artery bypass grafting is required, clopidogrel or ticagrelor should be stopped and surgery delayed for at least 5 days.
elf the decision is made to withhold a P2Y1 2 inhibitor until time of angiography and a P2Y 12 inhibitor is desired, clopidogrel, ticagrelor, or prasugrel can be initiated.
Recommendations based on Amsterdam EA, Wenger NK, Brindis RG, et al; ACC/AHA lask Force Members. 2014 AHAJACC guideline for the management of patients with non-ST-elevation acute coronary syndromes: executive summary: a
report of the American College al Cardiology/American Heart Association Task force on Practice Guidelines. Circulation. 2014 Dec 23;1 30(25):2354-94. IPMID: 25249586]
CJ A n tia ngi n a / Med ications t ions. I n travenous n i t rates are ty pic a l ly used in patients with
Un less con tra i nd i cated. o ra l � - b l o cke rs should be i n i tiated i n ongoing chest pai n or borderline blood pressure or hemody
CONT.
a l l patients who present w i t h a N STE-ACS . I n travenous narn ics. In patients without act ive symptoms. topical and oral
P-blockers can also be adm i n istered; however, caution should formulations should be prescribed in order to prevent recu r
be used in patients with heart fa i l u re . advanced age. advanced rent symptoms. Subl i ngual n i t roglyce rin is often p res c ri b e d on
atrioventricular block, and hypotension . an as- needed basis for new or worse n i ng angina_ All forms of
N i t rates that can be a d m i nistered to NSTE-ACS patients ni t rntes should be avoided in patients who have t a ke n a PDE- 5
include i n t rave n o u s . topica l . o ra l , and sublingual formula- i n h ibitor (such as silclen<Jfi l or varclenaf11) w i t h i n 24 hours.
27
Coro n a ry Artery Disease
'
Cardioprotective Medications
�- B l ockers Atenolol, metoprolol, Vari a b l e All patients with prior M l or Caution is advised i n
carvedilol, nebivolol LV systolic dysfunction patients with significant
obstructive lung disease
(e.g., COPD) or advanced
atrioventricu lar block
ACE i n h i bitors Benazepril, captopri l , Va riable All patients with LV systo lic
enal a pril, fosino pril, dysfu ncti on, hypertension,
peri ndopril, tra ndola pril, dia betes, or proteinuric
l i sinopril, ra mipril, chronic kid ney disease
q u i n a pril
Angiotensin receptor Losartan, valsartan, Varia b l e ACE i n h i b itor-intolerant The use of ang iotensin
blockers o l mesartan, candesartan, patients receptor blockers in
irbesartan, tel m isartan combination with ACE
i n h i b itors is not well
esta b l ished (class l i b
recommendation)
Aldosterone blockade Spironolactone, Varia ble ACS patients with LV Caution is advised i n
eplerenone systo l i c dysfu nction on patients with sign ificant
therapeutic doses of kidney dysfu nction or
�-bl ockers and ACE hyperka lemia
i n h i b itors
28
Coro n a ry Artery Disease
P-blockers Atenolol, metoprolol, Va riable All patients with prior M l or Caution is advised i n
carved ilol, nebivo l o l LV systolic dysfunction patients with s i g n ificant
obstructive l ung
d i sease o r advanced
atrioventricular b lock
Long-acting nitrates lsosorbide, transdermal Va riable Useful for rel i ef of N itrates are
patch, ointment symptoms when maximal, contraindi cated i n
tolerated dose of patients taking PDE-5
P-blocker has been i n h i bitors for erectile
achievedb dysfu nction
Short-acting nitrates S u bling ual, spray, o r Variable Useful for relief of N itrates are
aerosol symptoms, usually contra indicated i n
prescribed on a n patients ta king PDE-5
as-needed basis i n h i b itors for erectile
dysfunction
Ca lcium channel Amlod i pine, d i ltiazem, Va riable Useful for relief of
blockers vera pa m i l , nifed i pine, symptoms when maximal,
nicard i pine tolerated dose of
P-blocker has been
ach ievedb
ACS = acute coronary syndrome; BID = twice daily; LV = left ventricular; M l = myocardial infarction; N/A = not applicable; PCI = percutaneous coronary intervention;
PDE-5 = phosphodiesterase S; TIA = transient ischemic attack.
l"l"'I Calcium channel blockers and ranolazine do not have considered for early invasive treat ment. Patients treated w i t h
LI.I clear i nd ications for use i n pat ients presenting with an ACS. an ischemia-gu i ded strategy should undergo noninvasive
CONT.
Opioids, such as morph i ne. may be administered for sympto- stress testing before hospital discharge; coronary angiography
matic relief o f pat ients w i t h ongoing chest pai n. although cau and subsequent PCI or CABG should be reserved for t hose
tion is advised because of their blood pressure-loweri ng with recurrent symptoms or h igh-risk features on stress test
effects. i ng. H igh - risk patients assigned to an early i nvasive strategy
(coronary angiography and subsequent PCI or CABG within
L ipid-Lowering Medica tions 24 hours of i n i t ial hospitalization) have been shown to have
The benefits of statin therapy i n ACS patients a re wel l estab i mproved outcomes when compared w i t h high -risk patients
l ished; however, the timing or i nitiation (hospital adm ission treated conservatively. For patients who are appropriate for an
versus at the t i me of· PCl versus hospital d ischarge) is less clear. early i nvasive strategy. t here is no evidence t hat very early
Recent studies suggest t hat h igh-intensity dosing of atorvasta angiography (<6 hours or at hospital admission) offers incre
l" i n or rosuvastat i n is associated with i mproved 2-year survival mental benefit.
after ACS. Current treatment guideli nes recom mend the i n i Clinical guideli nes recom mend the use of early i nvasive
t ia tion of a h igh- i ntensity statin in patients at very high car treatment i n NSTE-ACS patients regardless or risk stra t i fica
d iovascular risk, i ncluding t hose patients w i t h an ACS. tion who have recurrent symptoms. hemodynamic i nstability,
or elect rical i nstabi l i ty.
Invasive Versus Ischemia-Guided Treatment CABG is often recommended tor ACS patients w i t h spe
Once medical t herapy has been i n i t iated and risk stra t i ficat ion c i fic angiographic criteria ( that is. le!'t main disease and multi
has occurred, guideli nes recom mend that pa tients with a low vessel CAD w i t h or w i t hout proximal left anterior descending
risk N STE-ACS be treated conservatively with an ischemia stenosis) with concomitant lert ventricular systolic dysfu nc
guided strategy and that intermedi ate- to h igh- risk patients be tion and /or d iabetes mel l i tus. Cl
29
Coro n a ry Artery Disease
c:J Acute Coronary Syndromes Not Associated ment with a �-blocker for at least 3 years following an ACS,
although many clinicians choose to continue these medica
with Obstructive Coronary Disease
tions indefinitely if they are well tolerated.
A l though plaque rupture al the s i te or al herosclerolic p laque Current guidelines recommend that patients who are
depos i t ion is t he most com mon cause or ACS. ot her d isease entering cardiac rehabilitation programs after ACS should
e n t i t ies may cause patients to presen t w i t h chest pa i n . tran undergo routine exercise ECG testing. Routine stress testing is
sient ECG changes. and elevated cardiac biomarkers. The diag not currently recommended for asymptomatic patients who
nosis of t hese d i seases is genera l ly made once diagnos t ic are not entering a cardiac rehabilitation program. For patients
coronary a ngiography has confirmed t he absence or obstruc with an ACS who are symptomatic after hospital discharge, the
t ive coronary stenoses.
use of stress testing is acceptable and should be determined by
Coronary \·asospasm of'ten occurs in normal or nea r
the patient's symptoms (for example, unstable angina, stable
normal coronary arteries. and spasm can be t riggered by the angina) , ability to exercise, and interpretability of ECG.
use of i l l icit drugs. such as coca ine or met hampheta rn i ne. In
patients who presenr with ischern ic chest pa i n at rest and KEY POINT
t ransient ST-segment elevat ion or depression. t he d iagnosis of • Routine stress testing is not currently recommended for HVC
vasospasm is often one of exclusion once angiography con asymptomatic patients following an acute coronary
firms t h e absence or obst ruc t ive CAD. Vasospasm is most fre syndrome who are not entering a cardiac rehabilitation
quen t ly t reated w i t h long-term n i t rates and calcium channel program.
b lockers and avoidance of t riggers such as i l l ic i t drugs.
Takotsubo cardiomyopathy. onen temporally associated
w i t h a stressful event. can m i m i c an ACS with t he presence of M a n a g ement of Coronary
chest pa i n . ECG changes. and ele·ated cardiac biomarkers (see
Artery Disease i n Wo men
Heart Fai lure) . H owever. co ro na ry angiography is usu a l ly nor
mal or shows o n ly m i n i mal a therosclero l ic d isease. and left Clinical Presentation
ven tricu lography is classical ly defined by t he presence of mid Women typically develop ischemic heart disease at a later age
wa ll <1l1d apical wa l l motion abnorma l i t ies w i t h sparing of the in life than men. Unlike men, women present more frequently
basal segments. Treat ment is support ive. and more than 95% with stable angina pectoris rather than an ACS or sudden car
of pat ients have resol u tion of symptoms and recovery of l e ft diac death. More than 50% of women who present with typical
ventricular function within 7 days. angina are diagnosed with nonobstructive coronary stenoses,
Owi ng to the i mproved sensitivity of troponin assays. and the presence of microvascular disease is thought to be
other systemic d iseases (such as chronic kidney d isease. sepsis. significantly higher in women than in men.
30
Coro n a ry Artery Disease
------- ----- ---·------
In women presenting wilh acute myocardial infarction. significantly more frequently in persons with diabetes com
chest pain remains the predom inant symptom; however. the pared with those without the disease. The diagnostic accuracy
Likelihood of atypical symptoms . such as fatigue, dyspnea. of noninvasive stress testing in symptomatic patients with dia
nausea. and abdominal complai nts. is significantly h igher betes is similar to that in patients without diabetes; however, the
than in men . In women wi thout sign i ficant obstruction on assessment of CAD in asymptomatic persons with diabetes is
invasive angiography. microvascular dysfunction (either controversial. Currently, the American Heart Association rec
endothelium-dependent or endothelium-independent) is ommends stress testing in patients with diabetes who are
t hought to be the cause of symptoms. Cl (1) symptomatic, (2) initiating an exercise program, or (3) known
to have CAD and have not had a recent (>2 years) stress test.
Evaluation and Treatment
l:::J
The sensitivity and specificity of noninvasive stress testing for I nvasive Treatment
the evaluation of chest pain are lower in women than in men. Jn patients with dh:1betes who are candidates for revasculariza-
ST-segment deviation is less accurate in women than in men. tion. the decision to pursue PC! or CABG remains controversial
Clinical guidelines report an improved diagnostic test accu and depends on a mul titude of factors, i ncluding severity and
racy with the use of stress testing with imaging (see Diagnostic extent of CAD. the presence of comorbid conditions, and the
Testing in Cardiology) . However, no specific diagnostic evalu degree of atherosclerotic narrowing of' smal l . distal vessels.
ation guidelines exist for women, and the same guidelines Multiple studies have analyzed outcomes of patients with diabe-
apply to men and women. tes undergoing PC! or CABG. Although CABG is general ly asso
In the COURAGE trial, women with stable angina pectoris ciated with fewer repeat revascularization procedures, mortality
had reduced overall mortality or nonfatal myocardial infarction is similar between the t\.vo procedures. When a decision is made
with revascularization therapy as compared with men. However, to pursue PC!. the use of a drug-eluting stent is recommended
women have a higher complication rate, particularly bleeding and to reduce the occurrence of target vessel revascularization
vascular complications, surrounding revascularization proce because of the more extensive coronary a11ery disease and
dures. For these reasons, it is recommended that guideline higher rate of' restenosis in patients with diabetes. CJ
directed medical therapy be initiated in women prior to
consideration for revascularization. In women undergoing PCI, a
Medical Therapy and Secondary Prevention
trend toward fewer such complications has been found with the
Aggressive risk factor reduction, control of plasma glucose
use of radial rather than femoral arterial access. Overall, treatment
levels, and medical therapy are essential in patients with dia
guidelines do not currently differ between men and women. The
betes. In most patients with diabetes and CAD, high-intensity
use of estrogen is not recommended to reduce the occurrence of
statin therapy and antihypertensive treatment with a target
future cardiovascular events in post-menopausal women.
blood pressure below 140 /90 mm Hg are recommended.
K EY P O I NTS Because of the protective renal effects of ACE inhibitors and
• In women presenting with acute myocardial infarction, angiotensin receptor blockers in patients with proteinuric
chest pain remains the predominant symptom; however, nephropathy, these agents are preferred over other antihyper
atypical symptoms, such as fatigue, dyspnea, nausea, tensive agents, such as thiazide diuretics.
and abdominal complaints, are more likely than in men. Common medications used for the treatment of diabetes
are of special concern in patients with CAD. In a meta-analysis,
• In women with coronary artery disease, it is recom
thiazolidinediones, specifically rosiglitazone, had been asso
mended that guideline-directed medical therapy be
ciated with an elevated risk of cardiovascular events, espe
initiated prior to consideration for revascularization;
cially myocardial ischemia. However, a more recent clinical
otherwise, treatment guidelines do not currently differ
trial demonstrated no elevated risk of myocardial infarction
between men and women.
or death in patients being treated with rosiglitazone when
compared with standard-of-care diabetes drugs. The use of
M a n ag ement of Coro n a ry metformin at the time of coronary angiography, after myo
cardial infarction, and in patients with heart failure should
Artery Disease i n Patients
be avoided because of a rare but potentially fatal risk of lactic
with Diabetes M e l l itus acidosis.
Risk and Evaluation KEY POINT
Patients with diabetes mellitus are at increased risk of develop
• In patients with diabetes, stress testing is recommended
ing CAD, and cardiovascular mortality is significantly higher in
by the American Heart Association for those who are
this population. Additionally, patients with diabetes mellitus
(1) symptomatic, (2) initiating an exercise program, or
often do not experience classic angina pectoris and can present
(3) known to have coronary artery disease and have not
with atypical cardiac symptoms, such as dyspnea, nausea, or
had a recent (>2 years) stress test.
hyperglycemic symptoms. Sudden cardiac death occurs
31
H e a rt Fa i l ure
Cl
myocardial contraction. Reduced systolic function results in Diagnosis
progressive ventricular dilation. In contrast, patients with I n p at ie n ts who presen t with acute dyspnea of undetermined
HFpEF have similar symptoms but normal systolic contraction e ti ol ogy B-type natri uret i c peptide (BNP) levels can be used to
.
and an abnormality in diastolic relaxation. This results in quickly d ifferentiate between dyspnea se con da ry to heart fai l
restricted filling and high filling pressures. To maintain a nor u re (elevated B N P) a n d dysp nea related to pulmonary disease
mal cardiac output (heart rate x stroke volume), patients with ( low to normal B N P) . The B rea th i ng Not Properly stud y evalu
HFpEF tend to have a higher heart rate. Clinically, because ated patients who presented to t he e m ergen cy de p a r t m e n t
these patients have a very small left ventricular size, they are with dyspnea. Patients who had heart fai l u re had a mean BNP
usually much more sensitive to volume loading than patients level greater than 600 pg/ml (600 ng/ L) whereas those with
,
32
Heart Fa i l u re
i ty of t he disease process as wel l as prognosis. Palierns with Eva luation for lschemia
acute heart failure syndromes and a di lated left ventricle likely Alt hough coronary artery d isease is the most com mon cause
have a chronic disease process with delayed onset or recogni of heart failure, owing lo expense and radiation exposure, the
t ion of symptoms. A small left ventricle (particularly without routine i nvestigation for coronary disease by stress testing or
wall thin ning) is associated with a grea ter chance of recovery cardiac catheterization or other i maging modalities (such as
of' ejection fraction compared with a markedly dilated left C M R i maging. P ET, or CT) is no longer considered part of the
33
H e a rt Fa i l u re
Cl rou t i ne evaluation or all patients with newly d iagnosed heart TABLE 1 5. New York Heart Association (NYHA)
fa i lure. Card iac catheterizat i on should be performed i n Functional Class
CONT. ,
patients presen t i ng with angina or sign i ricant ischernia. Class Description
Addit ional ly. cardiac cat heteri za t ion is recom mended !or
patients presenting with chest pai n t ha t may or may not be ol' No l i m itations of physical activity
cardiac origin a nd t hose with previous ly d iagnosed coronary S l ig ht l i mitation of physical activity
artery d isease w i thout chest pain if they are e l igible !or revas Ill M a rked l i m itation of physical activity
culariza tion. Non i nvasive stress test i ng i s reasonable i n lllA Symptoms with less than ordi nary activity
pat ients with a history or coronary artery disease L o evaluate
lllB Symptoms with minimal exertion
for reversible ischernia. as revascula riza t ion can dramatica lly
IV U n a ble to carry on any physical activity without
improve lert ventricular funct ion . Add i t iona l ly. patients with symptoms
m u ltiple risk !'actors for coronary disease should undergo non
invasive testing to eva luate for signs of i schemia. I r st ress test
i ng identi fies signi fican t ischemic myocard i u m , coronary
TABLE 1 6. Medical Therapy for Heart Failure with
a ngiography shoul d be consi dered . Cl Reduced Ejection Fraction
KEY POINTS , Therapies that Decrease Mortality
• B-type natriuretic peptide levels can be useful to distin ACE i n h i bitors/a ng iotensin receptor blockers
guish cardiac from noncardiac causes of dyspnea in the
�-Bl ockers
urgent care setting.
Aldoste rone antagonists (if NYHA class II to IV)
HVC • In patients with heart failure, with the exception of thy-
Hydralazine/isosorbide d i n itrate (b lack patients with NYHA
roid disease, an extensive evaluation of µnusual causes class 1 1 1/IV symptoms)
of heart failure should not be performed unless there
Therapies that Improve Symptoms
are suggestions of specific diseases by history or physi
cal examination. Digoxin
34
H e a rt Fa i l u re
c::J t h ese patients. it is reasonable to switch to an A R B instead. Less TABLE 1 7 . Therapeutic Doses of �-Blockers for Treatment
information regarding mortality is avai lable for ARBs so they
• of Heart Failure with Reduced Ejection Fraction
OOITT
should not be used as first-line t herapy. O t her common adverse Agent Target Dosage
effects of both cl rugs include hyperkalemia and. occasionally.
worsening kidney fu nction. I n patien ts with angioedema while Carved ilol 25 mg BID (SO mg BID if >85 kg ( 1 87 lb] )
taking ACE inhibitors, ARBs should not be used as an alterna Metoprolol succi nate 200 mg daily
tive because there are reports of angioeclema also occurring Bisoprolol 1 0 mg daily
with t hese agents.
BID = twice daily.
�-Blockers
�-Blockers shoul d be started in a l l patients with H FrEF a fter
acute clecompensation is t reated and the patient is hemocly D i u retics
namically stable. These d rugs block t h e adverse effects of Diuret ics are the mainstay of t herapy for symptoms of heart
chronic neurohormonal activation on cardiac funct ion . Three fai l u re associated with vol u me overload. To avoid hypo
�-blockers have been shown to decrease mortal i ty. reduce volemia. the lowest close of diuretic necessary should be used.
heart fa ilure symptoms. and improve left ventricular ejection Loop diuretics are the most commonly used agents. I n patients
fraction in patients with H FrE F: metoprolol succinate, carve w i t h refractory heart failure. t he addition of a t hiazide diuretic
cli lol. and bisoprolol. I t is important to use one of' t hese t h ree is occasionally used to augment t he effects of t he loop diuretic.
agents because they are t he only ones t hat have a demon There is no advantage to a conti nuous intravenous i n fusion
strated benefit in patients with heart fai l u re. O t her �- blockers. versus bolus therapy in decompensatecl heart fail ure. Adverse
including short- acting metoprolol (metoprolol tartrate) . have effects of diuret ics include hypokalemia . hypomagnesemia.
not shown similar benefit. Some pat ients experience i ncreased and worsening kidney functio n. As electrolyte abnormal ities
fatigue on �- blockade. but the vast majority experience a n can lead to malignant arrhythmias, electrolytes should be fre
improvement in heart fai lu re symptoms. q u e n t ly measured i n patients receiving h igh doses.
Addi t ionally. patients should be counseled to restrict their
Initiating and Managing ACE I n h i b itor sodi u m and fluid i ntake.
and �-Blocker Therapy
Pa tients with acute heart failure a nd volume overload should Digoxin
ini tially be started on an ACE i nh ibitor. Typical ly. a short Digoxin has been used for decades for t he treatment of heart
acti ng agent such as captopril should be used in divided daily fai lure. Digoxin has not been shown to reduce mortality but
closes so t ha t if the patient experiences symptomatic hypoten does decrease hospi tal izations for H FrEF i n comparison with
sion, t he effect w i l l be transient. ACE inhibi tors shoul d be placebo. In short- term t rials. cl igox i n has been shown to
t i t rated based on blood pressure and the presence or absence i mprove heart fai lure symptoms, quality of l i fe, and exercise
of adverse effects. For patients w i t h new-onset heart failure tolerance. The withdrawal of digoxin in patients is associated
and volume overload. a �-blocker should not be initiated until with increasing heart failure symptoms.
t he patient is euvolemic or close to euvolemic. Therapy w i t h d igox i n should be c losely fol lowed . I t i s
I n contrast to ACE inhibitors. in which the close can be reasonable t o c h e c k a serum digo x i n level when a p a t i e n t i s
rapidly t i t rated upward. �-blockers should be started at a very stabl e. Pa t i e n ts w i t h kid ney impairment, low body mass.
low close once pa t ients a re euvolemic because these agents a n d o l de r age have reduced metabolism of d igox i n and can
h ave a negative i notropic effect. I nstead of up- titrat ing the q u ickly develop a toxic leve l . I t is important to check a
drug on a daily basis. t i t ra tion of a �-blocker should be per d igox i n level in patients w i t h worsen i ng kid ney fu nct io n .
formed slowly at 1 - to 2 -week i n tervals, on an outpatient basis. Ret rospect ive a n alyses h ave s h ow n t h a t serum leve l s
A number of studies have demonstrated a dose-response effect greater t ha n I ng/m L ( 1 .28 nmol / L) a re associated w i t h
with �- blockers. H igh doses compared with low closes of i ncreased r i s k o f morta l i ty, most comm o n ly re l a ted t o
�-blockers have been shown to be more beneficial for both arrhy t h m ias .
morta l i ty reduction and t he reduction of heart failure symp
toms. Although patients are often discharged on low doses. Aldosterone Antagonists
t hese agents should be up- t i t rated to t he maximal tolerated A ldosterone a n t agon ists (spironolactone. eplerenone) have
doses a fter the patient has been d ischarged (Table 17) . been studied in patients w i t h heart fai lure and N Y H A func
Limitations lo maximal up-tit ra t ion i nclude symptomatic tional class I I to I V symptoms and have been s hown to
hypotension and bradycardia . Once t he heart rate is below reduce mortal i ty and morb i d i ty. For patients w i t h class I I
60/m i n , the current close can be mai n tained. A history of symptoms, t he benefi t has been shown o n ly i n t hose w it h a
COPD is not a con t raindication to in itiating �-blocker therapy. history of prior hospitalization or an elevated B N P leve l .
a nd t here is no evidence that the nonselective �-blockers are The principal s i d e effec t of t hese agents is hyperka lemia .
not tolerated i n these patients. Spironolactone and eplerenone have not been compared
35
H e a rt Fai l u re
c:::J w i t h one another, but in c l inical trials. gynecomasti a occurs KEY PO I NTS
spec i fically w i t h spi ronolactone. Because of the risk of kid-
CONT. • Initial therapy for all patients with heart failure with
ney dysfunction a n d hyperkal e m i a , t hese d rugs should be
reduced ejection fraction should include an ACE inhibi
used o n ly i n patients w i t h a seru m crea t i n i n e level below
tor; those with volume overload should be given a diu
2 . 5 mg/d L (221 µ mol / L) i n men or below 2 . 0 mg/d L
retic, and once the acute heart failure episode has stabi
( 1 76 . 8 µ mo l / L) i n women . a n d with a serum potassi u m level
lized, all patients should be placed on a �-blocker.
below 5 . 0 m Eq / L (5 . 0 m mol / L) . Add i t ionally, i f' the patient
• �-Blockers in the treatment of heart failure should be
is on potassium supplementation. this should be disco n t i n
started at a very low dose and up-titrated slowly, at 1- to
ued when therapy is i n it iated. Electrolytes and kid ney func
2-week intervals.
t ion should be c hecked l week a fter i n i tiation of' therapy
a n cl be closely m o n itored over t ime. Aldosterone a n tago • Aldosterone antagonists should be started in patients
n i sts should be used very caut iously in elderly pa t ien ts . with New York Heart Association class II to IV symp
These d rugs a re not effect ive as d i u ret ics at the doses used toms with appropriate kidney function and a potassium
in heart fai l ure t herapy ( 1 2 .5 -25 mg/cl for spironolactone. level below 5 . 0 mEq/L (5.0 mmol/L).
25-50 mg/d for eplerenon e) . • The addition of isosorbide dinitrate and hydralazine to
standard heart failure therapy is associated with
lsosorbide Dinitrate and Hydra lazine improvements in quality of life and a mortality benefit
The combination of isosorbide d i n i t rate and hydralazine is in black patients.
an a lternative therapy for patients with heart fa i l u re who
have kid ney dysfu n c t io n that l i m its therapy w i t h e i t her ACE
i n h i b i tors or A R Bs . In t h is sett i ng. this comb i n a t io n is used M a n a g ement of Heart Fai l u re c:::J
for its vasod i la t i ng propert ies. t-.'1ore recen t ly, based on retro
spective clata from earl ier c l i n ical trials. a c l i n ical t rial i n
with Preserved Ejection Fraction
black patients w i t h heart fai l u re and reduced ejection frac ACE i n hibitors. A R Bs, �-blockers, and a ldosterone antagon ists
tion and NYHA class I l l and IV symptoms was performed have been studied i n patients with H FpEF. U n fortunately, none
t hat demonstrated a reduction i n mortality w i t h a speci fic of these agen ts have demonstrated any clinical benefit com
for m u l a t ion of t h i s combination compared w i t h pl acebo. pared with placebo. At this t i me, no medications have demon
There was a high incidence of adverse effects (pri mari ly strated a reduction i n mortality i n this pat ien t population.
peripheral edema. dizzi ness. gastro i n testi n a l sy mptoms. and Therapy for H FpEF should i nstead be based on treating the
headaches) and d rug w i t h d rawa l . For black pat ients. studies causes and symptoms of the heart failure. Hypertension is a
have demonstrated i mp rovements i n qual i ty of l ife i n addi common cause of H FpEF. and aggressive control of blood pres
tion to a mort a l i ty benefi t . Note t ha t t h i s combination was sure is necessary. Additional ly, controll i ng tachycardia can be
studied as an additional t herapy for patients already on a n helpfu l in pa tients with atrial arrhythmias.
A C E i n h ibitor or a n A R B and a �-blocker, n o t a s a replace Patients with H FpEF are often quite vol u me sensitive. with
m e n t t herapy. and should o n ly be i ns t i t u ted after t hese a small t herapeut ic window between hypovolemia and hyper
agents have been max i m ized. volemia. Judicious use of diuretics to maintain euvolemia is
importan t . These patients should be encouraged ro monitor
Calcium Cha nnel Blockers t heir weight closely. as small d i fferences in volume can quickly
Because of t he i r vasod i l a t i ng effects, ca lcium channel block cause volume overload and subsequent hospital admissions.
ers have been closely studied for their potential role in the
management o f heart fai l u re. U n fortunately, t he non
d i hydropyridi ne calc i u m channel b lockers ( for exa m ple , Device Th era py
d i l t i azem or verapa m i l ) also h ave myocard ial depression Sudden cardiac death is t he cause of deat h i n approximately
activity and have been demonstrated to either have no ben 50% of patients with heart fai lure. The only reliable predictor
efit or worse outcomes i n patients w i t h heart fa i lure. Patients of an arrhythmic event is left ven tricular ejection fraction. For
who have been t reated for hypertension with cl i l t iazem or this reason. i mplantable cardioverter-defibril lators ( ! CDs) are
verapa m i l should have t h ose agents d isco n t i n ued once a used for primary prevention of sudden cardiac death i n
d iagnosis of heart fai l u re has been made. The second patients w i t h heart failure a n d low ejection fraction .
generation d i hydropyrid i ne calcium channel blockers, such
as amlodipine and felod i p i ne. have been shown to be safe i n I mplantable Cardioverter-Defibrillator
patients w i t h heart fai l u re. but d o not reduce morb i d i ty or for Prevention of Sudden Cardiac Death
morta l i ty. For patients who are s t i l l hypertensive o n h ig h In patients with m i l d to moderate heart fai l u re symptoms and
doses o f A C E i n h i bi tors and 0-blockers. a periphera l ly acting left ven t ricular eject ion fraction less t han or equal to 35%.
d i hydropyridine calcium channel blocker can be used as an placement of an !CD reduces mortal ity compared with medi
anti hypertensive agent. CJ cal t herapy or placebo i n patients with both ischemic and
36
H e a rt Fa i l u re
History of hemodynamically sign ificant ventricu l a r • Cardiac resynchronization therapy is recommended for
arrhythmia or cardiac a rrest (secondary prevention) patients with New York Heart Association class II to IV
Biventricular Pacemaker heart failure, a left ventricular ejection fraction less
(cardiac resynchronization therapy) than or equal to 35% on guideline-directed medical
therapy, and a left bundle branch block with QRS
All of the fo l l owi n g :
duration greater than or equal to 150 msec.
NYHA class I I t o IV
37
H e a rt Fai l u re
intake, and exercise regularly. Patients who appropriately take <14 mL/kg/min) or a high ratio o f ventilation-to-carbon dioxide
their medications and avoid sodium and excess fluid intake production (VE/VC02 >34) have a poor 1-year prognosis.
can greatly improve their functional status. The Seattle Heart Failure model (www. SeattleHeart
Despite multiple studies demonstrating the benefit of FailureModel.org) is an online program that uses clinical char
medical therapies in heart failure, fewer than 60% of patients acteristics to predict outcomes in patients with heart failure.
are discharged from the hospital on ACE inhibitor and �-blocker This model can be used to help assess prognosis based on
therapy. It is important to review medications at every visit to clinical characteristics and can be used to guide patients as
ensure that patients are on the appropriate therapy. they ask questions about their prognosis.
K EY P O I NTS
Serial B-Type Natriuretic Peptide Assessment
• Patients with chronic heart failure should be seen regularly
Serial assessment of BNP levels in patients with chronic heart
for assessment of clinical status as well as ongoing patient
failure have been evaluated in a number of studies. Although
education regarding taking medications as prescribed,
higher BNP levels are associated with increased mortality,
measuring their weight daily; reducing dietary sodium and
change in level in an individual patient does not predict pro
avoiding excess fluid intake, and exercising regularly.
gression of disease. Additionally, there is no evidence of
benefit to using BNP for serially following patients to assess • In patients with chronic heart failure who are clinically HVC
volume status or for dose adjustment of medications. stable, armual or more frequent follow-up echocardiog-
raphy rarely provides therapeutic or diagnostic benefit
Echocardiography in Chronic Heart Failure and is not recommended.
Echocardiography should be performed in patients with severe
left ventricular dysfunction after optimization of medical
therapy to determine if the left ventricular ejection fraction
has improved to above 35% before consideration of !CD
I n patient Management CJ
of H ea rt Fa i l u re
implantation. For patients with chronic heart failure who are
clinically stable, echocardiography rarely provides diagnostic Acute Decompensated Heart Fai l u re
benefit, and obtaining annual echocardiograms is not likely to Patients with heart failure admitted to the hospital usually have
change therapy or outcome. For patients hospitalized with symptoms of volume overload as the primary concern. Reasons
acute heart failure, obtaining a repeat echocardiogram to eval to admit patients include progressive heart failure symptoms
uate left ventricular function or for worsening valvular abnor with dyspnea at rest, an inability to respond to oral diuretics.
malities is reasonable. If a patient has progressive heart failure recurrent ICD firings, symptoms of ischemia. worsening kid
symptoms as an outpatient, a repeat echocardiogram can be ney function. and signs of poor perfusion (such as cool extrem
helpful to evaluate for progressive valvular abnormalities, new ities, a low pulse pressure, or pulsus alternans) . Therapy i
wall motion abnormalities, or an increase in left ventricular primarily focused on diuresis. Additional evaluation should be
size that may alter treatment and affect prognosis. performed to determine the reasons for the decompensation,
including a review of medications and whether the patient was
Assessing Prognosis taking his or her medications properly, an echocardiogram to
Multiple retrospective studies have been performed looking at look for reversible causes of worsening function. and, i f appro
methods to evaluate prognosis in patients with heart failure. priate. an evaluation for ischemia . Generally. the initial dose of
Current 1-year survival rates for patients undergoing heart intravenous diuretic should be at least equivalent to the total
transplantation or placement of a left ventricular assist device daily oral dose. l f the patient does not respond appropriately to
are between 85% and 90%. Patients with a higher risk of death that dose, rapid up-titration should be performed to assist in
should be considered for these therapies. Important risk factors fluid removal. The patient's usual outpatient medications (for
for death include NYHA class IV symptoms, repeat hospitaliza example, ACE inhibitor, �-blocker) should be continued unless
tions, hyponatrernia (serum sodium <133 mEq/L [133 mmol/L]) , the patient is hypotensive or demonstrates signs of poor perfu
worsening kidney function, higher doses of diuretics, intoler sion, in which case dose reduction or discontinuation of both
ance of ACE inhibitors or �-blockers, and arrhythmias result the ACE i n hi b i t o r and �-blocker should be considered . I n
ing in !CD firings. Patients with multiple risk factors should be patients with signs o f low-output heart failure (hypotension,
referred to a heart failure cardiologist for further evaluation. worsening kidney or liver function. cool extremities) . the
Additionally, occasional discussions with patients regarding �-blocker should be discontinued.
end-of-life issues and their wishes for advanced heart failure Patients should be adequately diuresed during the hospi
therapy should be initiated while the patient is still stable. talization . Orthopnea and an elevated central venous pressure
Patients' advanced care plans often change over time. are suggestive of elevated fJlling pressures. Patients often have
Cardiopulmonary exercise testing is routinely performed to symptomatic improvement before they are euvolemic. and
assess prognosis in patients being evaluated for transplantation. striving to achieve euvolernia may result in a reduction in read
Patients with a low oxygen consumption (peak 02 consumption mission rates. Volume status can be difficult to assess and a
38
H e a rt Fa i l u re
Strength o f effect: ++ indicates very strong; + indicates strong; ( + ) indicates weak; 0 indicates neutral; - indicates opposite effect.
39
H e a rt Fa i l u re
CJ include intra-aortic balloon pumps and percutaneous or sur transplant is 65 to 70 years. Patients with kidney dysfunction,
gically implanted short-term mechanical ventricular assi.st diabetes with end-organ manifestations. malignancy. chronic
CONT.
devices (VADs) . These assist devices have catheters that are infection, or other comorbidities are often denied transplant.
placed into the vascular system (left atrium or ventricle) ; they Options tor patients who are not candidates for heart transplan
then pump the blood into the aorta, essentially assisting the tation include mechanical circulatory support as destination
failing left ventricle. These devices augment cardiac output therapy and inotropic therapy. However, inotropic therapy does
and improve end-organ perfusion . Because all of these devices not decrease mortality and may actually increase it. The survival
require large catheters, a common complication is vascular of inotropic-dependent patients is less than 10% at 1 year.
compromise at the point of insertion.
Mechanica l Circulatory Support
Strategies to Prevent Readmission With the development of continuous-flow left ventricular assist
At many U.S. hospitals. heart failure is the most common dis devices (LVADs) , the survival of patients vvith advanced heart
charge diagnosis. Currently. 30-day readmission rates are greater failure after implantation of these devices has dramatically
than 20%, and reducing these admissions is a major focus of improved. The pumps are smgically inse11ed into the left ventri
study and resources. Studies evaluating dimesis have shown that cle, and the blood is pumped through the device from the left
greater fluid removal during the hospitalization is associated ventTicle to tJ1e aorta. The patients have a line (driveline) coming
with a lengthening of the time to readmission. Additionally, it is through the skin through which the power is transmitted to tlle
imp011ant that patients are discharged on appropriate medical pump. Ninety percent of patients receiving an LVAD as a b ridge to
therapy, including an ACE inhibitor and a �-blocker. Early physi heart transplantation are alive at 1 year. For patients who are not
cian fol low-up, ideally within 7 days of discharge, has also been candidates for transplant and have an LVAD placed as destination
associated with a reduction in readmissions. therapy, the survival at 2 years is more tJ1an 60% and improving.
Because it is often difficult to schedule patients for an CompUcations related to these devices include ischemic and
office visit within 1 week of discharge, multidisciplinary heart hemoIThagic stroke (>10% o f patients), driveline-related infec
failure clinics have been created by a number of hospitals. tions (approximately 30%) . and gastrointestinal bleeding related
These programs often include telephone monitoring of signs to arteriovenous malformations (20% in some reports) . Cl
and symptoms. evaluating whether patients are actually tak
Management of Post-Transplant Patients
ing their medications, educating patients on salt and fluid
restriction, and providing a mechanism for early fol low-up The prognosis of heart transplant recipients has improved
after hospitalization. Cl greatly in recent years. Most patients have no functional limita
tions and return to a normal quality of life. Patients typically
KEY POINTS begin on a three-drug immunosuppression regimen early after
• In hospitalized patients with acute volume overload, the transplantation that includes a calcineurin inhibitor (cyclo
initial dose of intravenous diuretic should be at least sporine or tacrolimus) , an antiproliferative agent (mycopheno
equivalent to the total daily oral dose; rapid up-titration late mofetil, sirolimus, or everolimus) , and prednisone. Most
should be performed if needed to assist in fluid removal. centers try to wean patients off of prednisone by 1 year.
• Reversible causes of cardiogenic shock include acute lmmunosuppressive medications are associated with a number
myocardial infarction, ventricular septa! or free wall of adverse effects, including hypertension (>90% of patients)
rupture, and acute valvular regurgitation. and new-onset diabetes (20% of patients) . During the first year
post-transplant, while doses of immunosuppressants are high,
HVC • In patients discharged with a diagnosis of heart failure,
patients have an increased risk for infection. Rejection occurs
early physician follow-up, ideally within 7 days of dis
in approximately 20% of patients in the first year but is almost
charge, has been associated with a reduction in hospital
nonexistent after the first year unless a patient stops taking
readmissions.
immunosuppressants. Signs of rejection include heart failure
and atrial arrhythmias (typically atrial flutter) . However, most
patients with rejection manifest no clinical symptoms, neces
Adva nced Refractory Heart Fa i l u re sitating routine surveillance with endomyocardial biopsy. The
40
H e a rt Fa i l u re
intervention and coronary artery bypass grafting, are usually Characteristic electrocardiographic changes include ST-segment
not beneficial. Lymphoproliferative disorders and skin can elevation and diffuse deep T-wave inversions with some pro
cer are the most common malignancies. longation of the QTc interval. Takotsubo cardiomyopathy is
Because the heart in transplant patients is denervated, they usually associated with recovery of systolic function in the acute
usually do not experience typical ischemic chest pain, leading to period. Nevertheless, these patients should be treated with
atypical presentations of coronary artery disease and acute coro ACE inhibitors and �-blockers acutely. There is no accepted
nary syndromes. Additionally, without vagal innervation, heart length of time to continue this therapy in patients whose left
rates tend to run between 90/min and 110/min. Heart transplant ventricular function returns to normal. For the rare patient who
patients have a marked response to adenosine but are not does not recover, this therapy should be continued.
responsive to digoxin or atropine. For transplant patients pre
senting with atrial arrhythmias, caution should be used before Acute Myocarditis
giving adenosine to diagnose the arrhythmia because it may Myocarditis usually presents with heart failure symptoms over
cause prolonged atrioventricular conduction block. a few days to weeks. Occasionally, patients have symptoms for
several months before heart failure is discovered. The classic
KEY POI NTS
presentation of viral myocarditis includes a viral prodrome
• Cardiac allograft vasculopathy occurs i n more than 50% with fever, myalgia, and upper respiratory symptoms, but a
of heart transplant recipients by the fifth year after prodrome is not required for the diagnosis. Patients present
transplant; because of its diffuse nature, revasculariza with dyspnea, chest pain, and arrhythmias. ECG abnormalities
tion is usually not beneficial. are often present, along with evidence of myocardial damage
• Because the heart in transplant patients is denervated, with elevated troponin levels.
they usually do not experience typical ischemic chest Various infectious pathogens can cause myocarditis. The
pain, leading to atypical presentations of coronary most common causes are adenovirus, coxsackievirus, and
artery disease and acute coronary syndromes. enterovirus. The pathogenesis of myocarditis is unclear and
may involve direct infection of the myocardium with the virus
or an immune system response to the infection.
S pecific Ca rdiomyopathies Endomyocardial biopsy can define myocarditis with evi
The most common cause of heart failure is coronary artery dis dence of myocardial necrosis, degeneration, or both, with an
ease. Other common causes include hypertension and idiopathic adjacent inflammatory infiltrate. Indications for endomyocar
cardiomyopathy. Approximately 10% of patients with heart fail dial biopsy include ventricular arrhythmia, high-grade con
ure have heart failure related to a specific etiology. This includes duction block (type II or III) or lack of response to usual heart
medication-induced cardiomyopathies (primarily chemothera failure therapy.
peutic agents) , myocarditis, amyloidosis, sarcoidosis, infectious Therapy for acute myocarditis is supportive and con
etiologies such as HIV, periparturn cardiomyopathies, and alco sists of usual heart failure therapy. Placebo-controlled
hol or other drug-induced cardiomyopathies. Others will be immunosuppressive trials have not demonstrated improve
discussed later. Restrictive cardiomyopathies with such causes as ments in mortality or ejection fraction. Patients often take
chemotherapeutic agents, amyloidosis, and sarcoidosis are dis months (6 -12) to recover left ventricular function.
cussed in Myocardial Disease. Peripartum cardiomyopathy is Approximately 50% of patients eventually recover cardiac
discussed in Pregnancy and Cardiovascular Disease. function; therefore, it is important to wait and not place an
!CD for the usual indications (ejection fraction <35% and
Takotsubo Cardiomyopathy NYHA class II or III symptoms) until at least 6 months of
Takotsubo, or stress-induced, cardiomyopathy is a syndrome of heart failure therapy.
reversible ventricular systolic dysfunction usually precipitated
by an acute emotional or physiologic stress. Although takotsubo Giant Cell Myocarditis
cardiomyopathy was initially described in elderly women fol Giant cell myocarditis is an acute, rapidly progressive form of
lowing intense emotional stress, the syndrome may occur in myocarditis associated with ventricular arrhythmias and pro
men and in some patients an antecedent stress may not be gressive cardiac dysfunction despite medical therapy. For
identifiable. It is believed to be caused by sympathetic-mediated unclear reasons, this process usually occurs in persons younger
myocyte injury, but the precise pathogenesis is unknown. It than 40 years. The underlying mechanism is unknown but is
often mimics an acute myocardial infarction with elevated tro thought to be autoimmune. On endomyocardial biopsy, the
ponin levels and electrocardiographic changes, but it is usually pathognomonic "giant cell" is seen. Unlike other forms of
associated with normal coronary arteries. The hallmark is wall myocarditis, aggressive immunosuppressive therapy has been
motion abnormalities that extend beyond a single coronary ter shown to improve survival but this process is still often fatal.
ritory, identified by echocardiography or other imaging study. These patients should be considered for cardiac transplantation
For example, on left ventriculogram, the apex of the heart will but often need to be bridged with VADs. There are case reports
be hypokinetic and the mid heart will contract normally. of giant cell myocarditis recurring post-transplantation.
41
M yo ca rdial Disease
F I G U R E 1 1 A patient with hypertrophic cardiomyopathy. Resting 1 2-lead electrocardiogram ( top left panel) demonstrating findings of left ventricular hypertrophy and second·
.
ary ST-seg ment changes. Echocardiography (parasternal view) (bottom left panel) demonstrating severe myocardial hypertrophy of the ventricu lar septum (asterisk). Systolic ante·
rior motion of the mitral valve is present (arrow) (top right panel). Apical long axis view of the left ventricle (bottom right panel). Systolic anterior motion of the mitral valve (arrow
head) leads to decreased coaptation of the mitral valve and secondary mitral regurgitation (arrow). Ao = ascending aorta; LA = left atrium; LV = left ventricle; RV = right ventricle.
42
Myocard ial D isease
43
Myocard i a l Disease
�������-
F I G U R E 1 3 . Pathology of hypertrophic cardiomyopathy. Gross specimens demonstrate severe myocardial hypertrophy due to hypertrophic cardiomyopathy with concen
tric hypertrophy (A and B) and apical hypertrophy (C). M icroscopy demonstrates myocyte disa rray, the histologic hal l mark of hypertrophic cardiomyopathy (0). Ao = ascending
aorta; LA = left atrium; LV = left ventricle; RA= rig ht atri um; RV = right ventricle.
Images courtesy of Dr. William D. Edwards, Department of Pathology, Mayo Clinic
risk stratification for sudden cardiac death (Table 21) . Therapy Because a significant number of sudden deaths occur during or
with an implantable cardioverter-defibrillator (!CD) should be following exercise, abstention from competitive sports or stren
considered in patients with one or more risk factors. The uous aerobic activities is advised for all patients. Patients with
clinical efficacy of !CDs for prevention of sudden cardiac death HCM also should be counseled on the need to avoid dehydration
in HCM has been demonstrated in several large registries. and states of severe peripheral vasodilatation, such as from hot
baths or saunas. Pregnancy generally is well tolerated, although
KEY POINT
precautions should be taken to minimize peripheral vasodilata
• Placement of an implantable cardioverter-defibrillator tion as for all patients with HCM.
should be considered in patients with hypertrophic car
diomyopathy with one or more major risk factors for Phannacologic Treatment
sudden cardiac death: prior cardiac arrest, massive Negative inotropic agents are the cornerstone of medical ther
myocardial hypertrophy, family history of sudden car apy for patients with symptomatic obstructive HCM. Negative
diac death, ventricular tachycardia, blunted blood pres inotropes (�-blockers, nondihydropyridine calcium channel
sure response to exercise, unexplained syncope.
blockers [verapamil], and disopyramide) depress contractility,
thereby reducing the intraventricular flow velocities that pre
Management dispose to LVOT obstruction. These agents also lengthen dias
The major management goals for patients with HCM are allevia tole, facilitating more time for ventricular filling. Appropriate
tion of symptoms, risk stratification for sudden cardiac death, drug therapy suffices for most patients with symptomatic
ICD implantation for those at high risk, and family counseling. obstructive HCM, although high doses may be required.
44
Myocard i a l Disease
TABLE 20. Clinical Features Distinguishing Hypertrophic Cardiomyopathy from Athlete's Heart
Feature Hypertrophic Cardiomyopathy Athlete's Heart
Fa m i ly history Positive Negative
El ectroca rdiography Patho l ogic Q waves, T-wave inversions, Absence of these features
conduction defects
Doppler echocardiography Diastolic fil l ing abnormalities Normal d iastol ic fil l i n g
Extent of hypertrophy > 1 5 mm Often :'> 1 2 m m
Pattern of hypertrophy Asymmetric, concentric, or eccentric Concentric
Left ventricu l a r end-d iastolic d i mension <45 m m >55 mm
Gadolinium hyperenhancement o n Present Absent
card iac magnetic resonance i m a g i n g
Objective exercise testing % Predicted peak Vo2 < 1 00% % Predicted peak Vo2 > 1 20% or
>50 m Ukg/min
Genetic testi ng Positive Negative
Eva l uation after period of deconditioning No regression in hypertrophy Regression >2 mm
TABLE 2 1 . Risk Factors for Sudden Death in Patients with Hypertrophic Cardiomyopathy
Risk Factors Comments
Major"
Cardiac arrest (ventricular fi brillation) Portends high rate of recurrence or death ( 1 1 % per year)
Spontaneous susta i n ed VT
Fam i ly h istory of premature sudden death Most predictive if occurs i n a close relative or m u ltiple relatives
U n explained syncope Most predictive if occurs i n young patients, is exertional, or is
recurrent
B l u nted increase (<20 mm Hg) or decrease i n systo lic blood More predictive in patients <SO years of age
pressure on exercise
Nonsusta i n ed spontaneous VT Less predictive if very brief (:'>3 beats) a n d asym ptomatic
Heart fai l u re that has progressed to dilated cardiomyopathy with Occurs in 5% to 1 0% of patients with hypertrophic
ejection fraction :'>35% and NYHA class I I or Ill symptoms cardiomyopathy
CMR = cardiac magnetic resonance; NYHA = New York Heart Association; VT = ventricular tachycardia.
a Risk factors from Gersh BJ, Maron BJ, Bonow RO. et at; American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. 20 1 1 ACCF/
AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy: a report of the American College of Cardiology Foundation/American Heart Association Task
Force on Practice Guidelines. Developed in collaboration with the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear
Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll
Cardiel. 201 1 Dec 1 3;58(25):e21 2-60. IPMID: 220754691
45
M yo ca rdial Disease
46
Myocard i a l Disease
TABLE 22. Recommended Screening Intervals for Evaluation of First-Degree Relatives of Patients
with Hypertrophic Cardiomyopathy
Age Group Recommendation
Presence of symptoms
LV = left ventricular.
NOTE: These recommendations are for relatives of patients with hypertrophic cardiomyopathy in whom genetic testing is negative, inconclusive, or not performed.
Recommendations from Gersh BJ, Maron BJ, Bonow RO, et al; American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. 20 1 1
ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy: a report of the American College of Cardiology Foundation/American Heart Association
Task Force on Practice Guidelines. Developed in collaboration with the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of
Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am
Coll Cardiol. 201 1 Dec 1 3;58(25):e21 2-60. [PMID: 22075469]
the interpretation of the results, which can include known and heart failure. In rare cases, restrictive cardiomyopathy is
pathologic mutations and likely pathogenic mutations, as well familial, with an autosomal dominant pattern of inheritance.
as variants of unknown significance. Restrictive cardiomyopathy is a diagnosis of exclusion and
Genetic testing is performed as a panel, with the mutations must be distinguished from constrictive pericarditis (see later) ,
associated with the greatest likelihood of pathologic conse eosinophilic syndromes, radiation-induced disease, storage
quences being �-myosin heavy chain, myosin-binding protein diseases (such as Fabry disease, hemochromatosis) , as well as
C, troponin T, troponin I, o:-tropomyosin, actin, regulatory light infiltrative cardiomyopathies, such as amyloidosis and sar
chain, and essential light chain. A negative genetic test result coidosis. Patients with other forms of heart failure can also
does not rule out HCM in patients with phenotypic evidence of have restrictive patterns of diastolic ventricular filling but are
disease, so their immediate family members still should undergo not defined as having a primary restrictive cardiomyopathy.
interval screening. Whereas all patients with HCM can be con Patients with restrictive cardiomyopathy may present at
sidered for genetic testing to facilitate diagnosis in family mem any age, with symptoms and signs of pulmonary and systemic
bers, the decision to pursue testing is individualized based on congestion. The diagnosis can be considered when there is
likelihood of detecting mutations, patient and family desire, and severe diastolic dysfunction and dilated atria in the absence of
reimbursement concerns. The likelihood of detecting mutations ventricular hypertrophy or cavity dilatation. Systolic function
is increased with a positive family history and a reversed curva is preserved in most patients or, at least, is disproportionately
ture morphology of the ventricular septum. In reversed curva high given the degree of diastolic dysfunction. Pulmonary
ture, the hypertrophy of the septum is maximal in the hypertension, secondary to diastolic dysfunction, is common.
mid-portion, with relatively less hypertrophy in the apical and Endomyocardial biopsy can be performed for patients
basal segments. To date, genetic test results have not been with suspected restrictive cardiomyopathy to evaluate for infil
strongly linked to risk of sudden cardiac death, so they should trative disease, such as storage diseases or amyloidosis, when
not be used for risk stratification. clinical assessment or less invasive testing (such as protein
electrophoresis or fat biopsy) is inconclusive. The yield of
KEY POINT
biopsy is low in disorders with patchy myocardial involvement
• All first-degree relatives of patients with hypertrophic such as sarcoidosis.
cardiomyopathy should undergo screening for the dis
ease, with screening intervals recommended according
Cl
Differentiating Restrictive Cardiomyopathy
to age, symptoms, and family history. from Constrictive Pericarditis
Both restri c t ive cardiomyopat hy and constrict ive pericarditis
present with e levation of d iasto lic pressures and hea rt fai lu re
Restrictive Ca rd i omyopathy that is disproportionate to the degree of systo l i c dysfunction .
Clinical Presentation and Evaluation Distinction of the two di sorders is i mportan t as pericardiec
,
Primary restrictive cardiomyopathy is an idiopathic disorder tomy w i l l result in symptom relief and i mprovemen t in lon
characterized by nondilated, poorly compliant ventricles, lead gev ity i n patients with constriction . I maging and hemody na mi c
ing to severe diastolic dysfunction, elevated filling pressures, eva l ua tion a re most usef'ul for di ffe re n t iat ing t he se en ti ti es.
47
Myocard i a l Disease
48
Myocard i a l Disease
KEY POINT
F I G U R E 1 5 . Transesophageal echocardiography across the right atrium (RA) • Myxomas should be resected after diagnosis owing to
and left atriu m (LA) demonstrates a large mass (arrow) attached to the atrial sep· the risk of embolization and cardiovascular complica
tum i n proximity of the Iossa ovalis (asterisk); the mass was surgically removed tions, including the potential for sudden death.
and proved to be a left atrial myxoma.
49
Arrhyth mias
Antia rrhyth mic Medications atrial fibrillation. Class IC agents are avoided in patients with
coronary artery disease and structural heart disease as they
Antiarrhythmic medications are used t o prevent recur
have been shown to cause proarrhythmic activity (ventricular
rent arrhythmias and maintain sinus rhythm. Although anti
arrhythmias) and increase mortality. Class II agents
arrhythmic medications have historically been organized
(�-blockers) and class IV agents (nondihydropyridine calcium
according to their predominant mechanism of action using
channel blockers) are frequently used to slow heart rates in
the Vaughan-Williams classification system (Table 23) , it is
increasingly recognized that this nomenclature system has patients with supraventricular or atrial arrhythmias; however,
limitations because most antiarrhythmic drugs have several they should be avoided in patients who have atrial fibrillation
mechanistic actions. with preexcitation. Class III agents are used to treat atrial and
The membrane-active antiarrhythmic agents (class I and ventricular arrhythmias. These agents are cleared by the kid
class III) principally affect ion channels. Class I agents decrease neys and should be avoided in patients with significant chronic
impulse formation and speed of depolarization and are often kidney disease owing to increased toxicity and proarrhythmia.
used in patients with atrial arrhythmias and no structural Because class III agents lead to QTc-interval prolongation, ini
heart disease. Several class IA agents are used less frequently, tiation of this therapy is usually done on an inpatient basis
Class IC Sod i u m channel Flecainide, Decreases speed of Atrial fi bri l lation, SVT,
blockade propafenone depola rization and ventricular a rrhyth mias;
shortens repola rization avoid with CAD or
structural heart disease.
Class I I �-adrenergic blockade Metoprolol, Decreases sympathetic Rate control of atrial
propranolol, carved ilol, tone; suppresses arrhythmias, SVT,
atenolol, bisoprolol automaticity, sinoatrial ventricular arrhythmias;
conduction, and AV avoid if pre-excitation is
cond uction present.
Class I l l Potassi u m channel Sotalol, dofetilide Prolongs action Atrial fibrillation, atria l
blockade potential d u ration flutter, ventricular
arrhythmias; avoid in
CKD.
Class IV Ca lcium channel Verapamil, diltiazem Sup presses sinoatrial SVT, rate control of atrial
blockade and AV conduction arrhythmias, triggered
(nondihydropyrid i nes) arrhythmias (e.g.,
outflow tract VT); avoid
if pre-excitation is
present.
Multichannel blockers Severa l, incl u d i ng Amiodarone, Multi ple mechanisms, Atrial arrhyth mias,
potassi um, sod i u m , and d ronedarone although they a ct ventricular arrhythmias
calcium channel principally by extending
blockade repola rization
Adenosine receptor A1-receptor agonist Adenosine Slows or blocks Termination of SVT
agonists sinoatrial and AV nodal
conduction
Cardiac glycoside Increasing vagal a ctivity Digoxin Slows AV nodal Rate control of atrial
conduction fibrillation
AV = atrioventricular; CAD = coronary artery disease; CKD = chronic kidney disease; SVT = supraventricular tachycardia; VT = ventricular tachycardia.
50
Arrhyth m i a s
with regular assessment of the QTc interval. Patients taking malities Lhal require urgent in tervention . Evaluation includes
class I I I agents should avoid other QT-prolonging medications, a carefu l history. a focused laboratory evaluation (including an
and serum potassium and magnesium levels should be assessment of thyroid function) . resting 12-lead electrocardio
checked regularly. gram (ECG) . exercise treadmill testing to assess the heart rate
Amiodarone, a multichannel blocker, is among the most response to exercise (chronotropic competence) , and ambula
commonly used antiarrhythmjc medications. It is frequently tory ECG monitoring based on the nature and frequency of the
used to treat atrial fibrillation in older persons and to prevent patient's episodes or symptoms (see Diagnostic Testing in
recurrent ventricular tachycardia. Amjodarone is the preferred Cardiology) . Rarely. electrophysiologic testing can be used to
antiarrhythmic agent in patients with structural heart disease help ascertain i f sinus node dysfunction is present. Cl
and heart failure. Although hlghly effective, amiodarone has
multiple toxicities. Amiodarone therapy is associated with Sinus Bradycardia
risks for thyroid toxicity, hepatotoxicity, lung toxicity, photo
Sinus bradycardia (sinus rhythm with a heart rate <60/min)
sensitivity, corneal and lenticular deposits, optic neuropathy,
may be appropriate in several situations, i ncluding i n
and other neurologic adverse effects. Patients on amiodarone
trained athletes or during sleep, when t h e heart rate may
require routine monitoring of thyroid and liver function, pul
fall as low as 30/min. The most common intrinsic cause of
monary function testing at baseline and with symptoms, and
inappropriate or pathologic sinus bradycardia (sinus node
periodic ophthalmologic evaluation. Amiodarone interacts
dysfunction) is age-related myocard ia l fibrosis in the vicin
with several medications. Patients on amiodarone require
ity of the sinus node. The most common extrinsic cause o f
lower doses of warfarin, statins, and digoxin. Dronedarone is a
sinus bradycardia i s medication effect. S i nus node dysfunc
multichannel blocker used to treat atrial fibrillation. Owing to
tion can also present with chronotropic i ncompetence, and
increased mortality i n patients with heart failure or permanent
this is frequently overlooked. Other, less common, causes of
atrial fibrillation, its use should be restricted to patients with
sinus node dysfunction include right coronary ischemia,
i ntermittent atrial fibrillation and no overt heart failure.
i ntracranial hypertension, postsurgical scarring a fter car
Digoxi n is an oral positive inotropic agent that acts on the
diothoracic surgery, and infiltrative or inflammatory d isorders
sodium-potassium exchanger and has vagal properties that
(such as sarcoidosis ) .
lead to decreased atrioventricular (AV) nodal conduction. As a
result of its vagal mechanism, it primarily controls the heart
rate at rest and is less effective during activity. Adenosine is an Atrioventricular Block
A1-receptor blocker that can inhlbit AV conduction. Adenosine AV block is classified as first degree, second degree, or third
is frequently used as a therapeutic agent to terminate supraven degree. First-degree AV block is characterized by prolonged AV
tricular tachycardia. conduction, which manifests on the ECG as a PR interval
greater than 200 msec. First-degree AV block is not a true
KEY POINT
block because all P waves conduct to the ventricles. It has been
• Calcium channel blockers and �-blockers are often used associated with an increased risk of atrial fibrillation, pace
to treat supraventricular and atrial arrhythmias; how maker implantation, and all-cause mortality in long- term
ever, these agents should be avoided i n patients who follow-up.
have atrial fibrillation with preexcitation. ln second-degree AV block, some P waves conduct to the
ventricle and some do not. There are two forms of second
degree AV block. When progressive PR prolongation is
observed prior to a blocked beat, second-degree Mobitz type 1
Approach to the Patient
(Wenckebach block) is present. Second-degree Mobitz type 1
with Bradyca rd ia
c::J Clinica l Presentation
block is characterized by grouped beating and progressive
shortening of the R-R intervals. Mobitz type 1 block is almost
Symptoms ofbradycardia (heart rate less than 60 /min) include always localized to the AV node. It generally carries a benign
fatigue, exertional intolerance, dyspnea, light headedness. and prognosis and frequently i mproves with exercise or i ncreased
syncope. Bradycardia can result from pathology in the sinus sympathetic tone.
node. the AV node, or the His-Purkinje system. Physicians When the PR i nterval is constant prior to nonconducted P
should maintain a high suspicion for reversible causes of waves, the second-degree block is termed Mobitz type 2 block.
bradycardia. including elevated i ntracranial pressure. hypo When 2:1 block is present, Mobitz typ e 1 versus type 2 block
thyroidism, hyperkalemia. Lyme disease. and medication cannot be di fferentiated. Mobitz type 2 block usually repre
effects (most common ly AV nodal blockers, especi a l ly sents block lower in the conduction system and has a higher
�-blockers and digoxin) . risk of progression to complete heart block.
The diagnostic evaluation of bradycardia i ncludes ( 1 ) Third-degree AV block, or complete heart block, is defined
establishing a correlation between the rhythm (bradycardia) as the failure of any P waves to conduct to the ventricles, and
and symptoms and (2) excluding severe conduction abnor- it i s characterized by AV dissociation on the ECG.
51
Arrhyth m i a s
Cl Pacemakers
P<.1 t ienls w i t h i n t ravc n t ri c u l a r co n d u c t i o n delays have 8
Pace m a kers a re i n d i ca Lcd i n pal ien Is wi I h sy m p l o m a l ic brady low risk or p rogression to com p l c l e hearl block ( I %-3'Y., a n n u
carcl i a in t h e �1bscnce or 8 reversi b l e cause, hence l h c i m ror a l ly) a n d clo nol req u i re perma n c n l pa c i ng. W h e n a pa t i e n l
l <J nce o r cst abl i s h i ng sy m p toms when cva l u <JL i ng pal icn ts w i t h develops new-on set cond u c l i o n d i srnsc i n L h e set t i ng o r a n
braclyca rd i a . In pa l ien ls w i t h m i n i ma l sy m pl o m s, a pcrs islc n t aculc coronary sy n d ro me. t<.:m porary pac i ng 1rn1y be req u i red.
rcs l i ng hc<Jrl rn l c below 40 m i n is a lso co nsidered a n i nd ica but deci s ion s on permanent pac i ng s h o u l d be delayed u n i i i a
L i on for per m a n e n t paci ng. Pacemakers a l so a rc i ncl i c a l cd i n pa l i c n l has been revase u la r i i'.cd a n d s t ab i l i zed to determ i ne
have a h igh I i kcl i hood o r p rogress i ng to com p l e l e he�1 rL block Pa t ie n l s w i t h pacema kers who req u i re su rgery s h o u l d
or Ii le L h rea t c n ing sudden a sysl olc. I llC.I ical ions lcir perm a ncnt have a preopera L ive device eva l ua t ion lo determ i n e whet her
pacemaker i m p l a n l a t ion <Jrc s hown i n Table 24. preopera t ive reprogra m m i ng o r the dev i ce i s necessa ry.
/\ I L hough "MRI con d i t ional" pacemakers a rc now ava i l a b l e. L hc
TABLE 24. Selected Indications for Permanent Pacing presence or a pace m a ker re m a i n s a co n l ra i ncl ic a l i o n to MRI
sca n n i ng lor most pa t i e n ts.
Symptomatic bradycardia without reversible cause
There a rc severa l Lypes or i m p l a n lecl card i a c devices, w i l h
Asymptomatic bradycardia with significant pauses (>3 seconds
va rious ca p a b i I i t i cs . I m p l a n tecl c a rd iac e l ec t ro n i c devices
i n sinus rhythm) or persistent heart rate <40/min
i nc l u d e i m pl<i n ted loop mon i to rs. p8<.:c m a kers. i m p l a n table
Atrial fibri l l ation with 5-secon d pauses
ca rcl i overtcr- de libri l l a t o rs ( I CDs ) . a n d card i<Jc resy n c h ro n i i'.a
Asymptomatic complete heart block or Mobitz type 2 second
l io n dev i ces. Wi l h L h c exce p l i o n or subcula neous JCDs. w h i c h
degree atriove ntricular block
do not u t i l ize i n t ra c a rd i ac l e a d s . a l l I C Ds a l so have pacemaker
Alternating bundle branch block
fu nct ions. Table 25 reviews l h e V<J rious types or i m p l a n t ed
TABLE 25. Cardiac Implantable Electronic Devices for Treatment of Cardiac Rhythm Disorders
Functions
Device Components Indications Pacemaker Antitachycardia Defibrillation
Function Pacing
52
Arrhyth m i a s
CJ i n d icat ions. Cl
C<l!Tl i a c elcc l ro n i c devices. l h e i r !'u n c t i o n s . a n cl L h e i r general
Supraventricu lar Tachyca rd ias
CONT.
KEY POINT
Clinical Presentation
Supravc n t ri c u l a r tachycard ias ( SVTs) a rc a group or a rr hy l h -
CJ
• A pacemaker is indicated for symptomatic bradycardia 111 ias t h a t a rise i n a t ri a I t i ssue or t h e /\V nocle. Because conduc
without a reversible cause as well as for atrioventricular t i o n o l' su p ravc n t ri c u l a r i m p u l ses below t he /\V node i s
conduction abnormalities that are likely to progress to conducted normal ly. t he l�CG i n S V T usu<1 l ly revea l s a n a 1-row
complete heart block. complcx t;1chyca rd i a . a l t hough t he QRS complexes ca n be
w i de ( > 1 20 msec) i n t h e presence o l' b u n d l e bra nch b l ock,
abe rra ncy. paci ng. or a n t e rograde accessory pat hway conduc
53
Arrhyth m i a s
Symptomatic PACs are typically treated w i t h P-blockers or atrium over the fast pat hway (slow-fast) . This leads to a short
calcium channel blockers. RP interval with a ret rograde P wave inscribed very close to
Atrial tachycardia can occur in patients with or w i thout the QRS complex. The closely coupled retrograde P waves may
structura l heart disease; when symptomatic, first-l i n e treat be buried in t he QRS complexes and may not be visible. or
ment i s a P-blocker or nondihydropyridine calcium channel t hey may appear as a pseudo R' wave in lead V 1 and a pseudo
blocker ( d i l t iazem or verapam i l ) . Second- l i n e treatment S wave in the i n ferior leads. In atypical AVN RT. conduction
i ncludes catheter ablation or antiarrhythrnic drug therapy. I n goes clown the fast pathway and returns to the atrium via t h e
genera l , success rates for ablation o f atri a l tachycardia are slow pat hway ( fast-slow) : t his leads to a l o n g R P i nterval.
lower t h a n t hose for other SVTs. Rare ly. AVN RT can involve conduction over more t han one
Multifocal atri a l tachycardia, characterized by multiple slow pa t hway (slow-slow AV N RT) .
(2'.3) P-wave morphologies and a heart rate greater than 1 00/ Beyond acute term ination with physical maneuvers or
m i n , i s frequently seen in patients w i t h end-stage COPD. adenosine. treat ment to prevent recurrent AVN RT incl udes AV
Treatment is usually directed at t h e u nderlying etiology and nodal blocking t herapy w i t h P- blockers or nondi hyclropyri
electrolyte d isturbances, a l though P-blockers and calcium dine calcium channel blockers. Patients who have recurre n t
nonclihyclropyri cl i n e calcium channel blockers can be AVN RT or do not tolerate or prefer to avoid long-term medical
used cautiously. therapy are usually referred for catheter ablation, which has a
h igh success ra te. The major risk of' ablation is a 1 % risk of'
c::J Atrioventricular Nodal Reentrant Tachycardia injury to the AV node requiring pacemaker i mplantat ion .
AVN RT is t he most com mon type of SVT. account ing for two
t h i rds of a l l patients w i t h SVT (excl uding a t rial fibr i l lat ion Atrioventricu lar Reciprocating Tachycardia
and a t rial nu tter) . AV N RT is caused by reentrant conduct ion AVRT is an accessory pathway (bypass tract ) -mediated tachy
w i t h i n t he AV node, util izing both the fast and slow pat hways cardia . Accessory pat hway conduction is often observed as
(Figure 17) . In typical AVN RT, t he electrical conduction goes preexcita tion on ECG. Because of' early ven t ricu lar activa t ion
clown the slow pat hway and conducts back up toward the over the accessory pat hway. t he P R interval is shortened and
the i n itial part of the QRS complex is sl urred (del ta wave)
because of ventricular depolarization adjacent to the pat hway.
In AVRT, conduction is anterograde over t h e AV node (ortho
clromic AVRT) or anterogracle over the accessory pathway
(ant idromic AVRT) . Orthocl romic AVN RT. the most common
(To the atrium) type of' AVRT (more than 90% to 95'X, of cases) is char2cterized
by a na rrow QRS complex resu l t i ng from conduct ion over t he
AV node 2nd t he H is-Purkinje system. A n t i d romic AVRT is
characterized by a wide. sl urred QRS complex resu l t i ng from
conduction over t he bypass trnct and act ivation of the ven t ri
cle w i t hout use of t h e specia lized conduction system .
Adenosine c2n be given to termi nate ort hodromic AV RT:
however. adenosine or other AV nodal blockers are con t rn i n
clicated in preexcited atrial fibri l lat ion and a n t iclrom ic AV RT.
AV nodal blockade i n patients w i t h t hese rhythms can pro
mote rapid conduct ion down t h e bypass tract and induction
of ventricu lar fi bri l lation (VF) .
Pat ients w i t h evidence of preexci tation on t heir resting
ECG (delta wave) and symptomatic SVT have Wolff-Parkinson
White (WPW) syndrome. Up to one third of patients with WPW
syndrome have or w i l l develop atrial fibri l lation. Rapid con
duction over an accessory pa t hway i n atrial fibri l l a t ion can lead
to VF and sudden cardiac death (SCD). a l t hough t h is is a rela
(To the ventric le)
t ively rare event. Risk factors for VF in WPW syndrome include
documented AVRT. multiple bypass tracts. Ebstein anomaly
F I G U R E 1 7 . Mechanism of typical atrioventricu lar nodal reentrant tachycar· (right heart enlargement and severe tricuspid v;1Jve regurgita
d ia. The slow pathway has a short refractory period, a n d the fast pathway has a tion) . and a rapidly conducting accessory pathway. WPW syn
long refractory period. The blue line represents anterograde conduction down the
drome is often seen in patients with Ebstein anomaly.
slow pathway; conduction does not occ u r down the fast pathway because it is
refracto ry. The yellow line represents impu lse conduction into the ventricle and In genera l . eva lu ation of a pa tien t with preexcitation
retrog rade u p the fast pathway, which is no longer refracto ry. The red line repre· i ncl udes a 12- lead ECG. echocardiogra m , ambula tory ECG
sents completion of the circuit with activation of the atria and ventricles. moni toring. and an exercise stress test . Stress testing is an
54
Arrhyth m i a s
Cl e ffective means or noninvasive risk strat i ficat ion for patients At rial 11bri l lation i s classified a s first-detected. paroxys
with preexci tat ion. Loss of preexcitation duri ng exercise gen mal. persistent, or long-standing persistent atrial fibrillation.
CONT.
erally indicates low risk. Electrophysiology ( EP) test i ng can Paroxysm<1 l atrial fibrillation starts and stops spontaneously.
help determine rapidity of conduction and risk for sudden Persistent atrial fibril lation lasts for 7 clays or more and requires
death : it also can help local ize t he pat hway and facili tate cath elect rical or pharmacologic carc!ioversion. Long-stand ing per
eter ablation. which has a h igh success rate (alt hough success sistent atrial fibri llation is persistent atrial fibril lation t hat is
depends on the locat ion of the bypass t ract) . In general. cath more than J year i n duration.
eter ablation is first- li ne t herapy for patients with preexcita
t ion a nd symptoms. Antiarrhyth mic agents are reserved for Acute Management
second-line t herapy. pa rticularly i n patients with accessory Bot h acute and chronic management or atrial fibri llation are
pat hways in close vicinity to t h e AV node. based on t h ree t herapeutic goals: ( L ) preve n t i ng stroke.
Management of asymptomatic preexcitat ion on ECG is (2) con t roll i ng the heart rate (preventing tachycardia/rapid
con t roversial. I n t he absence of symptoms, however. i nvasive vent ricular rates). and (3) symp to m relief". Once a diagnosis of
testi ng is genera l ly not required. u nless the patient has a h igh at rial fibri l lat ion is made. a search for reversible causes should
risk occupation , such as an airl i ne pilot or bus d rive r. Cl be completed. including an evaluation of t hyroid function .
Pat ients with atrial fibri llation should undergo screening for
KEV POI NTS
sleep apnea with more extensive testing if t he clin ical h istory
• Therapeutic options for prevention o f recurrence o f
is suggest ive. An echocardiogram should be obtai ned to inves
atrioventricular nodal reentrant tachycardia include
t igate potential valvular or other structural heart disease.
atrioventricular nodal blocking drugs and catheter
ablation.
Acute Anticoagulation
• First-line therapy for Wolff-Parkinson-White syndrome In patients wi l h newly discovered atrial fi b r i l lation in whom
(preexcitation with symptoms) is catheter ablation. cardioversion will not be perlormed. i nst itutio n of i n t rave
nous a n t icoagulation is usua l ly not necessary. I n t hese
pat ien ts. oral a n ticoagulat ion can be started based on risk
factors (see Long-Term M a nagement) . I f cardioversion is
Atria l Fibril latio n
pJ,1 11 ned. ant icoagulat ion therapy is based on t h e duration of
Atrial fibrillation i s the most common sustained cardiac at rial l"i bri l lat ion. For patients who a re known to have been i n
arrhythmia. The diagnosis of atrial fibrillation is based upon atrial 11brillat ion fo r less than 4 8 hours, preprocedural a n tico
the demonstration of disorganized atrial activity, seen as an agulation is not necessary as the risk or t h rombus formation
irregularly irregular ventricular response on ECG. Fibrillation is low. Pat ients with atrial 11brillation of unclear duration or
of the atrial myocardium can lead to stasis and intracardiac t hose with atrial 11bri l la l ion for more t han 48 hours require
thrombus formation. In patients older than 40 years, the life preprocedural ant icoagu lat ion . These patients should receive
time risk of atrial fibrillation is 1 in 4. The incidence of atrial 3 weeks of t herapeutic ant icoagulation prior to cardioversion.
fibrillation is age-related, and more than 10% of persons aged Alternat ive ly. transesophageal echocardiography (TEE) can be
80 years and older have atrial fibrillation. Atrial fibrillation is performed to look f()r a n i n t racardiac t h rombus. If TEE is
associated with a fivefold increased risk of stroke as well as an negative for t h rombus, acute cardioversion can be performed
increased risk of heart failure and dementia. Atrial fibrillation i m mediately. All patients (regard less of t he duration of atrial
can occur secondary to reversible or acute physiologic insults, fibri l lation) must be ant icoagu lated at t he time of cardiover
including hyperthyroidism, cardiac surgery, and pulmonary sion and after carclioversion for a m i n i mu m o f 4 weeks owing
embolism. More commonly, atrial fibrillation is the result of to an i ncreased risk of t h romboembolic events a fter restora
long-standing disease affecting the heart, particularly hyper tion of' sinus rhythm .
tension, structural heart disease, and obstructive sleep apnea.
Cl Clinical Presentation
Cardioversion and Acute Rate Control
Many pat ients who present with an i n it ial episode of atrial
As with most arrhy t h mias, patients wit h atrial libri l lation can fibri l lation convert spontaneously. often w i t h i n hours.
experience a wide range of symptoms. i nclud i ng palpitations, However. the presence or hypotension, myocard ial ischemia,
l ight headedness or dizziness. dyspnea. exercise i n tolerance, or heart lailure is an i ndication for i mmediate cardioversion
chest pain. and syncope. Some patients are asymptomatic a nd rega rdless or the d uration or at rial fibri l lation . Acute cardio
are found to have atrial 11bri l lation as an i ncidental 11nding on version of atrial libril lalion should be synchron ized to the R
ECG. I n its most severe forms. part icularly in patients with wave so as to avoid an " R-on-T" event and provoca tion of V F.
advanced diastolic dysfunction or restrict ive card iomyopathy. Pat ie n t s w i t h rapid ven t ri c u l a r co nduct ion req u i re
atrial fibrillation can resu l t in hemodynamic compromise. heart rate con t rol i n order to i mprove cardiac fu n c t i o n a n d
Some patients i n i t ia l ly present with heart failure caused by symptoms. Ta rget heart rates should be between 6 0 /m i n
tachycardia-i nduced ca rdiomyopathy. a nd l t O / m i n i n t h e acute sett i n g. A c u t e ra te cont ro l is most
55
Arrhyt h m i a s
CONT.
Anticoagulation
m e t o p ro l o l . e s m o l o l . d i l t ia ze m , a n d verapa m i l . c a n b e Stroke is the most concerning consequence of atrial fibrillation.
used , w i t h su bseq u e n t t ra n s i t i o n ro o ra l f'o rm u l,1 l io n s. I n The absolute risk of stroke is 4% per year among patients with
pat i e n t s w i t h m i l d sym p to m s , ora l age n t s can be consid
nonvalvular atrial fibrillation, but comorbiclities can increase the
ered w i t hout i n i t i a l i n t rave nous t herapy. C a l c i u m c h a n n e l
risk 15 to 20 times. Hypertension is an important risk factor for
b l ockers s h o u l d be avo i d e d i n pa t i e n t s w i t h l e f't ven t ricu l a r
both atrial fibrillation and stroke; therefore, aggressive blood pres
dysf'u n c t ion . O i goxi n ca n b e a d ded to i m prove ra te con t ro l ,
sure control is paramount in the management of atrial fibrillation.
espe c i a l l y i n p a t i e n t s w i t h h e a r t fa i l u re. Pa t i e n t s w i t h
Stroke prevention with an ti thrombotic therapies is predi
e v i d e n ce of p reexc i ta t i o n s h o u l d not b e t reated w i t h
cated on a patient's aggregate risk profile. Several risk stratifi
� - b l oc ke rs o r c a l c i u m c h a n n e l b l o c ke rs . I n pa t i e n t s
cation scores are available to clinicians. In patients with
w i t h preexc i t ed a t r i a l fi b r i l l a t ion , p roca i na m i d e i s t h e
t re a t m e n t of cho ice. nonvalvular atrial fibrillation, the CHADS 2 score was, until
I f' cardioversion i s favored because or sign i ficant symp recently, the basis for most guideline and consensus docu
toms d espite rate con t ro l , pharmacologic or e lectrical ca r ments. Owing to the limited ability of the CHADS 2 score to
d i oversion can be p ur s ued . Class IC age n ts ( fleca i n i d e , discern between low and intermediate risk, the CHA2 DS 2 -
propa fenone) or i bu l i l ide (an i n t ravenous class I l l m e d i ca VASc risk score was developed and now is the recommended
t io n ) can be considered for pharmacologic cardioversion i n score to assess risk of stroke in patients with nonvalvular atrial
pa t ie n t s without structural hea rt d i sease. Cl fibrillation (Table 26) . The CHA2 DS 2 -VASc score is particularly
TABLE 26. Risk Stratification Scores, Adjusted Stroke Rates, and Antithrombotic Therapy Recommendations
Score Incidence of lschemic Stroke Antithrombotic Therapyb
(per 1 00 patient-years)•
CHADS2 Score<
0 0.6 Aspirin or no therapy
1 3.0 Aspirin or OAC
2 4.2 OAC
3 7.1 OAC
4 1 1 .1 OAC
5 1 2 .5 OAC
6 1 3 .0 OAC
0 0.2 None
1 0.6 None or aspirin or OAC
2 2.2 OAC
3 3.2 OAC
4 4.8 OAC
5 7.2 OAC
6+ 1 0. 3 OAC
aoata from Friberg L, Rosenqvist M, Lip GY. Evaluation of risk stratification schemes for ischaemic stroke and bleeding in 1 82 678 patients with atrial fibrillation: the Swedish Atrial
J. 2 0 1 2 Jun;33( 1 2) o 1 500· 1 0. [PMIDo 222464431
F;br;llat;on cohort study. Eur Heart
bCHADS2 recommendations from Fuster V, Ryden LE, Cannom DS, et al; American College of Cardiology; American Heart Association Task Force; European Society of Cardiology
Committee for Practice Guidelines; European Heart Rhythm Association; Heart Rhythm Society. ACC/AHA/ESC 2006 guidelines for the management of patients with atrial fibrilla
tion: full text: a report of the American College of Cardiology/American Heart Association Task Force on practice guidelines and the European Society of Cardiology Committee
for Practice Guidelines (Writing Committee to Revise the 2001 guidelines for the management of patients with atrial fibrillation) developed in collaboration with the European
Heart Rhythm Association and the Heart Rhythm So6ety. Europace. 2006 Sep;8(9):651 ·745. Erratum in: Europace. 2007 Sep;9(9):856. [PMID: 1 69879061. CHA DS VASc recom·
2
mendations from January CT, Wann LS, Alpert JS, et al; ACC/AHA Task Force Members. 201 4 AHA/ACC/HRS guideline for the management of patients with atrial fribrillation: a
report of the American College of Cardiology/American Heart Association Task Force on practice guidelines and the Heart Rhythm Society. Circulation. 20 1 4 Dec 2; 1 30(23):e199-
267. [PMIDo 246823471
'CHADS2 scoring {maximum 6 points): One point each is given for heart failure, hypertension, age �75 years. and diabetes mellitus. Two points are given for previous stroke/
transient ischemic attack.
d(HA2DS2 -VASc scoring (maximum 9 points): One point each is given for heart failure, hypertension, d iabetes mellitus, vascular disease (prior myocardial infarction, peripheral a rte·
rial disease, aortic plaque), female sex, and age 65 to 74 years. Two points each are given for previous stroke/transient ischemic attack/thromboembolic disease and age 2:75 years.
56
Arrhyt h m ia s
helpful in patients with O or 1 CHADS2 risk factors. In addition bleeding compared with warfarin. Apixaban also i s superior
to the CHADS 2 points, this score gives an additional point for to warfarin for the prevention of stroke and is associated
age 65 to 74 years, female sex, and the presence of atheroscle with less bleeding overall, including intracranial bleeding,
rotic disease, and gives 2 points for age 75 or older. Patients but similar rates of gastrointestinal bleeding. All of the novel
with a CHADS 2 score of 0 or 1 who have a CHA 2 DS 2 -VASc score oral anticoagulants are cleared by the kidneys. Thus, dose
of 2 or more may benefit from oral anticoagulation. Certain adjustment is required based on estimated glomerular filtra
high-risk features, such as mitral stenosis or rheumatic heart tion rate (eGFR) , and these agents are contraindicated in
disease, prior systemic embolism, a prosthetic heart valve, left patients with end-stage kidney disease. For this reason,
atrial appendage t hrombus, and hypertrophic cardiomyopathy annual measurement of serum creatinine level is recom
require oral anticoagulation regardless of risk score. mended for patients treated with these drugs. All of the
For patients who are treated with aspirin, the recom novel oral anticoagulants have shorter half-lives relative to
mended dose is 81 to 325 mg daily. For patients who require warfarin; however, there are no quick, readily available
oral anticoagulation, several agents are now available. Dose serum assays to accurately determine anticoagulant activity.
adjusted warfarin (a vitamin K antagonist) remains an effec Furthermore, currently there is no antidote for these agents
tive low-cost alternative for stroke prevention in patients with in patients with severe hemorrhage.
a higher risk of stroke. The efficacy and safety of warfarin In patients with concomitant coronary artery disease and
therapy are closely associated with the amount of time in the atrial fibrillation, antithrombotic therapy presents significant
therapeutic range (INR 2-3) . The chief limitations of warfarin challenges. For most patients with stable coronary artery dis
are its need for frequent INR monitoring and adjustment and ease, single-agent therapy with an oral anticoagulant is suffi
its numerous food and drug interactions. Recently, several cient for prevention of both acute coronary syndromes and
new oral anticoagulants have been approved by the FDA for stroke events. Combination antiplatelet and oral anticoagulant
the prevention of stroke in patients with nonvalvular atrial therapy increases the risk of bleeding, including intracranial
fibrillation, including dabigatran, rivaroxaban, and apixaban hemorrhage. However, patients with an acute coronary syn
(Table 27) . Warfarin remains the agent of choice in patients drome or revascularization in the previous 12 months are
with valvular atrial fibrillation, generally defined as atrial thought to benefit from combination therapy with low-dose
fibrillation with mitral stenosis or mitral valve replacement. aspirin (<100 mg/d) and oral anticoagulation. In patients who
Dabigatran is superior to warfarin for the prevention of receive a coronary stent, triple therapy with low-dose aspirin
stroke and is associated with less intracranial bleeding, but (<100 mg/d), a thienopyridine (such as clopidogrel) , and war
carries a higher risk of gastrointestinal bleeding. Rivaroxaban farin is indicated for as short a period as possible. In patients
is noninferior to warfarin for the prevention of stroke or with a drug-eluting stent, this period may extend to 6 months
systemic embolism and is associated with less intracranial or a year. Ongoing clinical trials are evaluating the combina
and fatal bleeding. Similar to patients receiving dabigatran, tion of anticoagulant therapy for atrial fibrillation and anti
patients on rivaroxaban have a higher risk of gastrointestinal platelet agents for coronary artery disease.
Warfa rin (vita m i n K Yes Dosing adjusted to I N R Va lvular or nonvalvu l a r Avoid i n pregna ncy. Caution with
antagonist) id iopathic throm bocytopenic
pu rpura, H IT, hepatic disease,
protein C or S deficiency. M a ny
d rug interactions.
Rivaroxaban (factor Xa No• Once daily Nonvalvu lar Avoid with CrCI <30, moderate
i n h i bitor) hepatic disease. Caution with
m i l d hepatic d i sease
Apixa ban (factor Xa No• Twice daily Nonvalvu l a r Avoid with severe hepatic
i n h i b itor) disease, strong dual i n h i b itors
or inducers of CYP3A4 and
P-glycoprote i n . Caution with
moderate hepatic disease.
AF = atrial fibrillation; CrCI = creatinine clearance {mUmin/1 .73 m2); HIT = heparin�induced thrombocytopenia.
"Early data suggest that factor Xa inhibitors may be able to be reversed with prothrombin complex concentrates. Additionally, several "antidotes" are in development for the factor
Xa inhibitors.
57
Arrhyth m i a s
Rate Versus Rhythm Control guided by risk factors. I n patients with symptomatic atrial
There is no evidence of a survival advantage or reduction in fibrillation who are undergoing cardiac surgery for other rea
stroke with restoration and maintenance of sinus rhythm in sons, the maze procedure can be performed as a means of
patients with atrial fibrillation, including those with heart maintaining sinus rhythm.
failure. Therefore, the decision to institute a rate or rhythm Patients with refractory symptomatic tachycardia despite
control strategy largely depends on symptoms and patient attempts at rate and rhythm control may be candidates for AV
preference. Patients who are asymptomatic can be managed node ablation. In this approach, patients receive a pacemaker
with rate control only, with a resting heart rate goal of less than and undergo therapeutic ablation of the AV node, rendering
110/min. Patients with tachycardia-induced cardiomyopathy, them pacemaker-dependent but no longer tachycardic. These
heart failure, or left ventricular ejection fraction of less than patients remain in atrial fibrillation and still require stroke
40% may require more stringent rate control (heart rate 60-80/ prevention therapy.
min at rest) . AV nodal blockers, including �-blockers and
KEY POI NTS
nondihydropyridine calcium channel blockers, can be used to
• All patients with atrial fibrillation who u ndergo cardio
control the heart rate. Combination therapy is often required
version require anticoagulation therapy for a minimwn
to adequately control the heart rate. In addition to assessing
for 4 weeks following the procedure.
the resting heart rate, assessment of the heart rate with activ
ity should be considered, e ither with ambulatory ECG moni • The CHA2DS2-VASc score for estimating stroke risk in
toring, a stress test, or a 6-minute walk test. atrial fibrillation is similar to the CHADS 2 score but bet
In patients who continue to have symptoms despite ter differentiates low- and intermediate-risk patients; in
adequate rate control, a rhythm control strategy should be addition to heart failure, hypertension, age, diabetes
considered to i mprove quality of lite. Rhythm control may mellitus, and previous stroke, the CHA2DS 2-VASc score
require cardioversion followed by antiarrhythmic therapy. incorporates lower age (65-74 years) , sex, and the pres
Antiarrhythmic drug selection is based on patient comorbidi ence of atherosclerotic disease.
ties and the safely profile of the antiarrhythmic drugs. Some • Options for long-term anticoagulation in patients with
patients with infrequent symptomatic atrial fibrillation may atrial fibrillation include warfarin, dabigatran, rivaroxa
not require daily therapy. Patients with infrequent atrial fibril ban, and apixaban; the latter three agents do not
lation and neither structural heart disease nor conduction require blood monitoring and lack the food and drug
disease may benefit from a "pill-in-the pocket" approach, interactions of warfarin, but they are substantially more
whereby patients take a class IC drug (flecainide or expensive.
propafenone) only when they develop an episode of atrial
fibrillation. Patients who follow this approach should be tak
ing an AV nodal blocker or should take one before taking their
"pill in the pocket." The first time this approach is used, it
Atria l F l utter
should take place in a monitored setting to ensure that the U nlike atrial fibrillation, atrial flutter is an organized
patient can safely tolerate the therapy without development of macro-reentrant rhythm with discrete and organized atrial
proarrhythmia or conduction disturbance (for example, post activity on the ECG, usually with an atrial rate of 250/min to
termination pause) . Regardless of the rate or rhythm control 300/min. Although they are distinct rhythms, atrial fibril
strategy used, stroke prevention should be guided by patient lation and atrial flutter are often found in the same patients
risk (CHA 2 DS 2 -VASc score) . because of similar risk factors and pathophysiology.
Episodes of atrial flutter can induce atrial fibrillation
Nonpharmacologic Strategies and vice-versa.
In patients who have refractory symptomatic atrial fibrillation Typical atrial flutter has a sawtooth appearance on ECG,
despite antiarrhythmic drug therapy, catheter ablation with with negative flutter waves in the inferior leads and positive
pulmonary vein isolation is an etlective rhythm control ther flutter waves in lead V1 (Figure 18) . Typical atrial flutter is
apy. Atrial fibrillation ablation is best reserved for patients caused by counterclockwise reentry around the tricuspid
with early atrial fibrillation without evidence of significant left annulus. Atypical flutter can be clockwise or can occur i n
atrial enlargement and those without multiple comorbidities. other locations in the atria, including the left atrium after
The success rates for atrial fibrillation ablation are variable, but atrial fibrillation ablation.
in patients with paroxysmal atrial fibrillation, between 70% In many respects, atrial flutter is managed similar to atrial
and 90% are symptom-free at 1 year. Complications can fibrillation, including stroke prevention. However, owing to the
include intraprocedural or late tamponade, vascular complica atrial rate and the ratio of conduction through the AV node (for
tions, and a 0.5% to 1 % risk of stroke. Patients who develop example, 2:1 or 4:1), rate control of atrial flutter can be difficult
dyspnea months to years after an atrial fibrillation ablation and often requires large doses of AV nodal blockers. Therefore,
may have pulmonary vein stenosis. Anticoagulation is manda atrial flutter is usually managed with a rhythm control strategy.
tory for lhe first 2 to 3 months after ablation, and thereafter is Catheter ablation oflypical atrial flutter is often preferred owing
58
Arrhyt h m i a s
I I
I _l I� !I • ij
Ir-
, J I,
I
1
rr
F I G U R E 1 8 . In this electrocard iogram demonstrating typical atrial flutter, negative sawtooth waves are seen in the inferior leads and positive waves are seen in lead V 1 . I n
the bottom rhythm strip, 2 : 1 and 4 : 1 conduction patterns are seen.
59
Arrhyt h m i a s
therapy. Antiarrhythmic drug t herapy c a n also be used when and susta ined VT/V F should u n dergo !CD implan tation for
PVCs persist despite �-blockade or calcium channel blockade. secondary preve n t ion . In patients wi t h an !CD. if VT recurs
EP study and catheter ablation can be considered in patients desp i te � - blocker therapy. a n t ia rrhy t h m i c d rug therapy
who cannot tolerate medical therapy or i f medical t herapy fails should be consi dered . In most patients wi t h s t ructural
to suppress the PVCs. heart disease. a m iodarone is fi rst - l i ne a n l i a rrhy t h m i c d rug
F I G U R E 1 9 . An electroca rdiogram demonstrating episodes of monomorphic ventricular tachycardia in a patient with cardiac sarcoidosis. Note that the repeated wide·
complex beats do not resemble a typical bundle branch block pattern. There are several sinus beats (na rrow com plexes) that help ascerta i n the etiology of the wide beats as
ventricular tachyca rdia.
60
Arrhyt h m i a s
Long QT syndrome Syncope, QTc interval usually >460 msec, torsades �-Bl ockers, ICD, exercise restriction
de poi ntes
Short QT syndrome Syncope, QT interval <340 msec, atrial fi bril lation, ICD i n all patients
VT, VF
Catecholam i n ergic polymorphic VT Syncope, polymorphic or bidirecti onal VT d u ring �-Blockers, ICD, exercise abstinence
exercise or emotional d i stress
Early repola rization syndrome Syncope, inferior and lateral early repolarization on ICD
ECG, VF
ARVC/D Syncope, T-wave i nversions in leads V1 to at least V3, ICD, �- b l ockers, antiarrhythmic
monomorphic VT, a b n o rm a l signa l-averaged ECG, medications, exercise abstinence
frequent PVCs, and abnormal right ventricular size
and function on echocardiography or CMR imaging
ARVC/D = arrhythmogenic right ventricular cardiomyopathy/dysplasia; CMR = cardiac magnetic resonance; ECG = electrocardiography; ICD = implantable cardioverter·
defibrillator; PVCs = premature ventricular contractions; QTc = corrected OT interval; VF = ventricular fibrillation; VT = ventricular tachycardia .
.,Treatment recommendations for ICDs in inherited arrhythmia syndromes are guided by risk stratification with criteria that are often disease specific. Additionally, antiarrhythmic
drugs are often required in several syndromes for recurrent ventricular arrhythmias.
61
Arrhyt h m i a s
n� :v;J� v---J
1 r rr
J �r.,-v-1.f � ;-v-1
�--r �� r\/'1 i v 1 v 11 �:
_ _ __
F I G U R E 2 0 . In this electrocardiogram demonstrating a type 1 Brugada pattern, �2 111 111 J-point elevation, coved ST-segment elevation (arrowheads), and T-wave inversions
are seen i n leadsV1 to Vr
cardiac arrest usually provoked by high-adrenergic states, accelerate disease progression and arrhythmogenesis. Patients
including strong emotion and exercise. Patients with this dis with ARVC/D and cardiac arrest or risk factors (nonsustained
order usually have provocable arrhythmias with exercise or VT, inducible VT) are offered !CD placement. �-Blockers are
epinephrine infusion. Treatment includes �-blocker therapy first- line therapy for ventricular arrhythmia; however, antiar
and often !CD placement. Patients with the disorder should rhythmic therapy with sotalol or amiodarone or catheter
abstain from exercise. ablation is often required for recurrent VT.
In patients with unexplained VF arrest, particularly when
provoked during exercise, early repolarization syndrome
should be considered. Whereas early repolarization (J-point Sudden Ca rdiac Arrest
elevation) is a common and benign finding on ECG, the pres Epidemiology and Risk Factors
ence of inferior and lateral early repolarization more than 1 SCD is defined as instantaneous death or sudden collapse
mm in a patient with VF or cardiac arrest should be consid within 1 hour of symptoms. Unwitnessed death is considered
ered early repolarization syndrome. Jn patients with VF or SCD if the patient was known to be well within 24 hours of the
cardiac arrest, !CD implantation is indicated. event. Most episodes ofSCD are caused by ventricular arrhyth
Hereditary structural heart disease, such as hypertrophic mias (VT/VF arrest) . In the general population, the risk of SCD
cardiomyopathy (see Myocardial Disease) or arrhythmogenic is 1 / 1000 per year. The incidence is greatest in patients with
right ventricular cardiomyopathy/dysplasia (ARVC/D) , often preexisting structural heart disease; however, most episodes of
manifests as sudden cardiac arrest in a young person. ARVC/D SCD occur in patients with normal left ventricular function.
is characterized by fibrous and flbro-fatty changes of the right Risk factors for SCD include (but are not limited to) heart fail
ventricle and subsequent ventricular arrhythmias. Penetrance ure. diminished left ventricular function. prior myocardial
is variable and age-related, with many patients presenting infarction, unexplained syncope, left ventricular hypertrophy,
between puberty and young adulthood. Patients with ARVC/D nonsustained ventricular arrhythmia, chronic kidney disease,
usually have ventricular ectopy or monomorphic VT, although and obstructive sleep apnea.
patients with severe disease may have heart failure. The diag
Cl
nosis of ARVC/D is guided by diagnostic criteria that include Acute Management
ECG abnormalities, family history, the presence of arrhyth Patients with cardiac arrest require immediate cardiopulmo
mias, and structural abnormalities of the right ventricle as nary resuscitation (CPR) and advanced cardiac lite support.
seen on cardiac imaging_ A RVC/D is usually progressive, and The two most impo11ant i n terventions for patients in cardiac
those with ARVC/D should abstain from exercise. as it may arrest are h igh-quality CPR chest compressions and rapid
62
Arrhyth m i a s
Cl clefibri l lation in pat i ents with VT/VF arrest. Basic l i fe support Patients w i t h modern ICDs have few li m itations. I n gen
guideli nes now emphasize t he acronym CAB (Chest compres era l , l ight to moderate exercise. i ncluding sexual intercourse, is
CONT.
sions. A i rway. Breat h i ng) lo high l ight the importance of permi sible and is associated with i mprovement in cardiovas
i m mediate, rapid. and sustained chest compressions and de cular healt h and q ua l i ty or l i fe. However. some d isorders carry
emphasizi ng assisted breath i ng. Once a code has been cal led or speci fic restrictions (see Table 28) . Patients with ICDs should
t he emergency medical system has been activated and a n au to avoid st renuous upper extremity exercises. i ncluding weight
mated external defibri l lator has been requested. t h e pat ient's l i ft i ng. because t hese activities can damage the leads coursing
pulse should be checked i m mediately. I f no defi nite pulse is t h rough t he chest. Electromagnetic i n terference can lead to
detected w i t h i n l O seconds, chest compressions should begin inappropriate detection of VT/VF and shocks: t herefore, patients
without delay. In pat ients with VT/VF. time to defibri l la t ion is an should avoid l a rge sources of electromagnetic i n terference,
i mportant determinant of t he l i ke l ihood of survival to hospital including arc welding and h igh -voltage machi nery. During sur
discharge. Therefore. when a shockable rhy t h m is present. defi gery. ICDs may need to be reprogram med or have a magnet
bri l lat ion should be performed as rapid ly as possible. applied to avoid false detection of VT/VF clue to electrocaute1y.
Once CPR has been started. the 2 0 1 0 American Heart For this reason . patients with ICDs should have an evaluat ion or
Association guidel i nes on CPR and emergency cardiovascular device program ming recom mendation from t heir electrophysi
care dictate management based upon the presence or absence of ologist before u ndergoing i nvasive procedures or surgery.
a shockable rhythm. In patients with asystole or pulse less electri Patients who experience shocks need to contact their ICD
cal activity (PEA ) . CPR is continued with reassessment of rhythm physician . Patients who experience more than one shock in
status for a shockable rhythm every 2 mjnutes. Epinephrine (I mg 24 hours or any shock accompanied by dyspnea. chest pain. syn
intravenously) should be given eve1y 3 to 5 mi nutes. alt hough cope, or heart failure �ymptoms require emergency medical care. Cl
vasopressin (40 units i ntravenously) can replace the fi rst or sec
KEY POINT
ond close of epinephrine. Atropine is not recommended for the
• Implantable carclioverter-defibrillator placement is
t reatment of asystole or PEA arrest. Further management or PEA
arrest should include ascertainment and t reatment of a ny cor inclicated for secondaiy prevention in patients with sus
rectable etiology (for example. tamponacle) . In patients with VT/ tained ventricular arrhythmias (>30 sec) or cardiac
VF, a shock is advised with i mmediate resumption or CPR and arrest without a reversible etiology.
reassessment of t he rhythm in 2 m i n u tes. Epinephrine should be
given after t he second shock and every 3 to 5 m i nutes thereafter.
Device I nfection CJ
I f VT/VF continues despite three shocks and epinephrine. am i Betvveen 1993 and 2008. the use of cardiac i mplanted electronic
odarone should be given as a bolus. devices i ncreased by 96%. As a resu l t . the number of patients
Patients w i t h symptomatic bradycard i a and hemocly suscept ible to device in fecl ion seen in c l i n ical practice has
namic distress should first be t reated w i t h atropine. I f a t ropine increased dramat ical ly. Device i n fect ions range from i n fections
is i neffect ive. dopa m i n e or epinephrine i n rusions can be i nvolving t he site of device placement (pocket in fection) to
a ttempted u n t i l transcutaneous pacing or a tempora ry pacing in fective enclocard i tis. Most device in fections are clue to staphy
w i re (pre ferred) can be implemented. lococcal i n fect ions. part icularly Staphylococcus epidermidis
Post-resusci tation care i ncludes t herapeut i c hypot herm ia and S. a u reus. When caring for patients with cardiac implan ted
i n patients who remai n comatose. Compl ica t ions o f t herapeu electronic devices who present with symptoms of i n fection .
t i c hypothermia i nclude ven t r i c u l a r a rrhy t h mias during c l i n icians must have a h igh suspicion f o r device i n fection.
rewar m i ng and i n fectious com p li c a t ions. i nclud i ng sepsis. Patients with cardiac device i n fect ion can presen t w i t h
Hemoclynamics and oxygenation should be opt i m ized in the fever. ch i l ls. a n d malaise. T h e physical exam i nation may reveal
post-a rrest sett i ng. Moderate glycemic control is also recom eryt hema. pocket swel l i ng. and d ra i n age from t h e pocket .
mended. Pat ients w i t h evidence of acute coronary syndrome Laboratory findi ngs frequen t ly include anemia, leu kocytosis.
should undergo i m mediate cat heterization and revascu la riza and an elevated eryth rocyte sed i mentation rate. In pat ients
tion provided t here are no con t ra i ndications. with suspected device i n fection, m u l t iple blood cultures should
be d rawn . Echocardiography ( most often with t ransesophageal
Device Therapy for Prevention echoca rcl iography) should be performed to ident i fy i n t racar
of Sudden Cardiac Death d iac or lead vegetat ions. The device pocket should never be
Pat ients w i t h sustai ned ven t ricu l a r arrhy t h m ias or cardiac asp i rated lor d i agnostic purposes because puncturing the
arrest w i t hout a reversi ble etiology have a class I recom menda pocket can damage the leads or i n t roduce i n fection.
tion tor secondary prevent ion ICD placement. I n patients w i t h Once a cardiac device in fection is d i agnosed. t reatment
st ructural heart d i sease w h o meet specific criteria, ICDs a rc includes complete removal of a l I hardware. debridement of t he
i ndicated for prima1y prevent ion (see Heart Fa i lu re) . ICD bat pocket, sustai ned a n t i biot ic t herapy, and rei mplantation at a
tery l i fe is approx i mately 7 lo 1 0 years but is variable. A l t hough new site ( i f and when appropriate) . Suppressive a n tibiotic
ICD malfunction is rare. when i t occurs. it is often clue to a t herapy w i t hout complete removal of' t he device is not curative
problem with the i n t racarcliac leads. and is associated w i t h a h igh fata l i ty rate. Cl
63
Pe r i c a rd i a ( D i sease
Acu t e perica rd i t i s m<1y occur as part or a s 1stc111i c d i sorder or d u ri n g a held end e x p i ra t i o n w i t h t he pa t ie n t lea n i ng ifJ rwa rd.
in i so l a t i o n . /\ I t hough t here a re m a ny potcn t i<1 l causes o r acute This m a neuver a l lows d ist i nct ion f'rom a pleuropericarc l i a l or
pe rica rcl i t is . most cases a rc id iopa t h ic o r presum ed to be v i ra l pleu ra l rub. \Nh ich arc p resen t o n ly d u ri n g resp i ra t ion .
F I G U R E 2 1 . El ectrocardiographic changes of acute pericarditis. Con cave ST-segment elevation is present in most of the leads (arrowheads). The PR segment is depressed
i n all leads except aVR (arrows).
64
Pe ricard i a ! D i sease
TAB LE 29. Electrocardiographic Features for Differentiating Acute Pericarditis from Myocardial lschemia
Feature Acute Pericarditis Myocardial lschemia
Evo l ution ST-segment c h a n g e initially, then T-wave change i n itial ly, then
T-wave change ST-segment change
QT prolongation No Yes
65
Peri card i a ! Disease
Cl states that a re pote n t i a l ly a menable to such t herapy ( for The jugu lar venous pressure is elevated i n nearly all patients.
example. autoi m m u ne d i sorders. u remic pericardi tis) . A with prominent x and y descents. Physical findings t hat also
CONT.
3-month course o f' predn i sone ( 0 . 25 to 0 . 50 mg/kg daily may be present include a Kussmaul sign (jugular vein engorge
starti ng dose) may be used in t hese circumstances. with a ment with i nspiration) . pericardia! knock. pulsus pa radoxus.
slow taper beginning at 2 to 4 weeks. Recurrent pericarditis pleura l e ffusion. congestive hepalomegaly. and peripheral
has been reported to be more com mon among pa tients pre edema or asciles. In patients with long-standing constrict ive
scribed glucocorticoids. but t hese reports have been ham pericarditis. hepatic fai lure a nd cirrhosis may be present.
pered by t he t,e ndency to use t hese agents i n patients whose The diagnosis of constri c tive pericard i t is can be made
pericarditis has been refractory to other t herapies. Thus. glu with a deta i led hemodynamic evaluation using e i t her
cocort icoids are sti l l considered to be an effect ive al ternal ive Doppler echocardiography or cardi ac cat heterizat ion . The
t he rapy for these patients. basis for the d iagnosti c hemodynamic fi ndi ngs i n constric
A fter an episode of'acule pericard it is. as many as 30% o r t ive pericarditis i s the concept o f en hanced ventricu l a r i nter
patients develop recurre n t pericard i t is. There is a low (<l 'Y.. ) depen dence. which classical ly resul ts i n equalization o f' d ias
risk of' developing constrict ive pericardi tis. Recurre n t peri tol ic pressures i n all heart chambers. The nonco m p l i a n t
card it i s can be t reated w i t h a n t i - i n fl a m m a tory agents wi t h pericardium also prevents the complete t ransmission of res
a s lower. longer taper ( for example, 4 m on t hs) . t he add i t ion piratory changes i n t horacic pressure to the cardiac cham
of col c h ici ne. or the use o f glucocorticoids. Etiologies of bers. Fi l l ing of t he right and left ven tricles varies sign i fi ca n t ly
acute perica rd i t is ot her t han v i ral or idiopa t h i c causes with respiration owing to ma rked changes i n t he early dias
should be suspected . Cl tolic gradient emptying i n to t hese chambers ( t hat is. disso
ciation of l horacic-cavitary pressures) . D uring i nspira t ion,
K EY P O I NTS
t he decrease i n thoracic pressure leads to relat ively less left
• Echocardiography should be performed i n patients with
ven t ricular f1 1 l i ng. while the i ncrease i n caval blood flow aug
suspected acute pericarditis to evaluate for the presence ments right ven t ricular preload. Reciprocal cha nges i n ven
of a hemodynamically significant pericardia! effusion; t ricular loading occur during expiration (Figure 22) . Because
however, the absence of an effusion does not rule out
the diagnosis.
• First-line therapy for acute pericarditis is high-dose
NSAIDs; colchicine can be used in conjunction with
standard NSAID therapy. 200
66
Pericard i a ! Disease
Cl constrictive pericard i t i s does not i nvolve the ventricular sep D ifferentiation o f constrictive pericard i tis from restrictive
tum, bu lging of t he septum towards t he left occu rs d u ri ng cardiomyopa t hy is c l i n i ca l ly i mport a n t because surgical
CONT. . . . . . .
111sp1rat10n a n d returns toward s t h e ng h t d u ri ng exp1rat1on, t reatment of' constrictive pericard i t is may be curative of t he
leading lo marked e n hancement of ven tricular i n terdepend condition , but t here are no therapies t h a t sign i fi c a n t ly
ence. The dissociation of thoracic and i n t racavitary pressu res improve the natural h istory or restrict ive carcliomyopat hy.
and t h e enhanced ventricular i nterdependence lead to recip Other echocardiographic fi ndings supportive of t he diag
rocal changes in fil l i ng and emptying of the right and left nosis of constrictive pericarcl i t is a re i ncreased pericardia!
ventricles, which manifest as a ltera tions in right- and left t hickness and plet hora of t he i n ferior vena cava. Pericardia!
sided forward stroke volumes during respiration (see Figure t h i ckening. with or without calcification. can also be detected
22) . These fi ndings a re d istinct from restrict ive cardiomyopa with cardiac CT or cardiac magnetic resonance ( C M R ) imaging
t hy, in which t here is not signi ficant enhancement of ven (Figure 23) .
tricular i n terdependence or d issociation of i n t racavitary A subset of patients w i t h chronic constrictive pericarcli
intrathoracic pressures (Table 30) . Restrictive cardiomyopathy tis develop effusive constrict ive pericard itis. There typical ly
is c h a racterized by severe l e ft ven t r i c u l a r diastolic dys is m i ni m a l pericard ia! fluid present in const rictive pericardi
fu nction due to myocardial abnorma l i ties or i nfil trat ion. t is : in some patients. however. perica rd i a ! i n flammation
TABLE 30. Characteristics of Constrictive Pericarditis, Restrictive Cardiomyopathy, and Cardiac Tamponade
Characteristic Constrictive Pericarditis Restrictive Cardiomyopathy Cardiac Tamponade
Mechanism Rigid, inelastic pericardium Myocardial d isease with severe I ncreased i ntra pericard i a l
resulting in fixed card iac d iastolic dysfunction pressure compromises
volume ventricular fil l i n g
Hemodynamic i m p l ications Rapid a n d l i m ited diasto lic Diastolic abnormalities lead to Ventricular fi lling is impaired
fi lling d u e to pericardia! elevated ventricular fi lling with a reduction i n cardiac
restra int; i n creased intracardiac pressures and pu l monary output; jugular ven ous and
fi l l i ng pressures, d i m i n ished hypertension pul monary venous pressures
cardiac output, and vo l u me increased
overload
Physical examination i JVP, Kussmaul sign, i JVP, Kussmaul sign, i JVP, pulsus paradoxus,
pericardia! knock, d i m i nished pericard i a ! knock, d i m i nished hypotension, reflex tachycardia
apica l i m p u lse, congestion a pical i m p u l se, congestion
(peripheral edema, pleural (peripheral edema, pleural
effusion, congestive effusion, congestive
hepatopathy) he patopathy)
El ectrocard iography No characteristic fi ndings No cha racteristic fi n d i ngs Decreased voltage, sinus
tachycard ia, electrical a lternans
Chest radiography Perica rd ia! calcification Atrial enlargement Enlarged cardiac silhouette
("water-bottle" heart)
Echocardiography Pericard i a ! thickening/ M a rkedly d i l ated atria with Pericardia I effu sion, d iastolic
cal cification; respiratory preserved systolic function; collapse of right atrium and
variation in fi l l ing of right a n d severe i m pairment of ventricle, enlargement of
left ventricles; ventricular septa I myocardial relaxation i nferior vena cava, ventricular
sh ift d u ri ng respiration; septal shifting d u ring
plethora of i nferior vena cava respiration, respi ratory
changes in mitral inflow
CMR i magi ng/CT Pericard ia! thickeni ng/ Normal pericard i u m Pericardia! effusion
calcification
Hemodynamic right and left Prom i n entx and y desce nts; Promi nent x and y descents; Promi nent x descents and
heart catheterization elevated atrial pressures; el evated atrial pressures; b l u nted y descents; elevated
elevated and equa lized concordance of LV and RV atrial pressures; blunting/loss
d ia stolic LV and RV pressures pressures d u ring respiration of early ventricular diasto lic
(within 5 mm H g ); decreased fi l l ing wave
transm ission of intrathoracic
pressure causes d i scordance of
LV and RV pressures and stroke
vol u me d u ring respiration
(enha nced ventricular
interdependence)
BNP = B-type natriuretic peptide; CMR = cardiac magnetic resonance; JVP = j ugular venOlJS pressure; LV = left ventricular; RV= right ventricular.
67
Perica rd i a ! Disease
68
Perica rd i a ! Disease
Cl total pericardiectomy, with removal of as much of the pericar pulsus paradoxus ( a decrease i n the systolic pressure of >10
dium as is technically possible, may be achieved. Patients mm Hg with i nspiration) in these patients. As compensation
CONT.
treated with total pericardiectomy have been shown to have for the reduced stroke volume and hypotension. tachycardia
i mproved outcomes relative to those who undergo more l i m is usual ly present.
i ted procedures. Predictors of poor outcome after surgical Less common presentations of cardiac tamponade also
pericardiectomy i nclude advanced age. severe symptoms. need to be considered i n some patients. A loculated pericardia!
chronic kidney disease, pul monary hypertension . left ven effu sion may occur after cardiac surgery or in other postopera
tricu lar dysfunction. and radiation t herapy as t he underlying t ive settings. It may occur in the posterior pericardiaJ space
cause of constrictive pericarditis. In one study, the 7-year sur adjacent to the atria. posing challenges for detection by echo
vival after pericardiectorny was 27%, 66%, and 88%, respectively. ca rdiography. Posterior loculated effu sions should be suspected
for patients with constrictive pericarditis due to radiation. prior i n postoperative patients with hemodynamic instability. Low
cardiac surgery, and an idiopathic cause. Medical therapy with pressure cardiac tamponade occurs without elevated jugular
diuretics can i mprove symptoms or be pal liative in patients who venous pressure because the intracardiac fil l i ng pressures are
are not surgical candidates, but the chronic nature of the disor low. Examples include patients with malignancy or tuberculo
der can prove to be drng-refractory. Cl sis complicated by severe dehydration. Pneumopericardium
with cardiac tamponade may resu l t from gas-forming bacterial
KEY POI NTS
pericarditis after penetrating chest trauma.
• I n some patients with constrictive pericarditis, particu Cardiac tamponacle should be suspected when there is a
larly those with mild symptoms, a potentially reversible compatible h istory, hypotension. and an elevated j ugular
cause of acute inflammation, and no evidence of venous pressure and pulsus paradoxus. An enlarged cardiac
chronic constriction, it may be reasonable to perform a silhouette may be seen on chest racliograph ( "water-bottle
trial of medical therapy before surgery. heart") . The ECG typically demonstrates sinus tachycardi a
• Cardiac surgery with pericardiectomy is the definitive and electrical a l ternans. Echocardiography readily detects
treatment for relief of heart failure in patients with con pericardia! effusions and i s the primary modal i ty for diagnos
strictive pericarditis. ing cardiac tamponade (Figure 24) . Signs of cardiac tarnpon
ade i nclude diastolic col lapse of t h e right atrium and right
ventricle, ven tricular septa! s h i ft i n g w i t h respiration, and
69
Pericard i a ! Disease
70
Va l v u l a r H e a rt Disease
Heart Disease is the key first step in identifying the presence ofVHD and the
likely valve or valves involved and in helping to guide an
Valvular heart disease (VHD) comprises cardiac dysfunction
appropriate diagnostic and therapeutic approach ( Figure 26 ) .
due to structural or functional abnormalities of the cardiac
A thorough cardiac examination entails determining the
valves. Dysfunction results from either failure of the valves to
intensity of the murmur as well as specific characteristics
competently close (regurgitation) or to effectively open (steno
that help define whether it is a clinically significant murmur.
sis) . Causes of VHD have changed markedly in developed
Not all murmurs require further evaluation (specifically
countries over the past 50 years, with a shift away from rheu
echocardiography) .
matic disease and toward calcific, degenerative, and congenital
The clinical grading of both systolic and diastolic mur
causes. Rheumatic heart disease continues to be the primary
murs is based on a scale of 1 to 6 (Table 31 ) . Diastolic mur
cause of VHD in the developing world.
murs are always considered abnormal and require further
The effect of VHD on the heart and on the patient varies
evaluation. Systolic murmurs may be either innocent or
based on the affected valve and the mechanism of dysfunction:
indicative of significant valve disease. Some murmurs are
• Aortic stenosis causes chronic pressure overload that typi
associated with normal valves but abnormal systemic pro
cally leads to concentric left ventricular (LV) hypertrophy
cesses (for example, anemia and other high-output states,
with increased wall thickness and normal chamber size to
such as hyperthyroidism) or increased flow (for example, a
compensate for increased LV afterload.
systolic murmur in the setting of aortic regurgitation) .
• Chronic aortic regurgitation causes increased LV preload I nnocent murmurs are characteristically brief and are asso
and afterload, leading to increased LV volume and mass. ciated with normal heart sounds and no hemodynamic
• Mitra! stenosis causes increased pressure within the left abnormalities. Patients with grade 1 or 2 midsystolic mur
atrium (LA), leading to increased pulmonary venous pres murs who are asymptomatic with no associated findings
sure, pulmonary hypertension, and atrial dilation. and those with continuous murmurs suggestive of a venous
• Chronic mitral regurgitation causes volume overload (in hum or mammary souffle (a continuous murmur heard
creased preload) of the LV and LA, leading to increased LA over the breast in lactating women) do not warrant echo
size and pressure and LV dilation. cardiographic evaluation.
The character of a murmur in response to clinical
Many of these changes in cardiac structure occur gradu
maneuvers is helpful in localizing the flow disturbance to a
ally because of physiologic mechanisms that compensate for
specific valve. For example, in general, right-sided murmurs
the increased loading conditions. For this reason, patients
increase with inspiration and left-sided murmurs increase
remain asymptomatic for some time even in the setting of
with expiration. A Valsalva maneuver decreases the length
significant valve disease. The exception to this is acute valve
and intensity of most murmurs, except for systolic murmurs
lesions, in which the compensatory changes do not have suf
associated with hypertrophic obstructive cardiomyopathy
ficient time to develop.
and mitral valve prolapse. Exercise causes the murmur of
mitral stenosis to get louder, whereas isometric maneuvers,
such as handgrip, increase the intensity of regurgitant mur
Diag nostic Eva l uation
murs such as aortic and mitral regurgitation. Postural
of Va lvu l a r Heart Disease maneuvers are ideal for differentiating hypertrophic obstruc
History and Physical Examination tive cardiomyopathy and mitral valve prolapse from other
The initial evaluation of the patient with possible VHD murmurs as they are louder with standing and softer with
serves to diagnose, quantify, and assess mechanisms of squatting. Physical examination findings, such as an enlarged
valve dysfunction. The evaluation should focus on disease or displaced apical impulse, abnormal peripheral pulses, and
severity, symptoms, quality of life, expected benefits of timing and intensity of heart sounds (including extra heart
71
Va l v u l a r H e a rt D isease
Physical examination
Systolic m u rm u r <". grade 3/6
Diasto l i c or conti nuous m u rm u r
Symptoms
Transthoracic echocardiography
Ventric u l a r size and function
Pulmonary pressu res
Measures of disease severity
_f
Transesophageal echocardiography Plasma B-type natriuretic peptide level
Measures of disease severity and/or stress echocardiography
Exercise tolerance
Exercise p u l m on a ry pressures
CT or MRI
Aorta assessment: ascending, arch, descending
Cardiac chamber size and function: ventricular vo l u me
TABLE 3 1 . Clinical Grading of Murmurs clues (such as a dilated left ventricle in the setting of chronic
aortic regurgitation) , but its utility in the evaluation of VHD is
Grade Description
less than other modalities, especially echocardiography.
Fai ntest murmur that can be heard (with difficulty) Transthoracic echocardiography (TIE) with color flow
2 Faint m u rmur but ca n be ide ntified i m mediate l y and spectral Doppler imaging is the primary noninvasive
3 Moderately loud m u rmur means of evaluating cardiac murmurs as well as the primary
means for following the course of VHD. TIE is indicated for
4 Loud murmur associated with a palpable thrill
patients with the following (see Figure 26) :
5 Very loud murmur but cannot be heard without
the stethoscope • Systolic murmurs <':grade 3/6 or late or holosystolic
sounds such as an S 3 or S4 or a systolic ejection click) may TTE permits assessment of LV size and function (systolic
help identify specific valve lesions as well. Common valve and diastolic) , estimation of pulmonary artery pressure (an
lesions and heart murmurs are further described in Table 32. indirect estimate based on the tricuspid regurgitant jet veloc
ity) , and determination of disease severity. Echocardiography
Laboratory and I maging Tests also helps establish a reference point for future comparisons if
Electrocardiography (ECG) and chest radiography are often significant abnormalities are detected. Transesophageal echo
performed during the initial evaluation. ECG provides valuable cardiography (TEE) provides improved image quality over TIE
insight into rhythm (such as the presence of atrial fibrillation) as the probe has only the thin tissue of the esophagus through
and cardiac chamber enlargement. Chest radiography is essen which to image. TEE is indicated for patients with severe dis
tial when interpreting dyspnea or clinical signs of heart failure ease who need further quantification, better identification of
with evidence of cardiac enlargement and pulmonary vascular leaflet involvement (particularly for patients with mitral
redistribution. Chest radiography may also provide secondary regurgitation) , or when poor image quality on TTE limits
72
Va lvu l a r H e a rt D isease
TABLE 32. Valvular and Other Cardiac Lesions aiid'Their Associated Examin�fion Findings
Cardiac Condition Characteristic Location Radiation Associated Severity and
Murmur Findings Pitfalls
Aortic stenosis Mid-systol ic; RUSB Right clavicle, Enlarged, Severe aortic
crescendo- carotid, apex nondispl aced stenosis may
decrescendo a p ical impu lse; S4 ; i nclude decreased
bicuspid valve A2 ; h i g h-pitched,
without cal cification late peaking
will have systol i c murmur;
ejection click d i m i n ished and
fol lowed by delayed carotid
m u rmur upstroke
Rad iation of
m u rm u r down the
descending
thoracic aorta may
mimic mitral
regurgitation
Mitral stenosis Diasto l ic; low Apex (heard best in None Opening snap after Interval between S2
pitched, left lateral s 2 if leaflets mobi le; and opening snap
decrescendo decubitus position) irregular pulse if is short i n severe
atria l fi bril lation mitral stenosis
present
Intensity of murmur
correlates with
transva lvular
gradient
P2 may be loud if
pulmonary
hypertension
present
Mitral regurgitation Systo l ic; holo- or Apex To axilla or back; Systolic click in Acute, severe
late systolic occasiona l ly mitral valve regurgitation may
anteriorly to prolapse; S3; have soft or no
precord i u m apical i m pulse holosystolic
hyperdynamic and murmur, mitral
may be displaced i nflow rumble, S3
if dilated left
ventricle; i n m itral
valve prolapse,
Va lsalva ma neuver
moves onset of
clicks and murmur
closer to S 1 ;
handgrip increases
murmur intensity
73
Va l v u l a r H e a rt Disease
TAB LE 32. Valvular and Other Cardiac Lesions and Their Associated Examination Findings (Continued)
Cardiac Condition Characteristic Location Radiation Associated Severity and
Murmur Findings Pitfalls
Tricuspid H olosysto l ic LLSB LUSB Merged and Right ventricular
reg u rgitation prominent c and v i m pulse below
waves i n jugular stern u m
venous pulse;
Pulsatile, enlarged
murmur i ncreases
liver with possible
d u ring i ns piration
ascites
May be higher
pitched if
associated with
severe pulmonary
hypertension
Pu l m onary stenosis Systolic; crescendo- LUSB Left clavicle Pu lmonic ejection Increased i ntensity
decrescendo click after S1 of murmur with late
( d i m i nishes with pea king
inspiration)
Hypertrophic Systol ic; crescendo- LLSB None Enlarged, Murmur may not be
obstructive decrescendo hyperdynamic present in
cardiomyopathy apical impu lse; nonobstructive
bifid carotid hypertro phic
i m pu lse with delay; cardiomyopathy
increased intensity
d u ring Va lsalva
maneuver or with
squatti ng to
standing
Atrial septa I d efect Systol i c; crescendo- RUSB None Fixed, split S2 ; right May be associated
decrescendo ventricular heave; with p u l monary
rarely, tricuspid hypertension,
inflow murmur including increased
intensity of P2 ,
pulmonary valve
regurgitation
Ventricular septa I Holosysto lic LLSB None Pa lpable thri l l ; M u rm u r intensity
defect murmur increases and d u ration
with ha nd-grip, decrease as
decreases with pul monary
amyl n itrite hypertension
develops
(Eisenmenger
syndrome)
Cyanosis if
Eisenmenger
syndrome develops
A2 = aortic valve component of 52; LLSB = left lower sternal border; LUSS = left upper sternal border; P2 = pulmonary valve component of 52; RLSB = right lower sternal border;
RUSB = right upper sternal border.
74
Va l v u l a r H e a rt Disease
image interpretation. The disadvantage compared with TIE is and a history of coronary artery disease, suspected myocardial
that it is invasive, requiring sedation and technical expertise ischemia, or LV systolic dysfunction; in men older than 40
distinct from TIE. years and postmenopausal women; in patients with one or
For stenotic lesions, Doppler-derived velocities across the more cardiovascular risk factors; and in patients i n whom
affected valve allow calculation of pressure gradients and valve mitral regurgitation is thought to be caused by LV dysfunction.
area. Based on the conservation of mass principle, the meas These patients may require concomitant revascularization at
ured flow through one heart chamber region and measured the time of surgery.
velocity of blood across the region (stenotic valve) of interest For patients in whom symptoms are equivocal, adjunctive
can be used to calculate the area of the steno tic valve. evaluation (such as B-type natriuretic peptide measurement,
Assessment of severity for regurgitant valves includes exercise evaluation for tolerance/symptoms, and blood pres
color Doppler jet size and the width of the narrowest segment sure response to exercise) , assessment of pulmonary artery
of the regurgitant jet (vena contracta). Additional quantitative pressures, and LV outflow tract gradient (by TIE or cardiac
measures for regurgitant lesions, such as effective regurgitant catheterization) may be helpful.
orifice area, regurgitant volume, and proximal isovolumic
KEY PO I NTS
surface area, although technically more demanding to acquire,
are strongly associated with prognosis and should be meas • Patients with grade 1 o r 2 midsystolic murmurs who are HVC
ured routinely in patients with moderate or severe regurgitation asymptomatic with no associated findings and those
by color Doppler jet size. Three-dimensional echocardiography, with continuous murmurs suggestive of a venous hum
both transthoracic and transesophageal, provides additional or mammary souffle do not warrant echocardiographic
information such as feasibility of repair (rather than replace evaluation. •
ment) and location of paravalvular leaks. Additional imaging, • Transthoracic echocardiography is indicated for the
such as cardiac magnetic resonance (CMR) imaging or multi evaluation of valve disease i n patients with systolic
detector CT (MDCT) , may provide information on LV func murmurs grade 3/6 intensity or greater; late or holosys
tion, aortic dimensions, chamber sizes and volumes, and tolic murmurs; diastolic or continuous murmurs; or
coronary anatomy. Consistency of quantitative evaluation is any murmur with accompanying symptoms.
important. Simple measurements, such as LV outflow tract
diameter, may vary greatly in the same patient when obtained
at different times and by different operators. These differ
Genera l Pri nciples of Ma nagement CJ
ences may affect valve area calculations and influence timing of Va lvu l a r Heart Disease
of surgery. Treatment is guided by valve lesion severity, which can be
Cardiac catheterization can provide important hemody classified into four stages (A to D) in a system similar to that
namic information, especially when the severity of the valve used for patients with heart failure (Table 33) .The mainstay of
disease as measured by noninvasive testing is not consistent treatment for VHD. both stenosis and regurgitation, is cen
with symptoms or physical examination severity, or when tered on interventions to treat the mechanical problem. The
accurate assessment of pulmonary pressures or detection of goals of t reatment are both to improve symptoms and reduce
intracardiac shunts is needed. Coronary angiography is rec the risk of complications such as i rreversible ventricular dys
ommended before valve surgery in patients with severe VHD function, pulmonary hypertension, and death. The most
C2: Asymptomatic patients with severe VHD, with decompensation of the left or right ventricle
D Symptomatic Patients who have developed symptoms as a result of VHD
severe
Reprinted with permission of Elsevier. Science a n d Technology Journals, from Nishimura RA, Otto C M , Bonow RO, e t al. 2 0 1 4 AHA/ACC guideline for the management o f patients
with valvular heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiel.
2 0 1 4 Jun 1 0;63(22):2438-88. (PMID: 246031 92]; permission conveyed through Copyright Clearance Center, Inc.
75
Va lvu l a r H e a rt Disease
Cl other
com mo n i ndica t i o n for t reatment is sy mptoms (Table 34) but preve n t i ng progression of aortic stenosis. have not been
fac tors. i ncluding chamber clys f'u nct ion . chamber shown to be usef'u l .
CONT.
enlargemen t . a ncl pulmonary hypertension may be i nd ica T h e pri mary therapy f·o r patients with severe V J-I D is valve
tions lor t reat men t. I n genera l . for bot h stenotic and regu rgi repa i r or valve replacement w i t h ei t h er bioprosthelic or
t a n t lesions. symptoms occur before t h e development of' mechan ical valves. For patients with h igh surgical risks, per
ven t ricular dysfunction : however. some pat ients may have cutaneous techni ques for aortic valve replacement and m i t rnl
asy mptomatic ventricular dysf'unction . Medical t herapy does valve repair a re now ava i lable.
not stall the progression of' disease but is indi cated lor patients r:ollow-u p is essen tial in asymptomatic patients. as dis
with symptoms and LV dysf'unction who are awa i t i ng valve ease progression may occur over t i me (Table 35) . After valve
repai r or replacement, as wel l as for those who are not opera repair or replacement. pat ients should ideally be followed i n
t ive cand idates. Preve n t ive measures, such as s t a t i n s lor conj u nction with a cardiovascular special ist. Cl
M itra I stenosis Symptoms (class I ) Percuta neous bal loon valvotomy (if anatomy
2 favora ble by echocardiography with less than
Very severe mitral stenosis ( MVA < 1 .0 cm ) a n d
moderate mitral regurg itation and no left atrial
no symptoms i f valve morphology favora ble for
thrombus)b
bal loon va lvotomy (class Ila)
M itral valve replacement
Severe mitral stenosis attime of other cardiac
surgery (class I)
Mitral regurgitation Symptoms (class I) M itra I valve repa ir if anatomy favora ble (presence
of a n n u l a r d i l ation, mitral leaflet prolapse, or
LVEF 30%-60% (class I)
myxomatous cha nges without cal cification or
LV end-systolic dia meter �40 mm (class I ) stenosis)
Tricuspid regurgitation Severe tricuspid regurgitation at time of surgery Tricuspid valve repair if anatomy favo ra ble
for left-sided valve (class I)
Tricuspid valve replacement ( b i oprosthetic)
Symptoms due to severe, primary tricuspid
regurgitation not responsive to medical therapy
(class I l a )
LV = left ventricle; LVEF = left ventricular ejection fraction; MVA = mitral valve area; PA = pulmonary artery.
bAll patients considered for percutaneous balloon mitral valvotomy should undergo transesophageal echocardiography to assess for left atrial appendage clot and mitral regurgi
tation severity regardless of whether patient has sinus rhythm or atrial fibrillation.
Recommendations from Nishimura RA, Otto CM, Bonow RO, et al; American College of Cardiology/American Heart Association Task Force on Practice Guidelines. 2 0 1 4 AHA/ACC
guideline for the management of patients with valvular heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force
on Practice Guidelines. J Am Coll Cardiol. 20 1 4 Jun 1 0;63(22):2438-88. Erratum in: J Am Coll Cardiol. 2 0 1 4 Jun 1 0;63(22):2489. [PMIO: 24603 1 92]
76
Va lvular H e a rt D i s e a s e
Mitral Stenosis
Stenosis severity M i l d and mod erate ( MVA > 1 .5 cm 2 , Cli nical eval yearly; echo every 3-5 y
d iasto l ic pressure half-time < 1 50 msec)
Severe ( MVA S:1 .5 cm 2 , d iasto l i c pressure Clinical eval yearly; echo every 1 -2 y for
ha lf-time � 1 50 msec or �220 msec with MVA 1 .0- 1 .5 cm 2 , every year for MVA
very severe stenosis, PASP >30 m m H g ) < 1 .0 cm 2
Aortic Regurgitation
Regurgitation severity; rate of progression; M i l d (VC <0.3 cm, E R O <0. 1 0 cm 2 , C l i nical eval yearly; echo every 3-5 y
LV ejection fraction; LV chamber size; RV <30 m Ubeat, RF <30%); normal EF
ascending aorta d i l ation if bicuspid aortic
Moderate (VC 0.3-0.6 cm, ERO 0. 1 0- C l i nical eval yearly; echo every 1 -2 y
valve
0.29 cm 2 , RV 30-59 m Ubeat, RF 30%-49%)
Severe (VC >0.6 cm, ERO >0.3 cm 2 ,
RV �60 m Ubeat, RF �50%)
EF �50%; LVESD S:50 mm C l i nical eval every 6- 1 2 mo; echo every
6- 1 2 mo, more frequently for d i l ating LV
EF <50%; LVESD >50 mm C l i nical eval every 6- 1 2 mo; echo every
6- 1 2 mo, more frequently for d i l ating LV
Mitra! Regurgitation
Reg u rgitation severity; rate of progression; At risk (VC <0.3 cm) Clin ica l eval yearly; echo only if
EF; LV cham ber size symptomatic
M i l d and mod erate (VC <0.7 cm, ERO Clini cal eval yearly; echo every 3-5 y for
<0.40 cm 2 , RV <60 m Ubeat, R F <50%) mild severity, every 1 -2 y for moderate
severity
Severe (VC �O. 7 cm, ERO �0.4 cm 2 , Clin ical eval every 6- 1 2 mo; echo every
RV �60 mUbeat, RF �50%) 6-1 2 mo, more frequently for d i lating LV
AVA = aortic valve area; echo = echocardiography; EF = ejection fraction; ERO = effective regurgitant orifice; eval = evaluation; LV = left ventricle; LVESD = left ventricular end-sys-
tolic dimension; MVA = mitral valve area; PASP = pulmonary artery systolic pressure; R F = regurgitant fraction; RV = regurgitant volume; VC = vena contrada width; Vmax = maxi-
mum aortic jet velocity.
Recommendations based on Nishimura RA, Otto CM, Bonow RO, et al; American College of Cardiology/American Heart Association Task Force on Practice Guidelines. 2 0 1 4 AHA/
ACC g uideline for the management of patients with valvular heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Task
Force on Practice Guidelines. J Am Coll Cardiol. 2 0 1 4 Jun 1 0;63(22):2438-88. Erratum in: J Am Coll Cardiol. 2 0 1 4 Jun 1 0;63(22):2489. [PMID: 24603 1 92]
77
Va lvular H e a rt Disease
Calcific aortic stenosis is a disease of the elderly, and coro aortic jet velocity (V ma.l below 4 mis have moderate aortic
nary artery disease and hypertension are common in these stenosis and medical therapy is appropriate. Some patients
patients. Patients may present with heart failure symptoms, with true aortic stenosis do not have contractile reserve and no
usually associated with preserved ejection fraction. Some change in LV function or aortic valve gradients is seen with
patients present with decompensated heart failure associated dobutamine stress. These patients have a worse prognosis. A
with reduced cardiac output. The chronic increase in LV sys lack of contractile reserve is an indicator of higher surgical
tolic pressure results in a compensatory increase in myocardial mortality but is not in itself a contraindication to surgery.
cell mass; interstitial fibrosis may follow, leading first to dias Paradoxic low-gradient aortic stenosis is a more recently
tolic dysfunction and then to systolic dysfunction. Increased described entity in which the measured aortic valve area is
LV end-diastolic pressure results in pulmonary congestion. reduced in a setting of preserved ejection fraction (>50%) but
These and other adaptive and pathologic processes contribute reduced stroke volume (<35 mL/m2) . Typically encountered i n
to the three cardinal symptoms of aortic stenosis: angina, t h e elderly, i t i s associated with factors related t o reduced
dyspnea, and syncope. Identification of the symptomatic stroke volume, such as small LV size and marked LV hypertro
patient is crucial; in the absence of symptoms, patients with phy. This may cause the severity of a011ic stenosis to be under
aortic stenosis- even severe aortic stenosis- have a low risk of estimated by echocard iography owing to the low gradient.
mortality, with a risk of sudden death estimated to be less than Identifying these patients-who are usually symptomatic-is
2
1 %. The rate of progression is approximately 0.1 cm annually. important, as these are patients who may benefit from aortic
Although variable, symptoms of heart failure, angina, or syn valve replacement.
2
cope generally begin once the valve area is below 1 . 0 cm . Once
symptoms develop, prognosis is poor without valve replace Management
ment, with an average survival of less than 10% over the I n asymptomatic patients with aortic stenosis, identifying
next 2 to 3 years. those who are in higher risk subgroups is important, as these
TIE is essential in the initial diagnosis and follow-up of patients may benefit from earlier intervention. Exercise tread
aortic stenosis, permitting quantification of valve area and gra mill testing is reasonable in asymptomatic patients to identify
dients (see Table 35) , chamber size, wail thickness, ventricular those who actually do have symptoms with exercise, have
function, and coexistent valve disease. Echocardiographic find ST-segment changes on ECG, or have an abnormal blood pres
ings for severe aortic stenosis include a heavily calcified aortic sure response (lack of i ncrease in systolic blood pressure by at
valve with restrictive leaflets, possibly a bicuspid aortic valve, least 20 mm Hg above baseline) , as these patients may benefit
and associated aortic regurgitation. TEE can provide additional from earlier surgery. Patients must be closely monitored dur
information regarding leaflet anatomy (bicuspid, unicuspid) ing exercise testing. Additional factors that may predict more
when TIE images are inadequate as well as information about rapid progression and thus indicate closer follow-up include
concomitant aortic root abnormalities. If echocardiographic elevated B-type natriuretic peptide level, LV ejection fraction
data conflict with clinical data or echocardiographic images are below 50%, pulmonary hypertension, and moderate to severe
suboptimal, CMR imaging may be useful. It can assess leaflet valve calcification. Appropriate follow-up of asymptomatic
anatomy, measure gradients, and, in patients with a bicuspid patients is based on lesion severity (see Table 35).
aortic valve, assess aorta and aortic root anatomy. Cardiac CT Aortic valve replacement i s indicated for asymptomatic CJ
can be used to measure valve area when echocardiographic patients with severe aortic stenosis and LV systolic dysfunction
images are inadequate and to quantify calcification, a marker ( LV ejection fraction <50°1..) as well as for those pat i ents with
for risk of disease progression. severe aortic stenosis who are undergoing coro n a ry artery
Some patients with calcific aortic stenosis have severe bypass grafti ng or surgery on t he aorta or other heart valves.
aortic stenosis based on valve area but a gradient that is less Surgical aortic valve replacement is the t rea tment of
than 30 mm Hg. Whether symptoms in this "low-flow/low choice for most patients with symptomatic severe aortic steno
gradient aortic stenosis" are caused primarily by aortic valve sis. It is associated with low mortality for pat ients younger
disease with resultant LV dysfunction or the effective valve is t ha n 70 years ( I %-3%) a nd for selected patients who are older
reduced owing to poor leaflet excursion ("pseudosevere aortic than 80 years. Balloon aortic valvuloplasty ( BAV) has a limited
stenosis") can be best determined with dobutamine stress role in t he t rea t ment of adult ao rt i c stenosis. Whereas BAV
echocardiography. Key to this evaluation is the understanding does result in reduction in gradient and increase in valve area.
of contractile reserve. Contractile reserve is defined as an t hese results typically last only for a few months. Recen tly. BAV
i ncrease in transaortic stroke volume of greater than 20% with in the adult has been used successfully as a bridge to definitive
dobutamine infusion. In patients with severe aortic stenosis t reatment (surgical or transcatheter aortic valve replacement
and resultant LV dysfunction but with contractile reserve, the [TAVR]) or to di fferentiate symptoms in high- risk patients
mean gradient will increase with dobutamine stress and the with comorbid conditions such as COPD.
aortic valve area will remain below 1.0 cm2; these patients will Medical therapy has limited benefit in the treatment of
benefit from aortic valve replacement. Those with an increase aortic stenosis. Statins do not delay the progression of aort ic
in aortic valve area to greater than 1.0 cm2 or with a maximum stenosis bu t should be prescribed as indicated to patients with
78
Va lvu l a r Heart D isease
79
Va lvu l a r Heart D isease
KEY POI NTS degree and rate of aortic dilation and by family history. Annual
evaluation should occur if the aortic diameter is greater than
• Urgent o r emergent surgical valve replacement is indi
4.5 cm. Multidetector CT or CMR imaging is appropriate for
cated for patients with acute severe aortic regurgitation.
further assessment of aortic pathology when TIE or TEE is not
• Valve replacement is indicated for symptomatic patients conclusive and for long-term follow up of aortic dimensions
with chronic severe aortic regurgitation regardless of when TEE images are inadequate. A bicuspid aortic valve may
left ventricular systolic function. be present in patients with other thoracic aneurysm syn
dromes as well, such as Loeys-Dietz syndrome (hypertelorism,
split uvula or cleft palate, and aortic aneurysms) .
B icuspid Aortic Va lve Echocardiography of first-degree relatives of patients with a
Bicuspid aortic valve is the most common congenital heart bicuspid aortic valve is indicated to screen for bicuspid aortic
abnormality, affecting 0.5% to 2% of the total population. valve or aortic aneurysms.
Although a bicuspid aortic valve may be suspected in patients As with calcific aortic stenosis, medical therapies for
with classic auscultatory findings (systolic ejection click at the bicuspid aortic valve stenosis are limited. Aortic valve replace
left lower sternal border: murmur of aortic stenosis or aortic ment is the only therapeutic option for adult patients with a
regurgitation in a young patient) , it is most often diagnosed by stenotic bicuspid aortic valve, and recommendations regard
TIE performed for some other indication. A bicuspid aortic ing when to intervene are the same as for tricuspid aortic
valve may be diagnosed initially in a patient presenting with a valves (see Table 34) .
complication, such as bacterial endocarditis or aortic dissec For patients with a regurgitant bicuspid aortic valve, valve
tion. Features that may raise suspicion include eccentric aortic replacement is the treatment of choice when regurgitation is
regurgitation, enlarged sinuses ofValsalva, a dilated ascending clinically significant; however, repair may be performed in
aorta, or an elliptical valve opening. Calcification may obscure selected patients by experienced surgeons.
valve leaflets and make the diagnosis difficult. TEE may pro Surgery to repair the aortic root or replace t he ascending CJ
vide confirmatory information as to leaflet anatomy as well as aorta is i ndicated i n patients wi th a bi cu sp id aortic valve when
the presence of associated conditions, including aortic aneu the aort ic root diameter is greater than 5 .5 cm . Surgery is rea
rysms and aortic coarctation, and is appropriate when the sonable if' t he dia meter of the ascending aorta or aortic root is
diagnosis is uncertain. greater t ha n 5 . 0 cm and a r i sk factor for d issection is present
The clinical course of patients with a bicuspid aortic valve ( fa m i ly history of aortic d issection or if' the rate of i ncrease i n
varies widely. Although overall survival is similar to that of diameter i s 2:0.5 c m per ye a r) . Ascending aorta re pl ace m en t is
age-matched controls, approximately one in three patients reasonable in patients who are u nd ergo i ng aortic valve surgery
with a bicuspid aortic valve may eventually require valve sur because of severe aortic stenos is or aortic regurgitation if t he
gery for either stenosis or regurgitation. Stenosis proceeds at a diameter of the ascen d i ng aorta is greater than 4 . 5 cm. CJ
faster rate when the aortic valve is bicuspid, and valve replace
KEY P O I N TS
ment may be required in the fifth or sixth decade of life. Some
degree of regurgitation is common with a bicuspid aortic valve • I n general, older, asymptomatic patients with a bicuspid HVC
but is less likely than stenosis to lead to valve replacement. The aortic valve and severe aortic valve stenosis or regurgita-
presence of a bicuspid aortic valve carries an increased risk of tion require yearly echocardiography; those with mild
infective endocarditis, and good dental care is important in aortic stenosis or regurgitation require echocardiogra-
these patients. Current guidelines no longer recommend anti phy every 3 to 5 years.
biotic prophylaxis for this patient population (see Infective • Echocardiography of first-degree relatives of patients
Endocarditis, later) . The timing of follow-up TTE depends on with a bicuspid aortic valve is indicated to screen for
many factors, including patient age and severity of the valve bicuspid aortic valve or aortic aneurysms.
lesion. In general, older, asymptomatic patients with a bicus
• Patients with a bicuspid aortic valve are predisposed to
pid aortic valve and severe aortic valve stenosis or regurgitation
aortopathy, and aortic surgery is indicated when the
require yearly echocardiography; those with mild stenosis or
aortic root diameter is greater than 5 . 5 cm.
regurgitation should have echocardiography every 3 to 5 years.
A bicuspid aortic valve may occur with other cardiovas
cular and systemic abnormalities, such as aortic coarctation,
aneurysm of the sinuses of Valsalva. and patent ductus arterio M itra l Ste nosis
sus. Patients with a bicuspid aortic valve are predisposed to Clinical Presentation and Evaluation
aortic aneurysm and dissection owing to aortic connective The primary cause of mitral stenosis is rheumatic carditis.
tissue abnormalities. For this reason, the ascending aortic Improved hygiene and the routine use of antibiotics for group
diameter should be reassessed by echocardiography if the A streptococcal pharyngitis have greatly reduced the incidence
aortic root or ascending aorta dimension is greater than or of rheumatic heart disease in the United States and developed
equal to 4.0 cm, with the evaluation interval determined by countries. Rarely, functional mitral stenosis occurs as a result
80
Va lvular Heart D isease
of outflow obstruction from tumor (such as myxoma) or left valvotomy is unava i la b le , unsuccessfu l , or contra indicated or
atrial thrombus. Other causes of mitral stenosis include con when the valve morphology is un favorable.
genital disease (such as parachute mitral valve, wherein the Medical therapy for mitral stenos is co n si s t s of d iuretics or
mitral chordae insert into one instead of two papillary mus long-act i ng ni trates, which may help i mprove symptoms such
cles) and mitral annular calcification. Symptoms can be indo as dyspnea. I n add i t ion. �-blockers or n o ndi hyd ropyr id i ne
lent, with patients remaining asymptomatic for years and then calciu m channel blockers can lower heart rate and improve LV
presenting with a gradual decrease in activity. Other symp diastoli c filling t i me. c:J
toms include dyspnea, orthopnea, fatigue, and, less com
KEY POINT
monly, hemoptysis or systemic embolization. Symptoms typi
cally are not present until the mitral valve area is less than • Mitra! valvotomy or surgery is indicated for sympto
1 . 5 cm 2 , although tachycardia may precipitate symptoms at matic patients (New York Heart Association functional
larger valve areas. The decrease in mitral valve area results in a class II-IV) with severe mitral stenosis and favorable
diastolic pressure gradient between the left atrium and ventri valve morphology.
Cl
valve, with thickened, calcified subvalvular apparatus and Acute severe m i l ral regurgi t a t i o n is associated w i t h
marked left atrial enlargement. Gradients are heart-rate papi l la ry muscle ru p t ur e fo llowing acute myocard i a l
dependent and can vary greatly in patients who are tachy i n fa rc t i o n . fla i l m i t ral valve (d issoc i a t i o n o f t h e valve l e a f
cardic or have atrial fibrillation. Echocardiography is essential l e t fro m t h e c horclae) . a n d i n fe c t ive endocard i ti s w i t h
for assessment of valve morphology, including factors such as leaflet p e r fo ra t ion . The sudden l a rge vol u m e i n the l e ft
valve calcification and thickening, leaflet motion, and iJ l r i u m and ven t ricle results i n ra pid i n c reases i n LV e n d
subchordal thickening. These factors, taken together, help d i as t o l i c pressure a n d left a t rial pressure, which leads t o
predict the likelihood of successful percutaneous mitral bal e l evated pu l m o n a ry a rtery p ressure a n d p u l m o n a ry
loon valvuloplasty. TEE provides better visualization for edema. The d i m i n i s h ed LV s t ro ke vo l u m e leads t o hypo
assessment of mitral regurgitation severity as well as the pres tension and shock. c:J
ence of left atrial appendage thrombus. Multidetector CT may Patients with chronic mild to moderate mitral regurgita
provide additional information by valve planimetry as well as tion may remain asymptomatic for many years. Progression
visualization of the coronary anatomy. This may be helpful for is variable and caused by either progression of lesions or
patients whose valve is not well imaged on TIE and ar,e unable increasing mitral annulus size. The increase in volume and
to tolerate TEE. subsequent increase in left atrial pressure lead to compensa
tory dilation of the left atrium and LV. Left atrial dilation
Cl Management
Percutaneous m i t ral balloon valvotomy is indicated f"or symp
predisposes to atrial fibrillation. A chronic increase in
preload and resultant eccentric hypertrophy, i n the setting of
tomatic patients (New York Heart Association [NYJ -IA] func increased stroke volume (normal forward stroke volume and
tional class I I , I l l or I V) with severe mitral stenosis (see Table 34)
, regurgitant volume) lead to contractile dysfunction and
a nd favorable valve morphology. TEE plays an important role increased end-diastolic volume, which lead to pulmonary
in assessment of patients being cons id e red for percutaneous congestion and pulmonary hypertension. Appropriate fol
bal loon valvotomy to evaluate for potential contraindications. low-up of asymptomatic patients with mitral regurgitation is
i ncluding left atrial appendage clot or signi ficant (moderate to outlined in Table 35.
severe) m itral regurgitat ion . M i t ra l valve su rgery (repai r if pos TTE serves as the main imaging modality in the evalua
sible) is indica ted in patients with symptomatic (NYHA func tion and management of mitral regurgitation. TIE allows diag
tional class I l l-IV) severe m itral stenosis when balloon nosis of the mechanism of mitral regurgitation, qualitative and
81
Va lvular H e a rt Disease
TABLE 37. Empiric Therapy for Infective Endocarditis which changed significantly in recent years, may contradict
long-standing expectations of patients and practice patterns of
Condition Therapy
health care providers. However, infective endocarditis is more
Com m u n ity-acq u i red native Consider vancomycin + likely to result from frequent exposure to random bacteremia
valve I E genta m i ci n
associated with daily activities than from bacteremia caused
Nosocomia l-associated I E Consider vancomycin + by dental, gastrointestinal tract, or genitourinary procedures;
genta micin + rifampin or
vancomycin + gentamicin +
additionally, the risk of antibiotic-associated adverse
a carbapenem or cefepime effects may exceed the benefit (if any) from prophylactic
Prosthetic valve I E Consider vancomycin + antibiotic therapy.
gentamicin + rifampin Infective endocarditis prophylaxis is currently recom
I E = infective endocarditis.
mended for patients with cardiac conditions with the highest
risk for adverse outcomes from infective endocarditis (rather
than those with an increased lifetime risk for infective endo
Cl within 60 days of a hospi tal admission that vvas associated wi th carditis) . Prophylaxis is recommended for patients with (1) a
prosthetic cardiac valve; (2) a previous episode of infective
_ _
a risk for bacterernia or mfecllve _
endocard1t1s. For nosocorn1al
CONT. endocarditis; (3) congenital heart disease, including unre
infections, coverage for multidrug-resistant bacteria, particu paired cyanotic congenital heart disease, a completely repaired
larly coagulase-negative staphylococci, is recom mended. congenital heart defect with prosthetic material or device dur
Surgery for native valve endocarditis usually entails resec ing the first 6 months after the procedure, and repaired con
tion of the vegetation and valve repair or replacement if appro genital heart disease with residual defects; or (4) valvulopathy
priate. Early surgery (during i nitial hospitalization and before following cardiac transplantation.
completion of a ful l course of antibiotics) is indicated for Unless there is a history of infective endocarditis, prophy
patients with acute infective endocarditis presenting with laxis is not recommended for patients with native valve dis
valve stenosis or regurgitation resu lting i n heart fail ure: left ease, including rheumatic heart disease, mitral valve prolapse
sided infective endocarditis caused by Stap h y lococcus a u reus, with regurgitation, or a bicuspid aortic valve.
fungal, or other highly resistant organisms; infective endocar In patients who meet the criteria, prophylaxis should be
ditis complicated by heart block. annular or aortic abscess, or administered prior to dental procedures that involve manipu
destructive penetrating lesion; and infective endocarditis with lation of gingival tissue or the periapical region of the teeth or
persistent bacteremia or fever lasting longer t han 5 to 7 days perforation of the oral mucosa (Table 38 ) . Infective endocardi
after sta rting antibiotic therapy. Surgery is recommended for tis prophylaxis is not recommended prior to nondental
patients with relapsing prosthetic valve endocarditis. l n procedures, such as TEE, genitourinary procedures, or gastro
patients with infective endocarditis who have documented intestinal procedures. Antimicrobial prophylaxis is recom
i n fection of pacemaker or defibril lator systems. complete mended for procedures involving incision or biopsy of the
removal of these systems (all leads and generator) is indicated. respiratory tract mucosa, such as bronchial biopsy, tonsillec
Early surgery is reasonable in patients with infective endocar tomy, and adenoidectomy.
ditis who h ave recurrent emboli and persistent vegetations on
antibiotic therapy, and may be considered in patients with KEY P O I NTS
native valve endocarditis who have mobile vegetations greater • In patients with a cardiac murmur suggestive o f organic
than 10 mm in length . The Early Surgery versus Conventional valvular or congenital heart disease or patients with a
Treatment in infective Endocarditis (EASE) trial compared the prosthetic heart valve, infective endocarditis should be
clinical outcomes of early surgery with a conventional treat suspected in the presence of fever, anemia, hematuria,
ment strategy in patients with left-sided i n fective endocarditis and physical findings suggestive of embolization.
and a high risk of embolism . In this study. early surgery • Early surgery is indicated for patients with acute infec
(within 48 hours a fter diagnosis and random ization) in tive endocarditis presenting with valve stenosis or regur
patients with large vegetations (>10 mm) significantly reduced gitation resulting in heart failure; left-sided infective
embolic events with similar in-hospital and 6 -rnonth mortal endocarditis caused by Staphy lococcus aureus, fungal,
i ty rates compared with delayed surgery. CJ or other highly resistant organisms; infective endocardi
tis complicated by heart block, annular or aortic abscess,
Prophylaxis or destructive penetrating lesion; and infective endocar
The significant morbidity and mortality associated with infec ditis with persistent bacteremia or fever lasting longer
tive endocarditis underscore the importance of appropriate than 5 to 7 days after starting antibiotic therapy.
administration of antibiotics before procedures expected to
• Surgery is recommended for patients with prosthetic
produce bacteremia. The American College of Cardiology/
valve endocarditis and relapsing infection.
American Heart Association (ACC/AHA) recommendations and
the European Society of Cardiology (ESC) recommendations, (Continued)
84
Va lvu l a r Heart D isease
TABLE 38. Prophylactic Infective Endocarditis Regiinens for Adults Before a Dental Procedure
Situation Agent" Dosage
Oral Amoxici l l i n 2g
Una ble to take oral medication Ampici l l i n 2 g I M or IV
or
Cefazo l i n or ceftriaxone 1 g I M o r IV
Allerg i c to penicillin or a m picillin - oral Cephalexin 2g
or
C l i nd a mycin 600 mg
or
Clindamyci n 600 mg IM or IV
IM = intramuscular; I V = intravenous.
aRegimen consists of a single dose 30 to 60 minutes before the dental procedure, or, if inadvertently not administered, drug may be given up to 2 hours after the procedure.
Adapted from Wilson W, Taubert KA, Gewitz M, et al; American Heart Association. Prevention of infective endocarditis: guidelines from the American Heart Association: a guide
line from the American Heart Association Rheumatic Fever. Endocarditis and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on
Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. J Am Dent Assoc. 2008
Jan; 1 39 Suppl:3S-24S. Review. Erratum in: J Am Dent Assoc. 2008 Mar;1 39(3):253. IPMID: 1 8 1 673941. Copyright 2008 American Dental Association.
K E Y P 0 I NT S (continued) valve selection (Table 39) . In general, patient factors that favor a
mechanical prosthesis include younger age, an absence of con
HVC • Infective endocarditis prophylaxis should be limited to
t raindications to long-term anticoagulation. a risk of acceler
those with a prosthetic cardiac valve; a history of infec
ated bioprosthetic structural valve deterioration (for example,
tive endocarditis; unrepaired cyanotic congenital heart
with pregnancy or kidney failure) . and a reasonable l i fe expec
disease or repaired congenital heart defect with prosthe
tancy in patients for whom future redo valve surgery wou ld be
sis or shunt (::;6 months post-procedure) or residual
h igh risk. Conversely, factors that would favor a bioprosthelic
defect; or valvulopathy following cardiac transplantation.
valve i nclude older age; patients in whom adequate long-term
anticoagu lation is unlikely (for example, owing to adherence
problems or ant icoagu lation agents not being readily ava ilable)
Prosthetic Va lves or contraindicated because of' high bleeding risk (prior major
Bio p rosthesis Recommended: patients of any age for whom anticoag u lant therapy is contra i nd icated,
cannot be ma naged appro p riately, or is not desired
Mechanical prosthesis Reasonable: AVR or MVR i n patients <60 y of age who do not have a contra i n d ication to
anticoagulation
Either bio- or mechanical prosthesis Reasonable: patients between 60 y and 70 y of age
NOTE: The guideline recommends that the choice of valve intervention and prosthetic valve type should be a shared decision-making process.
Recommendations from Nishimura RA, Otto CM, Bonow RO, et al; American College of Cardiology/American Heart Association Task Force on Practice Guidelines. 201 4 AHA/ACC
guideline for the management of patients with valvular heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force
on Practice Guidelines. J Am Coll Cardiel. 201 4 Jun 1 0;63(22):2438-88. Erratum in: J Am Coll Cardiel. 201 4 Jun 1 0;63(22):2489. IPMID: 24603 1 921
85
Ad u lt Cong e n ita l H e a rt D isease
Ad u lt Congenita l
H ea rt Disease
I ntroduction
Advances i n caTe of patients with congenital heart disease over
the past six decades have resulted in more adults U1an children F I G U R E 2 7 . Patent fora men ovale. The arrows demonstrate the mecha nism of
rig ht-to-l eft s h u nt through the patent foramen ova le. LA = left atri um; LV = left ven·
living in North America with these conditions. CardiovasculaT
tricle; RA = right atri u m ; RV = right ventricle.
residua are common in patients wiili previous intervention for
Redrawn from original supplied courtesy of Dr. William 0. Edwards, Department of Laboratory Medicine and
congenital cardiac lesions, underscoring ilie importance of peri- Pathology, Mayo Clinic, Rochester, MN.
86
A d u lt Congen ita l H e a rt D i sease
KEY P O I NTS
• Antiplatelet therapy i s recommended a s first-line ther abnormalities and congenital heart defects, most commonly
apy for patients with patent foramen ovale and crypto an ASD. Familial ostium secundum ASDs have also been
genic stroke. described and may be autosomal dominant or are linked to
HVC • There is no indication for patent foramen ovale closure chromosome 5. Down syndrome is commonly associated with
or for antiplatelet therapy in asymptomatic patients. congenital heart disease, most commonly a form of atrioven
tricular septa! defect, including ostium primum ASD.
HVC • Randomized trials do not support patent foramen ovale
closure to reduce risk of recurrent stroke or migraine.
Clinical Presentation
ASDs may initially be identified in adulthood; the age of presen
tation depends on the shunt size and associated defects.
Atrial Septa l Defect Common presenting teatures include fatigue; exertional dysp
Pathophysiology and Genetics nea; atrial fibrillation; paradoxical embolism; and abnormalities
An atrial septal defect (ASD) is a communication between the on the physical examination, including a pulmonary outflow
atria. Left-to-right shunt occurs and, over time, causes right murmur or fixed splitting of S 2 or, less commonly, findings that
sided cardiac chamber dilatation. Defects are classified accord are consistent with a genetic syndrome involving an ASD.
ing to location (Figure 28) . Ostium secundum ASDs are the most Occasionally, an ASD is identified as the cause for right heart
common type (75% of cases) . These are located in the middle enlargement found incidentally on an echocardiogram. Rarely,
portion of the atrial septum and are usually isolated anomalies. patients with isolated ASDs present with pulmonary arterial
Ostiurn primum defects (15%-20% of cases) are located in the hypertension (PAH); this usually occurs in young women, sug
lowest portion of the atrial septum; they are commonly associ gesting the coexistence of idiopathic PAH .
ated with a cleft mitral valve, ventricular septal defect, and sub Clinical findings in ASD include jugular venous disten
aortic stenosis, a collection of abnormalities tenned endocardial tion, a parasternal impulse, and a systolic flow murmur at the
cushion detect. Sinus venosus defects (5%-10% of cases) are second left intercostal space. Large shunts may cause a dias
usually located near the superior vena cava or, rarely, near the tolic flow rumble across the tricuspid valve owing to a large
inferior vena cava. More than 90% of patients with sinus venosus left-to-right shunt. Fixed splitting of the S 2 is the characteristic
ASD have associated anomalies of pulmonary venous connec auscultatory finding in patients with an ASD.
tion. A coronary sinus ASD (<l % of cases) is a communication
between the coronary sinus and the left atrium. These detects Diagnostic Eval uation
are commonly associated with complex congenital heart lesions The electrocardiogram (ECG) and chest radiograph findings in
or a persistent left-sided superior vena cava. patients with ASDs are outlined in Table 40. Complete heart
Most ASDs occur sporadically; however, several genetic block may occur in familial ASD.
syndromes associated with ASDs are recognized. The Holt TI'E is the diagnostic imaging modality of choice for iden
Oram syndrome involves bilateral upper extremity bone tification of ostium primum and secundum ASDs. Additional
87
Adult Con g e n ital Heart D i sease
TABLE 40. Imaging Findings and Late Complications in Adult Congenital Heart Disease
Lesion ECG and CXR Findings Late Complications
Ostium secu ndum ASD ECG : I n complete RBBB, RA enl argement, right Right heart enlargement, atrial fibril lation, PAH
axis deviation (rare)
Ostium primum ASD ECG : Left axis deviation, 1 st-deg ree Right heart enlargement, atrial fibrillation, m itral
atrioventricular block regurgitation (from mitral valve cleft), PAH (rare)
CXR: Right heart enlargement, prominent Post repair: residual s h u nt (rare), m itral
p u l monary artery, i ncreased pulmonary regurgitation (from mitral valve c left), left
vascularity ventricular outflow tract obstruction
Sinus venosus ASD ECG : Abnormal P axis Right heart enlargement, atria l fi bri l l ation, PAH
(rare)
CXR: Right heart enlargement, prominent
pulmonary a rtery, i ncreased p u l monary Post repair: residual s h u nt (rare), residual
vascularity anomalous pul monary venous co n nection
Large VSD ECG : RV or RV/LV hypertrophy Left heart enlargem ent, PAH , Eisenmenger
syndrome
CXR: LA a n d LV enlargement, increased
pulmonary vascu lar markings; with PAH : Post repair: residual VSD, residual s h u nt (rare)
prominent central pul monary a rteries, reduced
peripheral p u l monary vascular markings
Large PDA ECG: LA enl argement, LV hypertrophy; with PAH : Endocarditis, heart fa i l u re, PAH, Eisenmenger
RV hypertrophy syndrome
CXR: Cardiomega ly, i ncreased pulmonary Post repair: residual shunt (rare)
vascular markings; calcification of PDA
(occasional); with PAH : prominent central
pulmonary arteries, reduced peripheral
pulmonary vascu l a r markings
Pulmonary valve stenosis ECG : Normal when RV systolic pressure Post repair: Severe p u l monary valve regurgitation
<60 mm Hg; if RV systol i c pressure >60 m m Hg: after pul monary valvotomy or valvuloplasty
RA enlargement, right axis deviation, RV
hypertrophy
Aortic coa rctation ECG: LV hypertrophy and ST-T wave Hypertension ( 7 5% of cases), bicuspid aortic
a b normal ities valve (> 50% of cases), increased risk of aortic
aneurysm and intracra n i a l aneurysm
CXR: Dilated ascending aorta, "figure 3 sign"
beneath aortic arch, rib notching from col lateral Post repair: Recoarctation, hypertension, aortic
vessels aneurysm
Repaired tetra logy of Fa I lot ECG: RBBB, i ncreased ORS d u ration (ORS Post repair: Increased atrial and ventricular
d u ration reflects degree of RV d i l atation) arrhyth mia risk, pulmonary valve reg u rgitation or
stenosis; tricuspid regurgitation
CXR: Ca rd iomegaly with pul monary or tricuspid
valve regurgitation; right aortic arch i n 25% of ORS > 1 80 msec i ncreases risk of ventricular
cases tachycardia a n d sudden death
Eisenmenger syndrome ECG : Right axis deviation, RA enl argement, RV Right heart fa i l u re, hemoptysis, stroke
hypertrophy
ASD = atrial septal defect; CXR = chest radiograph; ECG = electrocardiogram; LA = left atrium; LV = left ventricle; PAH = pulmonary arterial hypertension; PDA = patent ductus arte
riosus; RA = right atrium; RBBB = right bundle branch block; RV = right ventricle; VSD = ventricular septa I defect.
88
Ad ult Con g e n ita l H e a rt D i s ease
findings on TIE include right-sided cardiac chamber enlarge Patients with small ASDs do not need a ny limitation
ment, tricuspid regurgitation related to annular dilatation, and of physical activity. I n patients with large left-to-right
increased right ventricular systolic pressure. Agitated saline shunts, exercise is often self-limited owing to decreased
contrast injection may help identify a right-to-left atrial shunt cardiopulmonary function. If pulmonary vascular disease
if Eisenmenger syndrome is suspected (see Adults with is present, patients should b e advised against isometric or
Cyanotic Congenital Heart Disease, later) . Sinus venosus and competitive exercise.
coronary sinus ASDs are less readily diagnosed by TIE in Pregnancy in patients with ASD is generally well tolerated
adults and often require TEE, MRI, or CT imaging. in the absence of PAH. The risk of congenital heart disease
MRI and CT can be used to quantify right ventricular transmission in patients with sporadic ASD is estimated to be
volumes and ejection fraction. These studies are rarely used as less than 10%. Genetic syndromes have variable inheritance; a
the primary imaging modality when ASD is suspected but may family history should be taken. Holt-Oram syndrome is inher
help quantify right heart enlargement in a patient with ASD. ited in an autosomal dominant fashion.
In addition, a CT or MRI performed for another reason may be
the first imaging study to demonstrate the ASD. MRI, CT, and Follow-up After Atrial Septal Defect Closure
TEE are useful for identifying anomalous pulmonary veins. Clinical follow-up is recommended for all adult patients after
Cardiac catheterization is the only reliable method to surgical or device ASD closure; the frequency of follow-up
calculate the pulmonary-to-systemic blood flow ratio should be individualized. TIE imaging is generally recom
(Qp:Qs) but is rarely required for uncomplicated ASDs. mended within the first year after closure and then periodi
Cardiac catheterization may be recommended in the patient cally after that. Pre- and post-closure atrial fibrillation occurs
with an ASD and PAH to aid in determining whether ASD more frequently the older the patient is at the time of ASD
closure is indicated. closure. Rare complications after device closure include device
migration, erosion into the aorta or pericardium, and sudden
Treatment
death. Chest pain or syncope after device closure warrants
89
A d u lt Congenital Heart D isease
Treatment
Although percutaneous device closure is possible for select c::J
VSDs, most patients a re treated surgically. Closure of' a VSD is
generally indicated when there is a significant shunt (Qp:Qs
ratio is 2 . 0 or greater) , and there is evidence of left ventricular
volume overload: an aclclitional ind ication for closure is a his-
tory of enclocarclitis. CJ
For small VSDs with a small left-to-right shunt and no
F I G U R E 2 9 . Positions of various ventricular septal defects viewed from the left left heart enlargement or valve disease, observation is
side of the heart. ( 1 ) perimembranous; ( 2 ) subpulmonary; (3) muscular; (4) in let. appropriate with periodic clinical evaluation and imaging.
Ao = aorta; LA= left atri u m . Large VSDs with right-to-left shunt reversal and PAH
Redrawn from original supplied courtesy o f Dr. William D. Edwards, Department o f laboratory Medicine and (Eisenmenger syndrome) should not be closed ; closure
Pathology. Mayo Clinic. Rochester, MN.
will result in clinical deterioration clue to reduction in
cardiac output.
Patients with a small VSD require no activity restrictions.
part of the atrioventricular septal defect complex and are
If pulmonary vascular disease is present (pulmonary artery
characteristically seen in patients with Down syndrome.
systolic pressure >50 mm Hg) , patients should be advised
against isometric or competitive exercise.
Clinical Presentation
In the absence of PAH , pregnancy in women with VSDs is
Clinical presentation of an isolated VSD depends on the defect
generally well tolerated. Women with VSDs and associated
size and pulmonary vascular resistance. Patients with a small
PAH should be counseled against pregnancy.
VSD and no PAH present with a loud holosystolic murmur
located at the left sternal border that often obliterates the S 1
Follow-up After Ventricular
and may be palpable. Small VSDs do not cause left heart
Septal Defect Closure
enlargement or PAH.
Complications following VSD repair include residual or recur
A moderate-sized VSD with a moderate left-to-right
rent VSD, arrhythmias, PAH , enclocarclitis, and aortic or tri
shunt may cause left ventricular volume overload and PAH.
cuspicl valve regurgitation. Cardiovascular evaluation with
Patients remain asymptomatic for many years but eventually
TIE imaging is recommended within 1 year of VSD closure.
present with symptoms of heart failure. The left ventricular
Subsequent clinical and TIE follow-up frequency depends on
impulse may be displaced, suggesting volume overload. A
clinical and cardiac status. Anticoagulation is not routinely
holosystolic murmur is noted at the left sternal border; the
recommended following VSD closure.
duration and quality depend on the pressure gradient between
the left and right ventricles. Progressive PAH results in short KEY POI NTS
ening of the murmur. • For small ventricular septal defects with a small left-to- HVC
Large VSDs associated with moderate or large left-to right shunt and no left heart enlargement or valve dis-
right shunts are usually detected in chilclhoocl by the presence ease, observation is appropriate with periodic clinical
ofa murmur, heart failure, and failure to thrive. Unless closure evaluation and imaging.
is performed early in life, fixed PAH will ensue within several
• Closure of a ventricular septal defect is indicated when
years, resulting in Eisenmenger syndrome and right-to-left
the pulmonary-to-systemic blood flow ratio (Qp:Qs) is
shunt reversal.
2.0 or greater, and there is evidence of left ventricular
90
Adult Congenital Heart D isease
Clinical Presentation
A PDA produces an arteriovenous fistula, usually resulting in a
Pu l monary Va lve Stenosis
continuous murmur that envelops the S2• A tiny PDA is gener Pathophysiology
ally asymptomatic and inaudible. Patients with a moderate Pulmonary valve stenosis is usually an isolated congenital
sized PDA may present with symptoms of dyspnea and heart cardiac lesion, causing obstruction to right ventricular out
failure. A continuous "machinery" murmur is heard beneath flow. Noonan syndrome, an autosomal dominant disorder, is
the left clavicle. Bounding pulses and a wide pulse pressure often associated with isolated pulmonary valve stenosis or
may also be noted. other congenital cardiac defects. Additional features of
A large PDA causes a large left-to-right shunt and, if unre Noonan syndrome include short stature, variable intellectual
paired, may cause PAH with eventual shunt reversal from impairment, unique facial features, neck webbing, and
right-to-left (Eisenmenger syndrome) . A characteristic feature hypertelorism.
of an Eisenmenger PDA is clubbing and oxygen desaturation
that affects the feet but not the hands owing to desaturated Clinical Presentation
blood reaching the lower part of the body preferentially Patients with mild or moderate pulmonary valve stenosis are
(differential cyanosis) . generally asymptomatic, whereas those with severe stenosis
may have exertional dyspnea. On physical examination, mild
CJ Diagnostic Eval uation pulmonary valve stenosis is characterized by a normal jugular
The ECG and c hest ra d iogra p h f i n d i ngs i n patients wi t h PDJ\ venous waveform and precordial impulse. A pulmonary ejec
a re o u t l i ned in Tab l e 4 0. TTE w i t h color llow Doppler i m a g i ng tion click decreases with inspiration. Moderate or severe pul
u sually confirms the presence o f a PDA. I n 1xl l i e n t s w i t h severe monary valve stenosis results in right ventricular hypertrophy
PAH , the PDA m ay be d i fficu l t to visua l ize ow i ng to e q u a l iza with a resultant prominent a wave on the jugular venous pres
lion of pressures i n t he aorta a n d p u l m ona ry artery. I n pa t i e n t s sure waveform and a right ventricular lift. An ejection click
w i t h a PDA a n d e l eva t ed p u lm on a ry a rt e ry pressures . cardiac may be audible, but as the severity of pulmonary valve stenosis
cat heteriza t ion is used to determ ine reversi b i l i ty and shu n t progresses, the click disappears owing to loss of valve pliabil
size. Angiography confi rms t h e size a n d s h a pe o r t he P DA and ity. An ejection systolic murmur, heard at the left sternal bor
helps to determ ine whether pe rc u t a n eou s closure i s fe a s i bl e . der, increases in intensity and duration as the severity of pul
Cardiac CT and MRI may i d e n t i fy a P DA b u t are not used as monary valve stenosis worsens. The pulmonary valve
p r i m a ry diagnostic tec h n iques. Cl component of S2 is delayed and eventually disappears with
increasing severity. A right ventricular S,1 is heard in severe
Treatment pulmonary valve stenosis.
C lo s u re of a PDA is i n d ica te d for lert-sided c ard i a c chamber
Cl e n l a rge me n t i n t he absence or severe PA H . C l osure may be Diagnostic Evaluation
Cl
performed su rg i ca l ly or percutaneously : h owever. su rgical The l·:CG and chest rndiogra p h fi n d i ngs i n patients wi t h p u l
closure of a calci fied PDA may be chal le ngi ng. Referral to a monary \·al\ c st enosi s :1rc o u t l i ned in Ta ble "1 0 . TTE w i t h
co n ge n i t a l cardiac center for considera t ion or c los u re options l )o ppl er co n f"irms t h e presence o r pulmonary valve ste n o s i s
is appropriate for t h ese p<1 t ients. Cl and a l lows assessment o f ' i ts se ver i t y. P u l monary valve stenos is
A tiny PDA requires no intervention. In patients with a i s considered severe if' t h e peak gra d i e n t is 60 mm Hg or
small PDA and prior endocarditis, closure is suggested. A gre<ller. Pulmonary cusp mobi l i ty. calcifka t i o n . and t h e effects
moderate-sized PDA is generally closed percutaneously. A or obs t ru c t i o n on t he rig h t ven t ricle may a f 'IC.'ct t rea t me n t
large PDA with severe PAH and shunt reversal should be o p t i on s. Right ,·c n t ricu la r hy pe rt ro phy is expected in pa t i e n t s
observed, as closure may be detrimental. In these patients, wi t h p u l m o n a ry \"<live ste nos is. b u t when right heart e n l a rge
medical therapy for PAH should be considered. m e n t occ ur s . <1 11 ;1ssociated lesi o n . suc h as pul monary regurgi
Patients with a small PDA without PAH do not need any t a t i o n or an ASD. should be su spected . Cardiac C<ll he teriza t i on
limitation of physical activity. Anticoagulation is not rou is pri1mir i ly used when percut<rneous i n t erve n t ion is co nsid
tinely recommended for patients with PDA or following ered . M R I ;1 11d CT <i re n o t rou t i nely used i n patients with pul
PDA closure. mon a ry val\·e stcnosis. Cl
91
Ad u lt C o n g e nital H ea rt Disease
Clinical Presentation
Patients with aortic coarctation may be asymptomatic or pre
sent with hypertension, symptoms of exertional leg fatigue, or
headaches. Upper extremity hypertension and reduced blood
pressure and pulse amplitude in the lower extremities are
characteristic findings and cause a radial artery-to-femoral
artery pulse delay. More than SO% of patients with aortic
coarctation have a bicuspid aortic valve.
Turner syndrome is a chromosomal abnormality (4S,X)
characterized by a female with short stature, a broad chest
with widely spaced nipples, webbed neck, and aortic coarcta
tion. Aortic coarctation is also associated with bicuspid aortic
F I G U R E 3 0 . Chest radiograph of a patient with aortic coarctation exh ibiting
valve, aortic valve and subaortic stenosis, parachute mitral the "fi g u re 3 sig n," caused by d i latation of the aorta above and below the area of
valve, VSD, and cerebral artery aneurysms. coarctation (arrow).
92
Ad ult Co n g e n ital Heart D isease
Cl
I ntervention klr coarctalion is recom mended when the
coarctat1on systolic peak (peak-to-peak) pressure gradient is
20 mm Hg or h igher (measured by TTE Doppler or cardiac
catheterization) or if" there is radiologic evidence or severe
coarctat ion with collateral flow. Surgical and percu taneous
in tervent ion options are ava i lable. and selection depends on
the length, location. and severi ty of the coarctalion as well as
t he presence or associated lesions. c:J
Patients with severe residual or unrepaired coarctation,
aortic stenosis, or a dilated aorta should be counseled to avoid
contact sports and isometric exercise.
Women with repaired aortic coarctation and no significant
residua generally tolerate pregnancy well. A comprehensive
prepregnancy evaluation is warranted to evaluate for residua.
Women with severe unoperated coarctation should avoid preg
nancy prior to intervention. Patients with mild or moderate
residual or unoperated coarctation will generally tolerate preg
F I G U R E 31 Tetra logy of Fa I lot. A subarterial ventricular septa I defect (asterisk)
.
nancy well but should undergo blood pressure monitoring and pulmonary stenosis (arrow) are associated with secondary aortic override and
during pregnancy and receive cardiovascular follow-up. right ventricular hypertrophy. Ao = aorta; lA = left atrium; LV = l eft ventricle;
RA = right atrium; RV = right ventricle.
Follow-up After Aortic Coarctation Repair Redrawn from original supplied courtesy of Dr. William 0. Edwards, Department of Laboratory Medicine and
Pathology, Mayo Clinic, Rochester, MN.
Hypertension occurs in up to 75% of patients following coarc
tation repair. Blood pressure control is recommended to
reduce hypertension-related morbidity. I ntervention is often Approximately 15% of patients with tetralogy of Fallot
required for bicuspid aortic valve, aortic aneurysm, aortic dis have the 22qll.2 chromosome microdeletion. This increases
section, recoarctation, coronary artery disease, systolic or the chance of congenital heart disease inheritance to approxi
diastolic heart failure, and intracranial aneurysm. Age at the mately 50% compared with 5% in patients without the micro
time of repair is the most important predictor of long-term deletion. Genetic testing is recommended for all patients with
survival. Regular follow-up should include evaluation with a tetralogy of Fallot who are planning reproduction. Tetralogy of
congenital cardiologist as well as TIE and aortic imaging. Fallot is also common in persons with Down syndrome.
Surgical repair of tetralogy of Fallot involves patch closure
KEY POI NTS
of the VSD and relief of right ventricular outflow tract obstruc
• Upper extremity hypertension and reduced blood pres tion by patch enlargement. The transannular patch procedure
sure and pulse amplitude in the lower extremities are invariably disrupts the integrity of the pulmonary valve, caus
characteristic findings of aortic coarctation and cause a ing severe pulmonary valve regurgitation, the most common
radial artery-to-femoral artery pulse delay. reason for reoperation in patients after repair of tetralogy of
• Intervention for aortic coarctation is recommended Fallot. Over many years, pulmonary regurgitation causes pro
when the coarctation systolic peak (peak-to-peak) pres gressive right heart enlargement, tricuspid regurgitation, exer
sure gradient is 20 mm Hg or higher or if there is radio cise limitation, and increased risk for arrhythmias. Annual
logic evidence of severe coarctation with collateral flow. follow-up by a congenital cardiologist is recommended to
monitor these sequelae and determine optimal timing for
• Patients with severe residual or unrepaired aortic coarc
intervention. Current surgical techniques include attempted
tation, aortic stenosis, or a dilated aorta should be
relief of pulmonary valve stenosis with preservation of native
counseled to avoid pregnancy, contact sports, and iso
pulmonary valve function.
metric exercise.
93
Adult Cong e n ital H e a rt Disease
CJ sustained ven tricular tachyca rdia a re risk factors for left intracardiac shunt, filters on intravenous lines should be
sudden cardiac death. used to preven t paradoxical air embolism. Early ambulation,
CONT.
TI'E can confirm t he presence of pulmonary or t ricuspid pneumat ic compression devices, or anticoagu lation is also
valve regurgitation. right ventricular outflow tract obstruction. recommended for prevention of' venous stasis and potential
residual VSD. aortic regurgitation. and aortic dilatation. M R I is venous thrombosis and paradoxical embolism. Because perio
used to assess right ventricular size and function . which helps perative cardiac complications a re common in t hese patients,
determine appropriate t iming for pulmonary valve replace elective operations should be performed at centers that care
ment . Diagnostic catheterizal ion may be requi red to assess for such patients with a coordinated multidisciplinary team
hemodynamics and residual shunts and deli neate coronary approach. Consu ltat ion with a congenital cardiac special ist is
artery and pulmonary artery ;rnatomy. Cl recommended when patients are hospitalized. Cl
Most patients with cyanosis have compensated erythrocy
Treatment of Tetralogy of Fallot Residua tosis and stable hemoglobin levels. Occasionally, hyperviscos
Pulmonary valve replacement is recommended in patients ity symptoms occur, including headaches and reduced
with repaired tetralogy of Fallot who have severe pulmonary concentration. Phlebotomy is recommended for a hemoglobin
valve regurgitation with symptoms, decreased exercise tol level greater than 20 g/dL (200 g/L) and a hematocrit level
erance, more than moderate right heart enlargement or greater than 65% associated with hyperviscosity symptoms in
dysfunction, or arrhythmias. Long-standing pulmonary the absence of dehydration. Phlebotomy should be performed
valve regurgitation may result in tricuspid regurgitation, no more than two to three times each year. Dehydration should
and repair of t he tricuspid valve may also be needed. be excluded before considering the procedure, and it should
Percutaneous pulmonary valve replacement is now availa be followed by intravenous fluid administration. Repeated
ble for select patients with previous operative intervention phlebotomies deplete iron stores and may result in the
for tetralogy of Fallot. production of iron-deficient erythrocytes or microcytosis,
Patients with repaired tetralogy of Fallot and residual increasing the risk of stroke. Iron deficiency in a patient with
sequelae should be cautioned regarding participation in con destabilized erythropoiesis is treated with oral iron therapy for
tact sports and heavy isometric exercise. a short time. Iron therapy is discontinued when serum ferritin
and transferrin saturation values are within the normal range.
KEY POINT
Maternal cyanosis impairs normal fetal growth and devel
• Pulmonary valve replacement is recommended in
opment and increases the risk of intrauterine growth retarda
patients with repaired tetralogy of Fallot who have
tion and miscarriage. Maternal and fetal morbidity and
severe pulmonary valve regurgitation with symptoms,
mortality are increased, related to the degree of cyanosis,
decreased exercise tolerance, more than moderate right
ventricular function, and pulmonary pressures. If pregnancy
heart enlargement or dysfunction, or arrhythmias.
occurs in a patient with cyanotic heart disease, physical activ
ity should be curtailed and supplemental oxygen is recom
Adu lts with Cya n otic Congenita l mended. Preventive measures to decrease the risk of venous
thrombosis and paradoxical embolism also are recommended.
Heart Disease
General Management Eisenmenger Syndrome
Right-to-left cardiac shunts, such as unrepaired or palliated Eisenmenger syndrome is severe PAH and reversal of a con
tetralogy of Fallot, truncus arteriosus, tricuspid atresia, and genital cardiac shunt caused by VSD, PDA, or, less commonly,
Eisenmenger syndrome, result in hypoxemia, erythrocytosis, ASD. Eisenmenger syndrome has become increasingly rare
and cyanosis. Erythrocyte mass is i ncreased in patients with owing to medical screening, including TIE and appropriate
cyanosis as a compensatory response to improve oxygen intervention.
transport. Conservative medical measures are recommended in the
Physical features include central cyanosis and digital management of Eisenmenger syndrome. Persons with
clubbing. Patients with cyanotic congenital heart disease are Eisenmenger syndrome should be cautioned regarding iron
predisposed to scoliosis. arthropathy, gallstones, pulmonary deficiency, dehydration, acute exposure to excess heat, and
hemorrhage or thrombus, paradoxical cerebral emboli or cer moderate or severe strenuous or isometric exercise. Routine
ebral abscess, kidney dysfunction, and hemostatic problems. phlebotomy based on hemoglobin or hematocrit level is not
Because of these problems, a congenital cardiac specialist recommended because iron deficiency and microcytosis may
should evaluate patients with cyanotic congenital heart dis lead to increased symptoms; rather, phlebotomy should be
ease at least annually. performed only when symptoms of hyperviscosity occur and
Cl
Patients w i t h cyanotic or complex congenital heart dis with saline volume replacement. In addition, chronic high
ease are at increased risk for endocard i t is: therefore, antim i altitude exposure should be avoided, as it further reduces oxy
crobia l prophylaxis is recommended prior to certain nonsterile gen saturation. Women should be cautioned to avoid preg
procedures. In patients who are hospitalized with a right-to- nancy because of the high risk for maternal mortality.
94
D iseases of the Aorta
KEY P O I N T
• Jn patients with Eisenmenger syndrome, meticulous
care of intravenous lines with filters to avoid paradoxi
cal air embolism is imperative.
95
D iseases of the Aorta
Tra nsthoracic Good visual ization of aortic root/proximal Limited visualization of the d i stal ascend i n g aorta
echocardiography (TIE) ascending aorta and aortic arch with branches of the great vessels
A l l ows definition of valvular pathol ogy, myocardial A negative TIE does not rule out aortic dissection,
function, pericardia! disease and other imaging techniques m ust be considered.
Bedside d ia g nosis
Tra nsesophageal TEE has su perior imaging q uality versus TIE Req u i res experienced operator
echocardiography (TEE)
Exce l l ent visual ization of the aorta from its root to I nvasive proced u re
the descending aorta
Bedside d iagnosis
CT Visual ization of entire aorta and side branches Exposes patient to radiation, iodi nated contrast
dye
Rapid imaging
M u ltiplanar reconstruction
MRI Vi sual ization of entire aorta and s i d e branches For acute disease, prolonged i mage acquisition
away from acute care area
No exposure to radiation or iodi nated contrast dye
Contra indicated i n patients with implanted
pacemaker or defi brillator
Gadolinium contrast dye contraindicated in
patients with kid ney d i sease
96
D iseases of the Aorta
Loeys-Dietz syndrome
Other genetic a n d/or congenital conditions Fa m i l i a l thoracic aortic aneurysms and aortic dissections (TAAD) syndrome
Bicuspid aortic valve
Tu rner syndrome
Vascu l itis Ta kayasu a rteritis
Syp h i l i s
Aortic injury Prior acute aortic syndrome
Chest trauma
symptoms. Annual echocardiography should be performed to transthoracic ultrasound is the preferred imaging modality in
c::J
monitor aortic growth. Earlier re-evaluation is indicated for these patients.
changes in symptoms or physical examination findings and for The repa i r ol' a t horacic aortic aneurysm is often recom-
hemodynamic assessment related to pregnancy. When the mended prophylacl ical ly lo preve n t lhe morbid i ty and mortal-
aortic dimension nears the threshold for intervention, patients i ty associa ted w i l h a neurysm rupture. In asymptoma t i c
should be referred to an appropriate specialist for evaluation. patients. elective t ho racic a o r l i c repa i r is recom m ended i f t h e
Because patients with a bicuspid aortic valve have an increased a o r t i c root or ascendi ng aorta is greater t ha n 5 .5 cm (5 .5 -
risk of aortic aneurysm and dissection, these patients should 6 . 0 cm for t he descendi ng aorta) or has rapid growth (>0. 5 cm /
undergo echocardiography of the aorta annually if the aortic year) . For genet ical ly media led d isorders (such as Marian syn
root or ascending aorta dimension is greater than 4.5 cm. In drome) . a lower t h reshold or 5 . 0 cm (4 .0 -5 .0 c m in certain
those with an aortic diameter between 4.0 and 4.5 cm, the pat ients) may be used for repa i r. For pat ients w i t h a bicuspid
examination interval depends on the rate of progression of aort ic valve. repa i r is indica ted if t he aortic d i a meter is greater
dilation and the family history. t ha n 5 . 5 cm <1 11CI is reasonable i f" t he diameter is grea ter than
Familial thoracic aortic aneurysms and aortic dissections 5 . 0 c m and Lhe patient has an increased risk of d issection
(TAAD) is an inherited autosomal dominant condition. ( fam i ly h istory or d i ssection or rapid growth) .
Screening is recommended for first-degree relatives of persons Repai r or ascend i ng aortic and aortic a rch aneurysms
with TAAD once a year or at least every few years. If the muta requ i res surgery and may include aort ic valve replacement or
tion is known, genetic testing can identify those relatives who repair in pa t ients w i t h sign i ficant a n n u l a r d i l a tation or associ
should be screened with aortic imaging. ated aort ic valve pathology. /I. conservative procedure whereby
In patients with Marfan syndrome, follow-up imaging is t h e a neurysm is replaced w i t h a Dacron graft and t h e aortic
recommended 6 months after diagnosis with annual surveil valve is preserved has ga i ned widespread use. I f t h e aortic
lance thereafter if the aortic root is less than 4.5 cm in diam valve n e e d s rep l acement and the pa t i e n t has a d i la ted
eter and otherwise stable. If the aortic diameter is 4.5 cm or aortic roo t . a composite aortic va lve and aortic root and
greater or shows significant growth over time, then more fre ascending aorta gran replacemen t ( Bental l operat ion) may be
quent surveillance is suggested (for example, twice yearly) . performed. The Ben tall operation i ncludes re-implan tation or
Most patients with Marfan syndrome present with enlarge t he coronary arteries into t h e ascend i ng aortic graft. Thoracic
ment of the ascending aorta; therefore, serial examination is endovascu l.:ir aort ic repai r (TEVAR) w i l h stent grafting has
focused mainly on assessing this portion of aorta, and emerged as a promising a l terna t ive lo open repa i r for
97
D iseases of the Aorta
------------�--
Cl an e u rysm or the descending t h oracic aorta . TCVAR has been Acute aortic d issection
associated \\'i t h shorter h ospi t <1 I stays and lower hosp i t a l mor
CONT.
b id i ry a n d has t he potcn t i<l l <ld\'<l n tages o r <1\'0 id i ng t h o rncot
omy. aortic cross c l <l m p i ng. and e x t rncorporea l su p port .
Adverse c\'e n t s fo l \ o\\·i ng TE\'A R i n c l u d e s t ro ke . s p i na l
isch e m i a . access com pl i ca t i o ns . and cnd o l ea ks. Cl
K EY P O I N T S
• Patients with a thoracic aortic aneurysm should
undergo annual echocardiography to monitor aortic
aneurysm growth.
• Patients with a bicuspid aortic valve should undergo Acute i ntra m u ra l hematoma
annual echocardiography of the aorta if the aortic root
or ascending aorta dimension is greater than 4.5 cm.
• In asymptomatic patients, elective thoracic aortic repair
is recommended if the aortic root or ascending aorta is
greater than 5 . 5 cm (5.5-6.0 cm for the descending
aorta) or has rapid growth (>0. 5 cm/year) ; for patients
with genetically mediated disorders, the threshold for
repair is lower.
Pathophysiology
In aortic dissection, blood passes through a tear in the aortic
intima, creating a false lumen that separates layers of the aorta. F I G U R E 3 3. Cross·sectional representation of acute aortic syndromes. Acute
aortic dissection: interruption of intima (blue) with creation of an intimal flap and
Propagation of the dissection can proceed in an anterograde or
false lumen formation with i n the media (red). Color flow by Doppler echocardiog
retrograde fashion from the initial tear, involving side branches raphy or intravenous (IV) contrast by CT is present with i n the false lumen i n the
and causing complications such as tamponade, aortic valve acute phase. Acute intramural hematoma: crescent-shaped hematoma contained
insufficiency, or malperfusion syndromes. An intramural with i n the media without interruption of the intima (blue). No color flow by
hematoma may result from rupture of the vasa vasorum or Doppler echocardiography or IV contrast by CT within crescent. Penetrating athero·
sclerotic ulcer: atheroma (yellow) with plaque rupture disrupting intimal integ rity;
"microtears" in the intima, resulting in a crescent of hema
blood pool contained with i n inti ma-medial layer (pseudoaneurysm). Color flow by
toma within the media without identifiable interruption of the Doppler echocardiography or IV contrast by CT enters the ulcer crater.
intima. Penetrating atherosclerotic ulcers are most likely
caused by atherosclerosis with subsequent erosion across the
internal elastic membrane of the aorta, allowing for a blood co m p l i ca t ion s. The c lassic prese n t a t i o n cons i s t s of .. aort i c
filled false space within the wall of the aorta. chest P<l in ·· described a s severe ripping o r tearing pa i n t ha t
The Stanford classification describes type A dissections as may rc1 d i a t c t o t he c1 ntcrior chest or back, jaw. or abdomen.
originating within the ascending aorta or arch, whereas type B clepen d i ng o n vvh i c h segm e n t o f t he aorta is i nvolved.
dissections originate distal to the left subclavian artery. This Hypert e nsion i s t he most i m porta n t risk factor: o t her risk fac
nomenclature has been generalized to all of the acute aortic tors i nclude smoki ng a nd atherosclerosis. In t he setting of an
syndromes, although most intramural hematomas and pene acute <lO rt ic d i ssec t i o n . hypertension a n d an aort ic regurgi t a
trating ulcers are type B lesions. t i o n m u r m u r t ha t is fa i n t . s h o rt in d u ra t i on . a n d low i n pi t c h
98
D iseases of the Aorta
c:J (<0 . 5 p g/ml [0: 5 �g/L]) .suggests against an acute aortic syn Aortic Atheroma
drome. In aortic d1ssect1on, chest radiography may demon-
CONT. Aortic atherosclerotic plaques are a manifestation of systemic
strate a widening of the mediastinum. An electrocardiogram is
atherosclerosis. Aortic atheroma may be detected incidentally
often abnormal. but nondiagnostic. Clinical suspicion should
during imaging (Figure 34) . The risk of embolism and stroke
be high, and CT, M R I . or TEE for confirmation of the diagnosis
in patients with aortic atheroma is significantly increased for
should not be delayed.
plaques that are mobile or protruding, particularly if the
plaque is greater than 4 mm in size. Thromboembolism may
Treatment
also result from dislodgment of debris from the aortic wall
Patients with a suspected acute aortic syndrome who are not
occurring as a complication of an invasive cardiovascular pro
in cardiogenic shock should receive medical therapy to control
cedure, such as catheterization, intra-aortic balloon pump
heart rate and blood pressure. Intravenous �-blockers should
placement, or vascular surgery.
be used to target a heart rate of below 70/min. In patients
Noncoronary atherosclerotic disease, including aortic
requiring additional blood pressure control. a rapidly titratable
atheroma, is a coronary heart disease risk equivalent and,
antihypertensive medication, such as sodium nitroprusside,
therefore, should be aggressively treated to reduce the risk of
labetalol. enalaprilat, hydralazine, or nicardipine, should be
future cardiovascular events, including treatment with anti
given intravenously, with a goal of decreasing the mean arte
platelet agents and statins. In an observational study, statin
rial pressure to the lowest level that still allows visceral and
therapy was associated with a 17% absolute reduction in
cerebral perfusion.
thromboembolic events in patients with aortic atheroma.
Emergency surgery is recommended for all patients with
type A aortic dissection as well as for type A intramural hema KEY POINT
toma. Any delays to surgery should be avoided or addressee!. as • Aortic atheroma is a coronary heart disease risk equiva
type A dissection has a very high short-term mortality rate. lent, and patients should be aggressively treated with
Concomitant aortic arch reconstruction. coronary artery antiplatelet agents and statins to reduce their cardiovas
reimplantation, aortic valve repair or replacement, or branch cular risk.
vessel repair may be required depending on the anatomy and
pathology of the lesion.
Uncomplicated type B aortic syndromes may be treated
Abd o m i n a l Aortic Ane u rysm
medically. The INSTEAD trial, which enrolled subjects with
Screening and Surveillance
uncomplicated chroni c type B dissection, showed no dif
An abdominal aortic aneurysm (AAA) is considered to be
ference in clinical or aortic outcomes at 2 years for patients
present when the minimum anteroposterior diameter of the
treated with TEVAR versus medical therapy alone. Among
aorta reaches 3.0 cm. The most important risk factors for AAA
patients who survived at 2 years. additional long-term fol
are increasing age, smoking, and male sex (men with AAA
low-up of the I NSTEAD trial demonstrated that TEVA R in
outnumber women by up to 6:1) . Other risk factors include
addition to optimal medical treatment was associated with
atherosclerosis, hypertension. and family history of AAA.
i m proved long-term aorta-speci fic survival and delayed
disease progression. S u rgery is i ndicated for compl icated
type B aortic dissection defined by refractory pain or
hypertension . rapid aneurysmal expansion, rupture, o r
malperfusion syndrome. c:J
KEY P O I NTS
• The classic presentation o f a n acute aortic syndrome
consists of "aortic chest pain" -severe ripping or tearing
pain that may radiate to the anterior chest or back, jaw,
or abdomen, depending on which segment of the aorta
is involved.
• Findings that increase the index of suspicion for an
acute aortic syndrome include pulsus paradoxus, asym
metric blood pressure in the upper extremities, and an
asymmetric pulse examination.
• Type A aortic dissection has a very high short-term
mortality rate, and emergency surgery is recommended
for all patients without delay. F I G U R E 3 4 . Transesophageal echocardiogram demonstrating a n aortic ather·
oma in the descending aorta (arrows). Left panel: short axis view; right panel: long
axis view.
99
Pe r i p h eral Arte rial Disease
Based on randomized data, the U.S. Preventive Services Task and aort ic anatomy. Random ized tri a ls comparing open repair
Force guidelines recommend a one-time ultrasonographic wi lh F.VA R have found sign i fica n t ly i mp roved shon -term
screening in men aged 65 to 75 years who are active or former (30 clay) morbidity and mortal i ty for E\IAR. but no sign ificant
smokers (see MKSAP 17 General Internal Medicine). The sen d ifferences in long- term mortal ity. Moreover. in the long term.
sitivity and specificity of ultrasonography for detection of E\IA R was associated with increased complication rates (includ
AAA are excellent. ing endovascular leaks. de\·ice migra t ion and fail ure. and post
AAA rupture has an exceedingly high mortality rate, yet implan tat ion syndrome) and the need [or re intervent ions.
most AAAs never rupture. Thus, deciding when and in whom Because of these potent i a l complications. patients who have
to intervene and electively repair an AAA is of major impor undergone E\IAR of' AAA req ui re dil igent fo l low-up wi t h imag
tance. The strongest risk factor for the rupture of an AAA is ing studies performed annually to eva l uate the status or t he
maximal aortic diameter; this measurement is the dominant graft. Additional long-term data from prospect ive randomized
indication for repair. Estimated annual rupture risk according trials are needed t o fu l ly eva luate t he benefit risk prof1le of' open
to AAA diameter is shown in Table 43. AAA repair versus E\IAR. In patients with severe comorbid d is
After AAA has been identified, surveillance imaging ease considered not eligible for open surgical correction. a trial
results determine the timing of repair. The frequency of sur o r E\IA R versus medical t herapy demonstrated no d if'!erence in
veillance is dependent on baseline aneurysm size; larger aneu al l-cause mort a l i ty a t 8 years. but greater cost and i ncreased
rysms expand faster than smaller ones and may require more complicat ions were associated wi t h endovascular repair. Cl
frequent surveillance. If AAA maximum diameter is 3.5 to
KEY P O INTS
4.4 cm, repeat ultrasonography is recommended annually; if
maximum diameter is 4.5 to 5.4 cm, repeat ultrasonography • The most important risk factors for abdominal aortic
should be performed every 6 to 12 months. Elective repair aneurysm are increasing age, smoking, and male sex;
should be considered for AAA of 5.5 cm in diameter, for those one-time ultrasonographic screening is recommended
that increase in diameter by more than 0.5 cm within a for men ages 65 to 75 years who have ever smoked.
6-month interval, and for those that are symptomatic (tender • Elective repair should be considered for an abdominal
ness or abdominal or back pain). For women, elective repair aortic aneurysm of 5.5 cm in diameter, for those that
may be considered for an AAA that reaches 5.0 cm in diameter. increase in diameter by more than 0.5 cm within a
6-month interval, and for those who are symptomatic.
Treatment • Medical therapies for abdominal aortic aneurysm focus
Medical therapies for AAA focus on targeting modifiable risk on targeting modifiable risk factors for abdominal aortic
factors for AAA and cardiovascular disease with the goals of aneurysm and cardiovascular disease; smoking cessa
reducing aneurysm expansion or rupture, reducing morbidity tion is the cornerstone of therapy for active smokers.
and mortality associated with repair, and reducing cardiovas
cular morbidity and mortality. Smoking cessation is the cor
nerstone of therapy for active smokers.
1 00
Pe ri p heral Arterial D isease
• Classic intermittent claudication (10%-35%) symptoms of leg ischemia, but many are asymptomatic.
• Ischemic pain and ulceration in a lower extremity from Among symptomatic patients, the perception of symptoms
chronic limb ischemia (1%-2%) can vary from minimal to severe and may not necessarily cor
relate with severity of disease. Discriminating claudication
PAD is associated with reduced exercise capacity and func
from pseudoclaudication (a result of narrowing of the lumbar
tional status regardless of the symptomatic state. Limb-specific
spinal canal) is important (Table 44) . In severe PAD or critical
outcomes (such as limb ischemia or amputation) occur less
limb ischemia, patients may describe discomfort that worsens
frequently than systemic manifestations of atherosclerosis
with elevation of the leg and improves with dependent posi
(myocardial infarction or stroke) . Both asymptomatic and
tioning, such as dangling the leg off the foot of the bed.
symptomatic patients with PAD are at increased risk of cardio
Components of the physical examination of patients with
vascular morbidity and mortality, and PAD is considered a coro
suspected PAD are shown in Table 45. Patients at risk for lower
nary heart disease risk equivalent. Patients with PAD are likely
extremity PAD should undergo comprehensive pulse exami
to have concomitant vascular disease in other arterial beds and
nation and inspection of the feet. Shoes and socks are removed
have an annual cardiovascular event rate of 5% to 7%. Thus,
and the feet inspected for skin changes. Physical examination
early recognition of PAD provides a unique opportunity to iden
may reveal diminished, absent, or asymmetric pulses below
tify persons at early increased risk for a cardiovascular event
the level of stenosis, with occasional bruits over stenotic
and to modify risk factors. However, the U.S. Preventive Services
lesions and evidence of poor wound healing. Other physical
Task Force concluded that the current evidence is insufficient to
findings seen in PAD include a unilaterally cool extremity; a
assess the balance of benefits and harms of screening for PAD
prolonged venous filling time (>20 seconds); shiny atrophied
and cardiovascular disease risk assessment with the AB! in
skin; ulceration; and thickened, ridged, and brittle nails. Most
adults. Initial testing may be considered in persons with the fol
patients with upper extremity PAD are asymptomatic, and
lowing characteristics that signify high risk:
PAD may be detected only by the finding of asymmetric arm
• Exertional leg discomfort blood pressures, with a typical differential in systolic blood
• Nonhealing wounds pressures of greater than 15 mm Hg.
• Age 50 years or older with a history of smoking or diabetes
Diagnostic Testing
KEY P O I NTS There are several diagnostic modalities to choose from to
• Patients with peripheral arterial disease are likely to have assess for PAD. Measurement of the AB! is a simple, inexpen
concomitant vascular disease in other arterial beds and sive, and noninvasive technique that correlates well with
have an annual cardiovascular event rate of 5% to 7%. angiographic disease severity and functional symptoms.
• Initial testing for peripheral arterial disease may be Patients should rest for 5 to 10 minutes before measuring the
considered in persons with exertional leg discomfort, ankle pressure and should be lying flat for an accurate ABI
nonhealing wounds, or aged 50 years or older with a measurement, with the head and heels fully supported. The
history of smoking or diabetes mellitus. study is performed by applying a blood pressure cuff to the
calf and measuring the systolic blood pressure by palpation or
Doppler at the ankle. The blood pressure is recorded for both
the dorsalis pedis (DP) and the posterior tibial (PT) arteries,
Eva l u ation and the higher of the two is used as the ankle pressure. This
H istory and Physical Examination procedure is repeated for the opposite ankle. The brachia!
Patients at risk for PAD should undergo a vascular review of artery systolic blood pressure is measured in a similar fashion
symptoms to assess walking impairment, claudication, and in both arms. For the measurement of AB!, measuring the
ischemic pain while at rest. Patients with PAD may have limb pressures in the following order is recommended: first
Nature of d iscomfort Cra m ping, tig htness, aching, fatigue Same as claudication plus tingl ing,
burning, n u m bness, weakness
Location of discomfort B uttock, h i p, thigh, calf, foot Same as claud ication; most often bilateral
Exercise-induced Yes Variable
1 01
Pe riph eral Arterial Disease
TABLE 45. Clinical Examination of Patients for Peripheral repetitive active pedal plantar flexion ("toe ups") while stand
Arterial Disease ing has been proposed for an office-based assessment of pos
texercise ABI. In patients with even mild PAD, the ankle
Measure blood pressure in both a rms (systo lic blood pressure
d ifference > 1 5 mm Hg is suggestive of subclavian ste nosis) pressure decreases more during treadmill exercise compared
with healthy patients, and the recovery time to the pre-exer
Auscu ltate for presence of arterial bruits (e.g., femoral artery)
cise value after exercise cessation is prolonged, proportional to
Pa l pate for presence of an abdominal aortic aneurysm
the severity of PAD. A decrease of the AB! by 20% after exercise
Pal pate and record pulses ( radial, brachia!, carotid, femoral, suggests significant PAD.
popliteal, posterior tibial, dorsa l i s pedis)
A common procedure in many vascular laboratories is
Evaluate for elevation pallor and dependent rubor
measurement of multiple or segmental pressures in the lower
I n spect feet for ulcers, fissures, call uses, tin ea, and tendin ous extremities along with pulse volume recordings, which meas
xanthoma; evalu ate overa l l skin care
ure the magnitude and contour of the blood pulse volume.
Once a patient is placed in a supine position for 5 to 10 min
arm, first PT artery, first DP artery, other PT artery, other DP utes, blood pressures are obtained at successive levels of the
artery, and other arm. If the systolic blood pressure of the first extremity, localizing the level of disease fairly accurately.
arm exceeds the systolic blood pressure of the other arm by Noninvasive angiography may be performed for ana- �
more than 10 mm Hg, measurement of the blood pressure of tomic delineation of PAD in patients requiring surgical or W
the first arm should be repeated (to temper the "white coat endovascular intervention. CT angiography (CTA) is rapid
effect" of the first measurement) , and the first measurement and easily available but requires the administration of intra
of the first arm should be disregarded. For each leg, the AB! is venous contrast dye. The risk of dye-induced nephropathy
calculated by dividing the ankle pressure by the higher of the must be considered in patients with chronic kidney disease,
two brachia! pressures. I n healthy persons, the ankle and arm especially if an endovascular repair is being contemplated,
systolic pressures are approximately the same or slightly because this would entail repeat administration of iodinated
higher in the ankle. Thus, a normal resting AB! is between contrast dye. Magnetic resonance angiography (MRA)
1.00 and 1 .40 (Table 46). However, ifa fixed obstruction of the requires intravenous gadolinium for PAD definHion; gado
arterial lumen supplying the lower extremity is present, a linium has been associated with nephrogenic systemic fibro-
pressure gradient occurs, resulting in a reduced downstream sis in patients with severe kidney disease. Additionally, MRA
pressure and concomitant reduction in the AB! (AB! <0.90). may be contraindicated in patients with implanted pacemak-
AB! values between 0.91 and 0.99 are considered borderline, ers or cardioverter-defibrillators. Both CTA and MRA com-
and while equivocal for PAD, these patients are at increased pare favorably with digital subtraction (invasive) angiography
risk for adverse cardiovascular events. An AB! greater than for the detection of occlusive arterial disease. CTA has addi
1 .40 is associated with calcification of the arterial wall and tional benefits of demonstrating vascular calcification, has
may occur in patients with medial calcinosis, diabetes melli higher spatial resolution than M RA, and aJ!ows visualization
tus, or end-stage kidney disease. This finding is uninterpret of adjacent soft tissues and of endovascular stent grafts.
able, and therefore a toe-brachia! index may be useful for Invasive angiography. in most instances, is used only as part
diagnosing PAD in patients who are at risk for PAD and have of an interventional procedure. CJ
an ABI above 1.40. A toe-brachia! index below 0.70 is consid
K E V P O I N TS
ered diagnostic of PAD. This test is typically performed in a
vascular laboratory. • An ankle-brachia! index of 0.90 o r below i s diagnostic
The sensitivity of the ABI is increased when it is measured for peripheral arterial disease.
after exercise. A common exercise protocol involves walking • An ankle-brachia! index greater than 1.40 is uninter
on a treadmill at 2 mph at a 12% incline for 5 minutes or until pretable, and a toe-brachia! index should be obtained
the patient is forced to stop because of leg pain. Alternatively, for diagnosing peripheral arterial disease.
• An exercise ankle-brachia! index (ABI) may help estab
TAB L E 46. Interpretation ofthe Ankle·Brachial Index lish the diagnosis of peripheral arterial disease (PAD)
Ankle·Brachial Index Interpretation in patients in whom the resting ABI is equivocal; a
> 1 .40 decrease of the AB! by 20% after exercise suggests
Noncompressible (calcified)
vessel ( u n i nterp reta ble result) significant PAD.
1 .00- 1 .40 Normal
0.91 -0.99 Borderline M ed ica l Thera py
0.41 -0.90 Mild to moderate PAD The treatment of PAD has evolved over the past decade to
0. 00-0 . 4 0 Severe PAD include a broad approach, focusing on the reduction of adverse
cardiovascular events, improving symptoms in claudication,
PAD = peripheral arterial disease.
and preventing tissue loss in critical limb ischemia.
1 02
Periph eral Arterial D isease
Cardiovascular Risk Reduction Diabetes is one of the strongest risk factors for PAD. Although
Cigarette smoking is the strongest risk factor for PAD and for intensive diabetic control (hemoglobin A1c �7%) can be effective
subsequent complications, and smoking cessation is impera to reduce microvascular complications, no prospective studies
tive for patients with PAD. Smokers with PAD who quit have have demonstrated that this strategy reduces macrovascular com
lower risks for myocardial infarction and stroke and an plications, including PAD. In general, diabetes treatment of
improved long-term survival versus those who continue to patients with PAD should follow current national treatment
smoke. Cessation of cigarette smoking is also associated with a guidelines, with meticulous attention paid to foot care.
lower amputation rate, lower incidence of rest ischemia,
improvement in maximal treadmill walking distance, and Symptom Relief
improved bypass graft patency rates. Exercise rehabilitation is a class I, level of evidence A, recom
A moderate- or high-intensity statin is indicated in all mendation for the treatment of claudication in patients with
patients with PAD. Although no prospective statin trials have PAD. Either treadmill exercise or resistance training improves
focused singularly on PAD, the Heart Protection Study (HPS) functional performance, but treadmill exercise has shown
studied 6748 patients with PAD. Use ofsimvastatin was associ greater increases in the 6-minute walk distance and in the
ated with relative reduction in overall mortality by 12%, vascu maximum treadmill walking time compared with resistance
lar mortality by 17%, and cardiovascular events by 24% at a training. Although supervised exercise therapy has statistically
mean follow-up of 5 years. significant benefit on treadmill walking distance compared with
Antihypertensive therapy is effective at reducing cardio non-supervised regimens, a randomized trial found benefit in
vascular events in patients with PAD. Current guidelines rec both a supervised exercise and a home-based exercise program
in patients with claudication. The National Institutes of Health
ommend a brachia! blood pressure goal of less than
140/90 mm Hg for patients with PAD. Whereas cardiovascular funded CLEVER trial evaluated the relative efficacy, safety, and
groups have advocated a lower target of 130/80 mm Hg for health economic impact of noninvasive and endovascular treat
patients with PAD and diabetes or kidney disease, the recently ment strategies for patients with aortoiliac PAD and claudication.
Whereas supervised exercise provided a superior improvement
released report of the Eighth Joint National Committee (JNC 8)
in walking outcomes, patients undergoing endovascular repair
suggests a therapeutic target of 140/90 mm Hg with or with
had greater improvements in self-reported physical function.
out diabetes or kidney disease. Data are insufficient to recom
Cilostazol is a phosphodiesterase inhibitor with antiplate
mend use of one class of antihypertensive agents over another.
let activity and vasodilatory properties. A meta-analysis of
Concern has been raised regarding the use of �-blockers in
eight randomized trials that included 2702 patients with PAD
the treatment of hypertension in the setting of intermittent
with claudication found that cilostazol improved maximum
claudication; however, data have not supported this concern.
and pain-free treadmill walking distance and quality-of-life
Thus, �-blockers are not contraindicated in patients with PAD
measures. Cilostazol may have an additional benefit of reduc
and should be used when appropriate. Nonetheless, thiazide
ing restenosis and repeat revascularization following endovas
diuretics, ACE inhibitors or angiotensin receptor blockers,
cular therapy. Because milrinone, another phosphodiesterase
and calcium channel blockers remain first-line therapy in
inhibitor, increased mortality in patients with heart failure,
patients with hypertension. In the Heart Outcomes Prevention
cilostazol should not be used in this population. In the absence
Evaluation (HOPE) trial, ramipril (5 to 10 mg/d) decreased
of heart failure, a therapeutic trial of cilostazol should be con
cardiovascular events in subjects with PAD with or without
sidered in all patients with lifestyle-limiting claudication.
hypertension. The use of an ACE inhibitor is reasonable in
Pentoxifylline is a xanthine derivative that affects platelet
patients with lower-extremity PAD to reduce the risk of
adhesiveness and whole-blood viscosity. A meta-analysis
adverse cardiovascular events.
demonstrated a modestly improved walking distance with
Current guidelines recommend antiplatelet therapy in
pentoxifylline, but it was substantially less effective than either
patients with PAD. Because most patients with PAD have con
cilostazol or a supervised exercise program.
comitant coronary artery disease or cerebrovascular disease, aspi
Some data have shown that therapy with statins and ACE
rin is an acceptable antiplatelet agent in this setting despite the
inhibitors leads to substantial improvements in pain-free
lack of good-quality evidence supporting improved outcomes in walking time and maximal walking time.
PAD. In addition to aspirin, clopidogrel (as monotherapy, not with
aspirin) is a reasonable alternative strategy, and some data suggest KEY P O I NTS
clopidogrel may be particularly effective in patients with PAD. • Smoking cessation, lipid control, antiplatelet medica
Oral anticoagulation with warfarin has not been estab tions, and antihypertensive therapy are important com
lished to reduce cardiovascular events in patients with PAD ponents of cardiovascular risk reduction in patients
because it is no more effective than antiplatelet therapy and with peripheral arterial disease.
confers a higher risk of bleeding. There are no data to suggest • Exercise therapy and cilostazol are effective treatments
using newer anticoagulants in patients with PAD for the for claudication.
reduction of cardiovascular events.
1 03
Per i p h e ra l Arteria l Disease
- --- --------
• The patient has not had an adequate response in symptom • Patients with critical limb ischemia should be consid
alleviation to exercise rehabilitation and pharmacologic ered for immediate revascularization, either surgical or
therapy. endovascular.
1 04
Card iovascu l a r D isease in Ca ncer S u rvivors
M a rginally threatened None to minimal (toes) None Inaudible Salvagea ble with
prompt treatment
Immed iately threatened More than toes M i l d to moderate Inaudible Salvagea ble only
with immed iate
revascula rization
I rreversi ble Profound anesthesia Profound/paralysis Inaud i ble Not viable; major tissue
loss inevitable
Adapted from Hirsch AT, Ha ska I ZJ, Hertzer N R , eta!; American Association for Vascular Surgery; Society for Vascular Surgery; Society for Cardiovascular Angiography and
Interventions; Society for Vascular Medicine and Biology; Society of lnterventional Radiology; ACC/AHA Task Force on Practice Guidelines Writing Committee to Develop
Guidelines for the Management of Patients With Peripheral Arterial Disease; American Association of Cardiovascular and Pulmonary Rehabilitation; National Heart, Lung, and
Blood Institute; Society for Vascular Nursing; TransAtlantic Inter-Society Consensus; Vascular Disease Foundation. ACC/AHA 2005 Practice Guidelines for the management of
patients with peripheral arterial disease (lower extremity, renal, mesenteric, and abdominal aortic): a collaborative report from the American Association for Vascular Surgery/
Society for Vascular Surgery, Society for Cardiovascular Angiography and Interventions, Society for Vascular Medicine and Biology, Society of lnterventional Radiology, and the
ACC/AHA Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Patients With Peripheral Arterial Disease): endorsed by the
American Association of Cardiovascular and Pulmonary Rehabilitation; National Heart, Lung, and Blood Institute; Society for Vascular Nursing; TransAtlantic Inter-Society
Consensus; and Vascular Disease Foundation. Circulation. 2006 Mar 21 ; 1 1 3( 1 1 ):e488. IPMID: 1 6549646]
KEY POI NTS Although pericarditis may occur acutely with chest radia
tion, manifestations of radiation-induced cardiotoxicity fre
• Physical findings of acute limb ischemia are character
quently develop after a long indolent period (5 to 20 years or
ized by the "6Ps" - Paresthesia, Pain, Pallor,
later) owing to the chronic nature of the pathology and there
Pulselessness, Poikilothermia (coolness) , and Paralysis.
fore require a high index of suspicion in at-risk patients. The
• Patients with acute limb ischemia should receive imme clinical manifestations are related to the affected portion of
diate anticoagulation therapy; those with a salvageable the cardiovascular system. Myocardial fibrosis leads to a
extremity should undergo an emergent evaluation that restrictive cardiomyopathy, resulting in poor chamber compli
defines the anatomic level of occlusion and leads to ance and diastolic heart failure (see Myocardial Disease) . Signs
prompt endovascular or surgical revascularization. of restrictive cardiomyopathy have been reported in 15% to
• Following limb reperfusion in patients with acute limb SO% of patients with previously treated Hodgkin lymphoma
ischemia, close monitoring is required for limb edema and are more evident in those who have received therapy with
and tissue swelling that may cause a compartment cardiotoxic drugs. Constrictive pericarditis also frequently is
syndrome. present. The potential for coexistent constrictive pericarditis
and restrictive cardiomyopathy presents significant challenges
in the management of these patients, as the clinical manifesta
tions frequently overlap while the treatments for these two
Ca rd iovascu lar Disease disorders vary considerably (that is, pericardiectomy versus
cardiac transplantation) . Pericardia! effusion is particularly
in Ca ncer S u rvivors common in patients treated for esophageal cancer (approxi
mately 25%) , with a median presentation time of 5 months
Ca rd iotoxicity of Radiation after therapy in one report. Any cardiac valve can be affected
Thera py to the Thorax by radiation injury (5%-40% of patients) , although left-sided
Chest radiation therapy is associated with significant cardio lesions predominate and frequently occur with mixed stenosis
toxicity, which can manifest as a complex, life-threatening and regurgitation. Coronary artery disease typically is ostial or
disorder. Radiation-induced toxicity can affect nearly every proximal in location; microvasculopathy (disease involving
component of the cardiovascular system, with manifestations vessels that are not epicardial in location) also can occur.
such as pericarditis (acute or chronic) , cardiomyopathy. aorti Recognition of the potential for cardiotoxicity has led to
tis, conduction system disease, valvulopathy, and coronary reductions in radiation exposure in the treatment of chest
artery disease (Table 48) . These pathologic alterations are malignancy. Most contemporary studies have demonstrated
likely caused by the generation of reactive oxygen species from significant decreases in cardiac mortality in comparison with
irradiation, which leads to blood vessel injury and a cascade of historical studies. However, the increased risk of cardiotoxicity
inflammation, ischemia with loss of capillary density, and and death due to vascular complications attributable to radia
fibrosis of the cardiovascular structures. tion therapy remains. Thus, lifetime clinical monitoring for
1 05
Cardiovascu l a r Disease i n Cancer S u rvivors
Acute pericarditis or pericardia! As early as 2 months, but A common cardiac compl ication historically ( 25%), less
effusion 5 months on average com mo n now ( 2% ) owing to methods m i n i m izing mediastinal
irradiation
Pericardia! fibrosis and As early as 1 .5 years, but often Risk persists for >25 years
constriction 1 0 to 1 5 years or more
RV more exte nsively involved, leading to ma rked fi nd ings of RV
fa i l u re
Acce lerated coronary Average o nset 7 years Pred i l ection for involvement of ostia or proximal segments of
atherosclerosis coronary arteries
Patients with traditional risk factors for CAD are at hig her risk
Valvu l a r fibrosis and 1 0 to 25 years or more Frequency greater i n left- vs. right-sided valves
reg u rg itati on
Clini ca lly significant aortic regurgitation may occur i n 2:25% of
lo ng-term survivors
Myocardial fibrosis, diasto l i c Years Concomitant anthracycline use i ncreases risk for heart fa ilure
dysfu nction, and restrictive
cardiomyopathy
Fibrosis of conduction pathways Years or decades
lead ing to bradycard ia,
dysrhythmias, or heart block
radiation-induced cardiotoxicity is warranted owing to the toward the predominant pathology, although it is recognized
chronic, lethal nature of this complication. Propensity for that concomitant abnormalities increase the surgical proce
radiation-induced injury is related to younger age at treat dural risk. For example, patients undergoing pericardiec
ment, female sex, radiation exposure (total dose, dose per frac tomy will be at significant operative risk owing to the
tion, and cardiac chamber affected) , the use of concomitant propensity for increased surgical bleeding and myopathy in
cardiotoxic drugs (such as anthracyclines) , and the presence of these patients. Thus, a high degree of individualization of the
cardiac risk factors (hypertension, smoking, hyperlipidemia) . treatment plan for patients with radiation-induced heart
There is no clearly defined threshold of radiation exposure for disease is recommended.
cardiotoxicity risk. In all patients with a history of significant
K E Y P O I N TS
chest radiation, aggressive management of risk factors for ath
erosclerosis is warranted owing to the heightened risk of • I n all patients with a history o f significant chest radia
ischemic heart disease in these patients. tion, aggressive management of risk factors for athero
Although contemporary studies have shown a lower inci sclerosis is warranted owing to the heightened risk of
dence of radiation-induced cardiotoxicity, the follow-up in ischemic heart disease in these patients.
these studies has been relatively short (frequently 5 to 10 years) • Cardiotoxicity should be considered in any patient with
and thus may be inadequate to ascertain the indolent effects of a history of chest radiation who develops symptoms or
radiation-induced cardiotoxicity. Recent analyses frequently signs of cardiovascular disease.
have focused on mortality rates without detailed examinations
of other complications, such as myopathy, valvular disease,
and constrictive pericarditis. The timing and clinical methods Ca rd iotoxicity of Chemothera py
for serial monitoring have not been defined, but cardiotoxicity Cardiotoxicity from chemotherapy can result from traditional
should be considered in any patient with symptoms or signs of cytotoxic chemotherapy agents, such as anthracyclines (doxo
cardiovascular disease and a history of chest radiation. rubicin, daunorubicin, mitoxantrone), as well as from newer
Owing to the frequent multiple cardiac pathologies in agents, such as monoclonal antibodies (trastuzumab) and
these patients, management of radiation-induced heart dis tyrosine kinase inhibitors. Cardiotoxicity can occur in patients
ease can be challenging. Treatment is directed primarily with normal hearts but is more common in patients with
1 06
Cardiovasc u l a r D isease in Cancer S u rvivors
preexisting cardiac disease. Cardiotoxicity from these agents occurring within 1 week after presentation. Chronic cardio
can manifest as dilated cardiomyopathy, myocardial ischemia toxicity due to anthracyclines, which begins with a subclinical
from coronary vasospasm, or arrhythmias (Table 49) . decline in systolic and diastolic function, manifests with
The cardiotoxic effects of chemotherapy can be short symptoms usually within months after completion of chemo
term, intermediate, or long-term. 5-Fluorouracil is associated therapy. However, cardiotoxicity from anthracyclines can have
with a high incidence of acute chest pain and electrocardio long latency periods (10 years or more) .
graphic changes (70% within 72 hours of the first treatment The strongest risk factor for cardiotoxicity related to
cycle) , resulting in death in 2% to 8% of patients affected by anthracycline agents is cumulative dose. The incidence of car
5-fluorouracil toxicity. diotoxicity for doxorubicin or daunorubicin has been reported
Early manifestations of anthracycline toxicity are rela to be less than 1 % for cumulative dose of less than 400 mg/m2,
tively uncommon (3%) and include high-grade heart block, but 26% for cumulative doses of 550 mg/m2 or more. It is gen
supraventricular and ventricular arrhythmias, heart failure, erally accepted that maximum cumulative doses for these
myocarditis, and pericarditis, with resolution in many patients drugs should be limited to 450 to 500 mg/m2 , but the doses
Mitoxantrone Heart fa i l u re
Alkylating agents
Cisplatin Heart fa i l u re
Mitomycin Cardiomyopathy
5-Fluorouracil Vasospasm (common) and heart fa i l u re (rare) from myocard ial i nfarctio n occurring d u ring treatment
Trastuzumab Heart fa i l u re
lnterleukin-2 Heart fai l u re from previous cardiomyopathy, myocarditis, or myocard ial infarction occurring during
treatment (rare)
I nterferon-a Heart fai l u re from previous myocard i a l infarction during treatment (rare)
1 07
Pregna ncy a n d Cardiovascu l a r D isease
that lead to toxic responses vary considerably among individ for serial monitoring of patients who have undergone chemo
ual patients. Other risk factors for cardiotoxicity include age at therapy, the thresholds for these markers as well as the appro
treatment, concomitant therapy with other cardiotoxic agents, priate timing for their measurement remain uncertain.
chest radiation, and preexisting cardiac disease. The toxic Patients who have left ventricular dysfunction should receive
responses to anthracyclines can be modified by liposome appropriate therapy with �-blockers, vasodilators, and diuret
encapsulation of the molecule, infusional rather than bolus ics as in patients with heart failure disorders not related to
administration, use of structural analogues (epirubicin and chemotherapy toxicity.
mitoxantrone) , and adjunctive cardioprotective agents.
KEY POINTS
Dexrazoxane is an EDTA chelator that reduces the risk of
chronic cardiotoxicity associated with doxorubicin and epiru • Hypertension is a potential adverse effect o f kinase
bicin and may be considered in patients being treated with _inhibitors that may require dose adjustment or, in
high anthracycline doses (>300 mg/m2) . patients with severe hypertension, discontinuation of
Cardiotoxicity due t o trastuzumab typically causes a the kinase inhibitor.
chronic, asymptomatic decline in ventricular function with a • Cardiotoxicity related to trastuzumab is not dose related
low frequency of overt heart failure (3%-7%) . Older patients and is reversible.
(age >50 years) and those with prior or concomitant exposure • Chronic cardiotoxicity with anthracyclines is dose
to anthracyclines are at increased risk of trastuzumab-induced related and is not reversible.
cardiotoxicity. In most patients, cardiotoxicity due to trastu
• In patients who have undergone chemotherapy, base
zumab is related to changes in contractility and is reversible.
line evaluation and routine surveillance of cardiac func
Unlike anthracyclines, trastuzumab-related cardiotoxicity is
tion using echocardiography should be performed with
not dose related and patients can be successfully rechallenged
assessment of left ventricular ejection fraction as well
after recovery of ventricular function.
as indices of diastolic function.
Kinase inhibitors, such as tyrosine kinase inhibitors, are
a relatively new approach to tumor receptor-targeted therapy.
Hypertension is a potential adverse effect that may require
dose adjustment or, in patients with severe hypertension, dis Pregnancy and
continuation of the kinase inhibitor.
In adult patients being considered for chemotherapy with Ca rdiovascu lar Disease
anthracyclines, baseline evaluation of left ventricular function
should be considered before initiation of therapy, although the
Ca rd iovascu lar Changes
need for this assessment is controversial in patients with no D u ring Preg nancy
symptoms or signs of abnormal left ventricular function and Understanding the normal physiologic changes of pregnancy
in whom the cumulative dose is expected to be low (<300 mg/ is important in the interpretation of signs and symptoms in
m 2) . For patients who receive treatment with trastuzumab, a the pregnant patient (Table 50) . During a normal pregnancy,
baseline evaluation of left ventricular function should be per there is an increase in plasma volume and a lesser increase in
formed, particularly if there is a history of anthracycline use. erythrocyte mass, resulting in increased total blood volume
Routine surveillance of cardiac function using echocardiogra and relative anemia. The systemic vascular resistance
phy should be performed in all patients with assessment of left decreases during pregnancy, but the heart rate and cardiac
ventricular ejection fraction as well as indices of diastolic output rise; as a result, there is generally a slight reduction in
function. The timing intervals for these assessments are indi mean arterial pressure. Maternal cardiac output peaks at
vidualized based on the patient's baseline function, chemo approximately 40% above the prepregnancy level by the 32nd
therapeutic regimen, risk profile, and evidence of change in week of pregnancy and then plateaus until delivery. During
function in serial evaluations. delivery, heart rate and blood pressure increase, leading to a
In adults undergoing doxorubicin therapy, the drug rise in cardiac output to as much as 80% above the prepreg
should be discontinued if there is evidence of heart failure, a nancy level.
10% or greater decline in left ventricular ejection fraction to
below the lower limit of normal, an absolute left ventricular
ejection fraction of less than 45%, or a 20% decline in left ven Prepreg nancy Eva l uation
tricular ejection fraction to any level. Owing to the reversible Prepregnancy evaluation i s recommended for all women with
nature of cardiotoxicity related to trastuzumab, this therapy cardiovascular disease who are anticipating pregnancy.
can be resumed after recovery of left ventricular function in Patients with congenital heart disease should consult with a
selected patients. cardiologist specializing in congenital conditions and a high
Although other echocardiographic indices (such as strain risk obstetrician to discuss the need for genetic counseling,
imaging or volume measurements) and serum markers (car evaluate the risks of future pregnancy, and develop a plan for
diac troponin, B-type natriuretic peptide) have been proposed management during labor and the postpartum period.
1 08
Pregnancy a n d Ca rd i ovascu l a r D isease
TABLE 50. Normal Versus Abnormal Cardiac Symptoms and Signs in Pregnancy
Symptom or Sign Normal Pathologic
Shortness of b reath M i l d , with exerti on. Orthopnea, PND, cough
Pa lpitations Atrial and ventricu lar premature beats Atrial fi bril lation or fl utter; ventricular tachycard ia
Chest pain No Chest pressure, heaviness, or pain
M u rmur Basal systolic m u rm u r grade 1 /6 or 2/6 present i n Systo l i c m u rm u r grade '2:.3/6; any d iastolic m u rm ur
8 0 % o f pregnant women
Tachycard ia Heart rate in creased by 20%-30% Heart rate > 1 00/min
Low blood pressure Blood pressure typica l ly is modestly decreased Low blood pressure associated with symptoms
( - 1 0 m m Hg)
The CARPREG index is used to estimate risk for cardio KEV POI NTS
vascular complications during pregnancy in women with
• Women with severe pulmonary hypertension are at
cardiovascular disease (Table 51) . Women with severe pul
high risk for pregnancy-related death.
monary hypertension (pulmonary artery pressure 2'. two
thirds systemic pressure) are at high risk during pregnancy, • Systolic ventricular dysfunction (ejection fraction <40%)
with an estimated maternal mortality rate between 30% and with New York Heart Association (NYHA) functional
50%. Systolic ventricular dysfunction (ejection fraction class III or IV heart failure is considered a contraindica
<40%) with New York Heart Association (NYHA) functional tion to pregnancy.
class Ill or IV heart failure confers a high risk of maternaI and
fetal complications and is considered a contraindication to
Management of Ca rd iovascu lar
pregnancy.
Patients with severe obstructive cardiac disease, such as Disease D u ring Pregnancy
mitral or aortic valve stenosis, are generally considered for Because pregnancy involves an increase in blood volume and
intervention before pregnancy, even if asymptomatic. cardiac output, women with severe obstructive cardiac lesions
TABLE 5 1 . Predictors of Maternal Cardiac Events in Women with Congenital or Acquired Cardiac Disease (CARPREG Index)
Risk Factor (Predictor) Operational Definition
Previous cardiac event or arrhyth m ia Heart fa i l u re, transient ischemic attack, stroke, arrhythm ia
Base l i ne NYHA functional class I l l or IV or cyanosis M i l d symptoms ( m i l d sh ortness of breath and/or angina) and sli g ht l i m itation
during ord i nary activity
2
Left-sided heart obstruction Mitra I valve area <2 cm ; aortic valve a rea < 1 .5 cm2 or resting peak left ventricular
outflow tract g ra d ient >30 m m Hg
Data and recommendations from Siu SC, Sermer M, Colman JM, et al. Cardiac Disease in Pregnancy (CARPREG) Investigators. Prospective multicenter study of pregnancy
outcomes in women with heart disease. Circulation. 200 1 ; 1 04(5): 5 1 5-52 1 . [PMID: 1 1 4792461
1 09
Preg nancy a n d Cardiovascu l a r Disease
generally develop symptoms during pregnancy, whereas function, which can result in clinical deterioration or even
women with regurgitant valve lesions tolerate pregnancy rea death.
sonably well.
Vaginal delivery is generally preferred for patients with Other Cardiovascular Disorders
cardiovascular disease because it results in a shorter and less Women with Marfan syndrome have been reported to have an
marked hemodynamic derangement than cesarean delivery. increased risk of aortic dissection during pregnancy. Women
To reduce the risk of fetal intracranial hemorrhage, cesarean with Marfan syndrome and an ascending aortic diameter of
delivery is recommended in women receiving warfarin antico 4.5 cm or greater are recommended to have aortic repair sur
agulation therapy. Cesarean delivery is also recommended for gery before considering pregnancy to reduce this risk. Some
obstetric reasons and in select patients with severe pulmonary women with Marfan syndrome and aortic diameter less than
hypertension or a markedly dilated aorta. 4.5 cm are at high risk for dissection during pregnancy and are
counseled to have aortic valve replacement before pregnancy;
Peripartum Cardiomyopathy these include patients with rapid dilatation of the ascending
Left ventricular systolic dysfunction identified toward the end of aorta or a family history of aortic dissection.
pregnancy or in the months following delivery in the absence of Spontaneous coronary artery dissections in women with
another identifiable cause is known as peripartum cardiomyo out risk factors for coronary artery disease may occur in the
pathy. This occurs with increased frequency in women who are peripartum setting. Spontaneous healing may occur with con
multiparous, older (age >30 years), and black; in those with servative medical therapy, but revascularization, either perCL1-
multifetal pregnancy, gestational hypertension, or preeclamp taneous or bypass surgery, has been utilized as well.
sia; and in those treated with tocolytic agents.
The leading cause of pregnancy-related maternal death Cardiovascular Medication Use
in North America is peripartum cardiomyopathy. Death in During Pregnancy
women with peripartum cardiomyopathy results from Limited data are available on the safety of cardiovascular
heart failure, thromboembolic events, and arrhythmias. medications administered during pregnancy. The FDA catego
Half of women who develop peripartum cardiomyopathy rizes drugs by their fetal effects during pregnancy (see MKSAP
show improvement in left ventricular function within 6 17 General Internal Medicine, Women's Health). Most cardio
months of delivery, and 20% to 40% have normalization of vascular drugs are not FDA-approved for use during preg
ventricular function. nancy. General guidelines for the use of several cardiovascular
Prompt initiation of medical therapy is recommended for drugs during pregnancy are provided in Table 52.
women with peripartum cardiomyopathy and includes Cardiovascular medications should be used only when needed
P-blockers, digoxin, hydralazine, nitrates, and diuretics. ACE and at the lowest possible dose, and the desired therapeutic
inhibitors, angiotensin receptor blockers, and aldosterone effect should outweigh the risk.
antagonists should be avoided until after delivery owing to When P-blockers are used during pregnancy or lactation,
teratogenicity. Anticoagulation with warfarin is recommended periodic fetal and newborn heart rate monitoring and initial
for women with peripartum cardiomyopathy with left ven newborn blood glucose assessment are indicated because
tricular ejection fraction below 35%, owing to the increased P-blockers cross the placenta and are present in human breast
risk ofthromboembolism related to this disorder; the duration milk. Atenolol is usually avoided during pregnancy because it
of anticoagulation is individualized, and anticoagulation is has been reported to cause small fetal gestational size, early
discontinued when the ejection fraction improves. delivery, and low birth weight. Patients taking atenolol are
In women with acute severe periparturn cardiornyopa usually transitioned to a different P-blocker.
thy, brornocriptine, which blocks prolactin secretion, has The treatment of choice for acute symptomatic supraven
been shown to improve left ventricular ejection fraction and tricular tachycardia during pregnancy is adenosine. Recurrent
clinical outcomes when added to periparturn-related heart tachycardia symptoms are often treated with P-blockers and
failure therapy. Bromocriptine inhibits lactation and may digoxin; sotalol and flecainide have also been safely used.
increase risk of thromboembolism; therefore, anticoagula Amiodarone is rarely used owing to toxicity concerns.
tion is suggested in conjunction with brornocriptine. For Spironolactone is considered compatible with breast
these reasons, discussion with the patient is an important feeding; although spironolactone and its active metabolite,
precursor to initiating therapy. Referral for ventricular assist canrenone, appear in breast milk, the concentrations are
device or heart transplantation should be considered for pharmacologically insignificant. ACE inhibitors, angiotensin
women with refractory severe heart failure related to peri receptor blockers, and aldosterone antagonists are terato
partum cardiomyopathy. genic and should be avoided during pregnancy. Some ACE
Women with peripartum cardiomyopathy with persistent inhibitors are safe to use while breastfeeding. Angiotensin
left ventricular dysfunction should be counseled to avoid sub receptor blockers are generally avoided during lactation
sequent pregnancy since another pregnancy is often associ because data are inconclusive regarding infant risk when
ated with recurrent or further reduction of left ventricular used during breastfeeding.
110
Pregna ncy a nd Cardiovascular D isease
ACE i n h i b itors
Captopril, enalapril No Yes Teratogenic in first trimester; cause feta l/neonatal kidney
fai l u re with second or third trimester exposure; scleroderma
renal crisis is only ind ication
Lisinopril No ? Same as above
ARBs No ? Teratogenic i n first trim ester; cause feta l/neonatal kid ney
fai l ure with second or third tri mester exposure
Adenosine Yes ? No change in fetal heart rate when used for su praventricu lar
tachycard ia
Amiodarone No No Fetal hypothyro idism, prematurity
Anti platelet agents
Diltiazem, verapamil Yes Yes Second-line agent; maternal hypotension with rapid
intravenous infusion; used for fetal supraventricu lar
tachycardia
Diuretics Yes Yes Second-line agent; use when needed for maternal volume
overload only
Flecainide Yes ? Second-line agent; i nadeq uate data but used for feta l
arrhyth mia; case report of fetal hyperbilirubinemia
Lidocaine Yes Yes Treatment of choice for ventricu lar a rrhyth mias
Phenytoi n No Yes Known te ratogenicity and bleeding risk; last resort for
arrhyth mia
Propafenone Yes ? Second-line age nt; used for fetal arrhyth mia
Adapted from Rosene-Montella K, Keely EJ, Lee RV, Barbour LA. Medical Care of the Pregnant Patient. 2nd Edition. Philadelphia, PA: American College of Physicians; 2008.
p 356-357. Copyright 2008, American College of Physicians.
111
Pre g n a ncy a n d Cardiovascu l a r Disease
Anticoagu lation Therapy During Pregnancy adjustment are recommended for all anticoagulation regi
Pregnancy is a hypercoagulable state, and anticoagulation is mens. Warfarin is stopped before delivery owing to the risk
often indicated during pregnancy; regimens and levels of anti of fetal intracranial hemorrhage if spontaneous labor occurs
coagulation depend on the specific indication (Table 53) . while the mother, and thus the fetus, is anticoagulated with
Prepregnancy counseling is recommended for all women warfarin.
receiving chronic warfarin anticoagulation to help them Women with mechanical valve prostheses represent a
understand maternal and fetal risk and to make an informed high-risk subset of patients during pregnancy, with excess risk
decision regarding which anticoagulation regimen to use dur of valve thrombosis, bleeding, and fetal morbidity and mortal
ing their pregnancy. ity. The optimal anticoagulation regimen for this patient group
Unfractionated heparin, low-molecular-weight hepa has not been established. Warfarin anticoagulation during
rin (LMWH) , and warfarin can all be used for anticoagula pregnancy may be the safest agent for prevention of maternal
tion during pregnancy. Meticulous monitoring and dose prosthetic valve thrombosis; however, warfarin poses an
Weight-based LMWH
Weight-based LMWH
Warfarin ( I N R 2-3)
Atrial Fibrillation
Weight-based LMWH
Warfarin ( I N R 2-3)
aPTI = activated partial thromboplastin time; IV= intravenous; LMWH = low-molecular-weight heparin; SQ = subcutaneous; UFH = unfractionated heparin.
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of abdominal aortic aneurysm. N Engl J Med. 20!0 May 20:362(20) :1863-71. ment in young women planning on pregnancy: maternal and fetal out
[PMID: 20382983] comes under low oral anticoagulation, a pilot observational study on a
comprehensive pre-operat ive counseling protocol. J Am Coll Cardiol. 2012
Peripheral Arterial Disease Mar 20:59(12) : 1110-5. [PMID: 22421305]
Aboyans V, Criqui MH, Abraham P, et al: American Heart Association Council European Society of Gynecology (ESG): Association for European Paediatric
on Peripheral Vascular Disease: Council on Epidemiology and Prevention; Cardiology (AEPC); German Society for Gender Medicine (DGesGM),
Council on Clinical Cardiology; Council on Cardiovascular Nursing; Regitz-Zagrosek V, Blomstrom Lundqvist C, Borghi C, et al; ESC Committee
Council on Cardiovascular Radiology and Intervention, and Council on for Practice Guidelines. ESC Guidelines on the management of cardiovas
Cardiovascular Surgery and Anesthesia. Measurement and interpretation of cular diseases during pregnancy: the Task Force on the Management of
the ankle-brachia! index: a scientific statement from the American Heart Cardiovascular Diseases during Pregnancy of the European Society of
Association. Circulation. 2012 Dec 11: 126(24):2890-909. Erratum in: Cardiology (ESC). Eur Heart J. 20ll Dec:32(24):3147-97. [PM I D : 21873418]
Circulation. 2013 Jan 1: 127(l):e264. [PMID: 23159553] Siu SC. Sermer M. Colman JM, et al: Cardiac Disease in Pregnancy (CARPREG)
Adam DJ, Beard JD, Cleveland T, et al: BASIL trial participants. Bypass versus Investigators. Prospective multicenter study of pregnancy outcomes in
angioplasty in severe ischaemia of the leg (BASIL): multicentre, randomised women with heart disease. Circulation. 2001 Jul 3 1 ;104(5):515-21. [PM I D :
controlJed trial. Lancet. 2005 Dec 3:366(9501):1925-34. [PMID: 16325694) 1 1479246]
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Sliwa K, Blauwet L, Tibazarwa K, et al. Evaluation of bromocript ine in the Committee to Develop Guidelines on the Management o f Adults With
treatment of acute severe peripartum cardiomyopathy: a proof-of-concept Congenital Heart Disease); American Society of Echocardiography; Heart
pilot study. Circulation. 2010 Apr 6 ; 12 1 (13) : 1465-73. [PM I D : 20308616] Rhythm Society: International Society for Adult Congenital Heart Disease:
Sliwa K, Hilfiker-Kleiner D, Petrie MC, et al; Heart Failure Association of the Society for Cardiovascular Angiography and Interventions; Society of
European Society of Cardiology Working Group on Peripartum Thoracic Surgeons. ACC/AHA 2008 guidelines for the management
Cardiomyopathy. Current state of knowledge on aetiology, diagnosis, man of adults with congenital heart disease: a report of the American College of
agement, and therapy of peripartum cardiomyopathy: a position statement Cardiology/American Heart Association Task Force on Practice Guidelines
from the Heart Failure Association of the European Society of Cardiology (Writing Committee to Develop Guidelines on the Management of Adults
Working Group on peripartum cardiomyopathy. Eur J Heart Fail. 2010 With Congenital Heart Disease). Developed in Collaboration With the
Aug:I2(8) :767-78. [PM I D : 20675664] American Society of Echocardiography, Heart Rhythm Society, International
Society for Adult Congenital Heart Disease. Society for Cardiovascular
Warnes CA, Williams RG, Bashore TM. et al; American College of Cardiology: Angiography and Interventions, and Society of Thoracic Surgeons. J Am
American Heart Association Task Force on Practice Guidelines (Writing Coll Cardiol. 2008 Dec 2:52(23):el43- 263. [ PM I D : 19038677]
117
Cardiovascular Medicine
Self-Assessment Test
This self-assessment test contains one-best-answer multiple-choice questions. Please read these directions carefully
before answering the questions. Answers, critiques, and bibliographies immediately follow these multiple-choice
questions. The American College of Physicians is accredited by the Accreditation Council for Continuing Medical
Education (ACCME) to provide continuing medical education for physicians.
The American College of Physicians designates MKSAP 1 7 Cardiovascular Medicine for a maximum of 21 AMA PRA
Category 1 CreditsrM . Physicians should claim only the credit commensurate with the extent of their participation
in the activity.
);;>- Use the printed answer sheet at the back of this book to record your answers. Go to mksap.acponline.org,
access the appropriate online answer sheet, transcribe your answers, and submit your test for instantaneous
CME credits. There is no additional fee for this service.
);;>- Go to mksap.acponline.org, access the appropriate online answer sheet, directly enter your answers, and
submit your test for instantaneous CME credits. There is no additional fee for this service.
);;>- Pay a $15 processing fee per answer sheet and submit the printed answer sheet at the back of this book by
mail or fax , as instructed on the answer sheet. Make sure you calculate your score and fax the answer sheet
to 215-351 -2799 or mail the answer sheet to Member and Customer Service, American College of Physicians,
190 N. Independence Mall West, Philadelphia, PA 19106-1572, using the courtesy envelope provided in your
MKSAP 17 slipcase. You will need your 10-digit order number and 8-digit ACP ID number, which are printed
on your packing slip. Please allow 4 to 6 weeks for your score report to be emailed back to you. Be sure to
include your email address for a response.
I f you do not have a 10-digit order number and 8-digit ACP ID number or if you need help creating a user name and
password to access the MKSAP 17 online answer sheets, go to mksap.acponline. org or email custserv@acponline .org.
CME credit is available from the publication date of July 31, 2015, until July 31, 2018. You may submit your answer
sheets at any time during this period.
119
..�
· .·
Directions .:c""�·
Each of the numbered items is followed by lettered answers. Select the ONE lettered answer that is BEST in each case.
1 21
Self-Assessment Test
Item 8
A 58-year-old man is evaluated during a routine appoint
ment. He is asymptomatic. He was diagnosed with type 2
diabetes mellitus 4 years ago and has hypertension, dys
lipidemia, and obesity. H is medications are enteric-coated
low-dose aspirin, lisinopril, fluvastatin (20 mg/d), and
metformin.
H is calculated 10-year risk of atherosclerotic cardiovas
cular disease (ASCVD) using the Pooled Cohort Equations
is l0%.
On physical examination, blood pressure is 126/78 mm
Hg and pulse rate is 72/min. The remainder of the examina
tion is normal.
Laboratory studies:
Total cholesterol 186 mg/dL (4.82 mmol/L)
LDL cholesterol 123 mg/dL (3.19 mmol/L)
HDL cholesterol 44 mg/dL (1.14 mmol/L)
Triglycerides 109 mg/dL (1.23 mmol/L)
Which of the following is the most appropriate immediate Which of the following is the most appropriate statin man
next step in management? agement?
1 22
Self-Assessment Test
1 23
Self-Assessment Test
Item 1 4 Item 1 5
A 5 1 -year-o l d woman is a d m i t ted t o t h e hosp i t a l w i t h A 7LJ -yea r-old man is eva l ua ted in the emergency depa rt
com m u n i ty-acq u i red pneumonia a ft e r outpatient t herapy men t for a 7-day h i story of progressive exert ional dyspnea
was unsuccessfu l . She presen ted 5 clays ago w i t h cough . associated w i t h a d ry coug h . increasing ort hopnea rn·om
lever. and clyspnea . a n d she was found to have righ t lower two to lour p i llows) . and i na b i l i ty to buckle h is bel t . H e has
lobe crack les on ex<1 m i n a tion and a corresponding i n fi l t m t e a 20-year h istory of' hypert ension t reated wi t h d i l t iazem .
on chest radiography . She was started on ora l moxi f l ox On physical ex a m i na t ion. b lood pressure is 1 6 2/86 mm
aci n . However. she has remained febri le wi t h worsen i ng H g. pulse rate is i rregul arly irregular a t 8-1 111 i n . a n d res
short ness or breat h and is now acl m i t tecl to t h e hospi t a l for p i ra t ion ra t e is 1 8/m i n . Est imated central venous pressure
l'u rther tre<H men t . Medical h istory is sign i ficant l'or hyper is J . J cm H 20 . Cardiac examination reveal s a n irregularly
tension and depression . for wh ich s h e l a kes carved i l o l and i rregu lar rhyt h m and a n S 1 • Bibas i l a r crnckles are heard on
a m i t ri p ty l i n e. Her cu rre n t medications a re mox i l loxacin. <luscu l tat ion or t he lungs. H i s l iver is e n l arged 2 c m below
carvecli l o l . <lllcl a m i tripty l i ne . t h e cos tal margin . H is e x t re m i ty exa111 i nation revea ls bi l a t
On physica l exa111 i na t i o n . te111perature is 38 .4 °C era l p i t t ing edema .
( 10 1 . 1 °F) . blood pressure i s 140 90 m m Hg. pulse rate is Serum e lectrolyte levels a n d k i d ney fu nction tests
88 111 in . and respi ra t ion rate is 1 8 m i n . Oxygen saturat ion a re nor111 a l . Serum B- type n a t ri uretic peptide level is 2472
brea t h i ng ambient air is 89'X. . BMI is 25 . Chest exa m i nation is pg/ m l (2472 ng L) .
consistent with right lower lobe consol idation . ·n1e re111ainder E lectrocardiogram shows a t ri a l fibri l la tion . Echocar
of t h e physical exa111inalion is u nre111arkable. d iogram shows a l e ft ven t ricu l a r ejection rraction of 60'X..
An e l ect rocardiogra m ( ECG) at t h e t i 111e of hospi t a l septa ! wal l t h ickness ol' 1.5 c111. and posterior wall t h i c kness
ad111ission is shown . o f l .4 cm. Chest rad i ograp h shows hazy b i l a tera l i n fi l t ra tes.
Which of the following medications should be discontin Which of the following is the most appropriate next step
ued based on this patient's ECG findings? in management?
(A) A m i t ri p ty l i n e (A) �- B locker
(B) A m i t ri p ty l i n e and can·e d i l o l ( B) Ca rd ioversion
( C ) A m i t ri p ty l i ne and mox i f loxacin (C) Furosemide
(D ) fvl ox i f loxacin (D) Spironolactone
� I
V3
ITEM 1 4
1 24
Self-Assessment Test
Cl IAt58e myear
16 On physical examination, he is afebrile, blood pressure
- -ol d man is eva l ua ted in the hospital for weakness, is 112172 mm Hg, pulse rate is 62/min, and respiration rate
fat i gue. and hypotension . ·111e patient reports a 3-week h is is 12/min. Cardiac examination shows a normal S1 and S2
tory of anorexia and 2 -year h i s t o ry of non-s m a l l cell l ung without S3, S4, murmurs, or rubs. Lung examination is nor
cancer w i t h metastatic i nvolvement in t h e liver t reated w i t h mal. He has no lower extremity edema. The remainder of the
combination cytotoxic chemotherapy. examination is normal.
On physical exa m i n a t io n . t he patient appears cachec Diagnostic coronary angiography reveals a 90% steno
t ic. B lood pressure is 70/52 mm Hg and pu lse ra te is
sis in the proximal left anterior descending artery; the left
l l O/ m i n . The l un gs a re clear to a uscultation . l l1e j ugu lar circumflex artery has a diffuse 70% stenosis, and the right
venous pulse contour is ! l at . Caro t i d upstrokes a re brisk. S1 coronary artery has a 70% ostial stenosis. Left ventriculog
a n d S" are dista n t . No rubs. ga l lops. or murmurs a re presen t . raphy shows a left ventricular ejection fraction of 50% with
N o peripheral edema is noted . T h e rema inder o f t he physical mild anterior wall hypokinesis.
exa m ination is unremarka ble. Which of the following is the most appropriate management?
Echocardiogram shows a small Jen ven t ricular ca v
i ty w i t h hyperdynamic funct ion (ejection frac t i o n . 75%) . (A) Change metoprolol to amlodipine
A moderately sized c i rcumfere n t i a l pericard ia! e ffusion is (B) Coronary artery bypass graft surgery
evident. Diastolic i nversion or the rig h t a t ri u m and s ig n i f (C) Multivessel percutaneous coronary intervention
ica n t respiratory vari a t ion in the m i tra l i n f low pattern are (D) Myocardial viability nuclear perfusion scan
presen t .
ll1e blood pressure does n o t change a n e r a d m i n is t ra
tion o f a 1 - L bo l us of i n t ravenous f l uid . Item 1 9
In addition to continued intravenous fluid, which of the A 74-year-old woman is evaluated for a 3-week history of
following is the most appropriate next step in treatment? left shoulder pain and dyspnea on exertion. Medical history
is significant for COPD, hypertension, and coronary artery
(A) I ntra - a o rt ic b a lloon pump disease; she underwent stenting of the mid-left anterior
(B) Pericardiocentesis descending coronary artery 3 years ago. Because of her lung
(C) Phenylep hrine disease, she has limited exercise ability. Medications are
(D) Wi ndow pericarcliectomy lisinopril, hydrochlorothiazide, atorvastatin, aspirin, fluti
casone, albuterol, and ipratropium.
On physical examination, the patient is afebrile, blood
pressure is 142/88 mm Hg, pulse rate is 82/min, and respi
Item 1 7
ration rate is 18/min. BMI is 29. Estimated central venous
A 54-year-old man is evaluated during a routine appoint pressure is 8 cm H?O. Cardiac examination reveals a grade
ment. He has hypertension, dyslipidemia, obesity, and 2/6 midsystolic m1irmur heard best at the cardiac base and
erectile dysfunction. He currently drinks three alcoholic late expiratory wheezing bilaterally.
beverages daily. His mother had a nonfatal myocardial Electrocardiogram shows left ventricular hypertrophy
infarction (Ml) at age 55 years, and he is concerned about and repolarization abnormalities.
his risk of MI. Medications are hydrochlorothiazide, ator
vastatin, and sildenafil as needed. Which of the following is the most appropriate diagnostic
test to perform next?
Which of the following components of this patient's med
ical history is associated with the greatest risk for future (A) Adenosine single-photon emission CT myocardial
myocardial infarction? perfusion imaging
(B) Coronary catheterization
(A) Alcohol consumption
(C) Dobutamine stress echocardiogram
(B) Dyslipidemia
(D) Exercise stress echocardiogram
(C) Hypertension
(D) Obesity
Item 2 0
A 68-year-old man i s evaluated for a newly diagnosed car
Item 1 8 diac murmur. He is active and swims and jogs regularly.
A 63-year-old man is evaluated for follow-up of coronary Medical history is otherwise unremarkable, and he takes no
artery disease that was diagnosed by exercise stress testing medications.
3 weeks ago. For his exertional chest pain, he was started On physical examination, he is afebrile, blood pressure
on a �-blocker and nitrate in addition to his baseline med is 140170 mm Hg, pulse rate is 82/min, and respiration rate
icatio n s . He reports that his symptoms have improved, is 16/min. Cardiac examination reveals a late-peaking sys
although he remains limited in his activities because of tolic murmur located at the right upper sternal border with
exertional chest pain. Medical history is significant for an audible s?.
hypertension, type 2 diabetes mellitus, and hyperlipidemia. Transt horacic echocardiogram shows normal left
His current medications are aspirin, lisinopril, simvastatin, ventricular systolic function. Aortic valve area is 0.8 cm2 .
insulin, metoprolol, isosorbide mononitrate, and as-needed The mean gradient is 44 mm Hg, with a peak gradient of
sublingual nitroglycerin. 53 mm Hg.
1 25
Self-Assessment Test
Which of the following is the most appropriate management? 98/m i n. BM! is 25 . Cardiac examination shows a normal S 1
and S2 wit hout S r S � . murmur. or rnb. Lung examination is
(A) Balloon aortic valvuloplasty
normal. Serum troponin T level is 2 .0 ng/m L (2 . 0 µg/L) .
(B) Follow-up echocardiography i n 6 to 12 months Electrocardiogram is shown.
(C) Surgical aortic valve replacement T he patient is administered aspirin. clopidogrel. and
(D) Transcatheter aortic valve replacement unfractionated heparin. E mergency coronary angiography
shows normal corona1y anatomy. Diastolic ( left panel) and
systolic ( righ t pane/) i mages from left ven t riculography are
Cl A 66-year-old woman
Item 2 1
is eva lua ted a t t h e hospital for shown.
6 hours of chest pressure and shortness of breath . Earlier Which of the following is the most appropriate manage
t h is day . the patien t 's husband was diagnosed with lung ment?
cancer. Medical h istory is otherwise u n remarkable.
On physical exa m i n a t ion . temperature is 36 .8 °C (A) Endomyocardial biopsy
(98 . 2 °F) . blood pressure is 1 10 62 m m Hg. and pulse rate is (B) I n t ra-aortic balloon pump
ITEM 2 1
1 26
Self-Assessment Test
CJ (C) Metoprolol and captopril On physical exa mi nation. the pa tient is in significant
(D ) ll1rombolytic therapy pain . He is a febrile, blood pressure is 1 18 /70 mm Hg i n both
CONT. upper extrem it ies. pu lse rate is 122/ min, and respiration
rate is 22/min . The estimated cen tral venous pressure is
Item 2 2
10 cm H.,O. The lungs are clear. The heart examination is
A 56-year-old woman is evaluated during a n appointment notable for an early diastolic decrescendo murmur heard
to establish care. She has a developmental delay, and she is loudest at the right upper sternal border. The dorsalis pedis
known to have pulmonary hypertension due to a congenital and posterior tibialis pulses are palpable and equal bilaterally.
cardiac condition. There is no history of cardiac surgery. She Serum cardiac t ropon in T level is 0. 4 ng/m L (0.4 µg/L) .
is on low-dose aspirin and thyroid replacement therapy. Electrocardiogram shows sinus tachycardia but is otherwise
On physical examination, blood pressure is 110/70 normal. Chest radiograph is normal. Chest CT with i n trave
mm Hg, pulse rate is 68/min and regular, and respiration rate nous contrast is shown.
is 18/min. BMI is 32. The central venous pressure is elevated
with a prominent a wave. The apical impulse is normal. There
is a prominent parastemal impulse at the left sternal border.
The s, is normal; the s2 is loud. There is a grade 116 holosystolic
murmur at the left lower sternal border. The toes demonstrate
cyanosis and digital clubbing; her hands appear normal. The
remainder of the physical examination is unremarkable.
Cl AItem 23
56-year-old man with heart failure i s admitted t o the
hospital with a 2-week history of increasin g exertional dys
pnea and fat igue. He also has type 2 diabetes melli tus. Med
ications are metform i n . l isi nopri l . carvedilol. furosem ide,
metola zo ne and digoxin .
,
Hg. pulse rate is 95/min . and respiration rate is 20 /min . He is Which of the following is the most appropriate next step
somewhat con fused and i nattentive. Jugu lar venous disten in management?
tion is present to the angle of the jaw wh ile sitting. Cardiac
examination reveals an S3 . There are bibasi lar crackles on (A) Conti nu ed medical t herapy alone
pulmonary examination . He has edema to the midth ighs. ( B) E me rgen cy surgical interve n t ion
Extremities appear mottled and are cool to t he touch . (C) Endovascular stenting
Serum creat inine level is 3. 1 mg/dL (274 µmol / L) : base (D) H eparin
l i ne value was 1 . 1 mg/dL (97 . 2 µmol/ L) . Serum sodium level
is 133 mEq/ L (133 mmol/L) . Electrocardiogram shows no
evidence of ischemia. Chest radiograph shows cardiomeg Item 2 5
aly and vascula r congestion .
A 46 year-old man is eva l uated in the hospital prior
-
In addition to intravenous diuresis, which of the following undergoing an elective hernia repair. Medical history is sig
is the most appropriate management? n i ficant for a bicuspid aortic valve and a mechanical aortic
valve replacement 3 years ago for severe aortic stenosis. H is
(A) D obutam ine on ly medication is warfa ri n .
(B) Intra -aortic bal loon pump O n physical examination . blood pressure is 130 /75
(C) M i lrinone mm Hg. pulse rate is 82/m i n , and respira tion rate is 1 4 /m in .
(DJ Right hea rt catheterization Cardiac exa mination reveals a grade 1 /6 m idsystolic mur
mur at the righ t upper sternal border.
Cl AI t e46m-year-old
24
man i s eva luated i n the emergency depa1i
Which of the following is the most appropriate manage
ment option for endocarditis prophylaxis?
men t for severe pain in the chest and upper back t h a t began (A) A moxici l l i n
acutely abou t l hour ago. ll1e pain is described as "deep" and
(B) Ceftriaxone
constant. H e has no other associated sym ptoms. Medical
history is significant for hypertension. and his medica tions (C) Clindamycin
are chlorthalidone and va lsartan. (D) No antibiotic prophy laxis
1 27
Se lf-Assessment Test
Item 2 6 Item 2 8
A 48-year-old man is evaluated for tightness in his calves. A 37-year-old woman is evaluated for exertional dyspnea.
His symptoms are exacerbated with walking and resolve She noticed mild shortness of breath with significant exer
with rest. His medical history is significant for hyperten cise several years ago. Although she is still active, she has
sion, type 2 diabetes mellitus, and chronic kidney injury. had to progressively decrease the amount of exercise she
Medications are hydrochlorothiazide, lisinopril, met is able to do because of her symptoms. She has no other
formin, glyburide, and atorvastatin. He was a cigarette health problems, takes no medications, and has no known
smoker with a 30-pack-year tobacco use history but quit drug allergies.
6 months ago. On physical examination, she is afebrile, blood pres
On physical examination, blood pressure is 138/74 mm sure is 120/70 mm Hg, pulse rate is 67 /min, and respiration
Hg, pulse rate is 68/min and regular, and respiration rate is rate is 14/min. Cardiac examination demonstrates a grade
1 6/min. BMI is 32. No abdominal or femoral bruit is present. 3/6 crescendo-decrescendo systolic murmur located at the
No skin changes are noted in the lower extremities. The right upper sternal border with delayed carotid upstrokes.
remainder of the physical examination is unremarkable. Transthoracic echocardiography demonstrates normal
Laboratory studies are significant for a serum creat systolic function with a left ventricular ejection fraction of
inine level of 1.9 mg/dL (168 µmol!L) , normal electrolyte 60%, mild concentric left ventricular hypertrophy, and a
levels, and a hemoglobin A 1c value of 6.4%. bicuspid aortic valve. The aortic valve has a mean gradient
Ankle-brachial index testing: of 42 mm Hg and valve area of 0.9 cm2.
Right systolic brachia! pressure 140 mm Hg Which of the following is the most appropriate manage
Left systolic brachia! pressure 132 mm Hg ment?
Right posterior tibialis pressure 200 mm Hg
Left posterior tibialis pressure 130 mm Hg (A) Balloon aortic valvuloplasty
Right dorsalis pedis pressure Not detected (B) Start an ACE inhibitor
Left dorsalis pedis pressure 140 mm Hg (C) Surgical aortic valve replacement
Which of the following is the most appropriate diagnostic (D) Transcatheter aortic valve replacement
test to perform next?
1 28
Self-Assessment Test
.
l���V4
.
V6
�
II
ITEM 29
2/6 diastolic decrescendo murmur is heard at the apex. Which of the following is the most appropriate manage
No opening snap is appreciated. The lungs are clear, and ment of this patient's abdominal aortic aneurysm?
there is no edema.
(A) Refer for aneurysm repair
A transthoracic echocardiogram demonstrates normal
left ventricular size and function. The mitral valve is thick (B) Repeat abdominal ultrasonography in 6 months
ened with diastolic doming. The mitral valve mean gradient (C) Repeat abdominal ultrasonography in 12 months
is 12 mm Hg; the calculated mitral valve area is 0.9 cm2 . (D) Switch amlodipine to propranolol
There is no mitral valve regurgitation. The estimated pulmo
nary artery systolic pressure is SS mm Hg.
Item 3 2
Which of the following is the most appropriate manage
ment at this time? A 74-year-old man is evaluated 4 months after under
going uncomplicated bioprosthetic surgical aortic valve
(A) Initiate an ACE inhibitor and dabigatran replacement. Within the past 2 weeks, he has developed
(B) Obtain cardiac magnetic resonance imaging exertional dyspnea, fatigue, and lower extremity edema.
(C) Proceed with mitral valve intervention Medical history is otherwise unremarkable, and he takes
no medications.
(D) Proceed with pregnancy without interventions or
On physical examination, vital signs are normal. The
testing
estimated central venous pressure is 12 cm H2 0, and the
jugular venous pulse shows prominent y descents. A peri
cardia! knock is present. Peripheral edema is noted.
Item 3 1
An echocardiogram reveals no evidence of pericar
A S2-year-old woman is evaluated in the office during a dia! effusion. The aortic and mitral valves are functioning
routine visit. Her medical history is significant for type 2 normally. The inferior vena cava is markedly enlarged. A
diabetes mellitus and hypertension. Medications are aspi Doppler ultrasound shows expiratory flow reversals in the
rin, lisinopril, amlodipine, insulin glargine, insulin aspart, hepatic veins consistent with constrictive pericarditis.
and rosuvastatin.
On physical examination, the patient is afebrile, blood Which of the following is the most appropriate next step in
pressure is 128/80 mm Hg, pulse rate is 73/min, and respi management?
ration rate is 18/min. BM! is 24. The lungs are clear to aus
(A) Ibuprofen
cultation, and no cardiac murmurs are heard. Abdominal
examination reveals a pulsatile mass in the epigastrium. (B) Invasive cardiac hemodynamic evaluation
An infrarenal abdominal aortic aneurysm with maxi (C) Pericardiectomy
mum diameter of S.7 cm is noted on abdominal ultrasound. (0) Transesophageal echocardiography
1 29
Self-Assessment Test
Which of the following is the most appropriate manage (A) Aortic coarctation
ment? (B) Essential hypertension
(C) Hypertrophic cardiomyopathy
(A) Amiodarone
(D) Renovascular hypertension
(B) Cardiac resynchronization therapy
(C) Catheter ablation of premature ventricular contrac
tions
Item 3 6
(D) Implantable cardioverter-defibrillator
A 29-year-old woman who i s 10 weeks pregnant i s evalu
ated for hypertension; this is her first pregnancy. She has no
symptoms and no prior cardiovascular disease. She is taking
Item 34
no medications. She has a family history of hypertension,
A 66 -year-o l d woman is eva l ua ted prior to d isc h a rge . S h e and she does not recall when she last had her blood pressure
h a s ischemic cardiomyopa t hy a n d w a s a d m i t ted to t h e h os checked.
p i t a l 5 days ago for worsen i n g sym p toms of heart fa i l ure. On physical examination, blood pressure is 156/96 mm
S h e ski pped t a k i ng her d i u re t ics dur i n g a rece n t busi ness Hg and pulse rate is 80/min. BM! is 31. There is an apical S_1,
t ri p . Today. she f'eels \Ne l l a n d is able t o walk around t h e but no murmurs are detected. Pulses are normal through
ward twice w i t h o u t a n y sym p toms . out. The remainder of the examination is unremarkable.
'Jl1is was her first hospi t a l iza t i o n in 3 years. a l t hough Serum creatinine level, plasma glucose level, and
s h e has skipped her d i u re t ics d ur i n g ot her busi ness tri ps urinalysis all are normal. An ambulatory blood pres
d u ri n g t h is t i m e w i t h o u t a p pa re n t i l l e f fec t . S h e had a n sure monitor demonstrates an average blood pressure of
i m p l a n t a b l e card iove rt e r-defibri l l a t o r p l aced 3 years ago . 155/92 mm Hg.
An echocardiogram 1 m o n t h ago s h owed a l e f'l ven t r i c u l a r
ejection fract ion of' J S 'Y., (stable fo r t he p a s t 6 yc,: 11-s) . Medica Which of the following is the most appropriate treatment?
1 30
Self-Assessment Test
is asymptomatic. She is in her 24th week of pregnancy. An exercise electrocardiographic treadmill test is per
Medical history is unremarkable, and there is no family formed. The patient is able to exercise for 4 minutes to a
history of heart disease. She takes prenatal vitamins and no heart rate of 82% of the maximum predicted and energy
other medications. expenditure of 4 metabolic equivalents until the study is
On physical examination, she is afebrile, blood pres discontinued because of fatigue. Testing did not reproduce
sure is 120/70 mm Hg, pulse rate is 86/min, and respiration her symptoms, and there were no significant electrocardio
rate is 18/min. Cardiac examination reveals a midsystolic graphic changes with exercise.
ejection click followed by a grade 3/6 early peaking, cre
Which of the following is the most appropriate next step in
scendo-decrescendo murmur at the right upper sternal bor
management?
der. The murmur radiates toward the apex and decreases
slightly with the Valsalva maneuver. No diastolic murmur (A) Cardiac catheterization
is heard. (B) Pharmacologic stress testing
Which of the following is the most likely diagnosis? (C) Switch lisinopril to metoprolol
(A) Bicuspid aortic valve (D) Clinical observation
(B) Hypertrophic obstructive cardiomyopathy
(C) Mammary souffie
Item 4 0
(D) Mitra! valve prolapse
A 72-year-old woman i s evaluated fo r sharp chest pain that
(E) Physiologic murmur of pregnancy occurs randomly. She walks 3 to 4 miles daily, and her
symptoms have never occurred with exertion. She has never
smoked. Medical history is significant for hypertension, type
Item 3 8 2 diabetes mellitus, and hyperlipidemia. Medications are
A 64-year-old man i s evaluated for chest discomfort that low-dose aspirin, metformin, lisinopril, and simvastatin
he has had over the past year. It does not always occur (10 mg/d). She has no known drug allergies.
with exercise. There is no associated nausea or diaphoresis. On physical examination, blood pressure is 122/76
Medical history is significant for hypertension and hyperlip mm Hg, pulse rate is 76/min, and respiration rate is 12/min.
idemia. Medications are metoprolol, hydrochlorothiazide, Cardiac examination shows a normal S1 and S2 ; there is no
and lisinopril. S3 , S4, murmur, or rub. The remainder of the examination
On physical examination, vital signs are normal, as is is normal.
the remainder of the physical examination. Electrocardio Laboratory findings include a serum total cholesterol
gram is normal. level of 200 mg/dL (S.18 mmol/L), LDL cholesterol level
The patient is scheduled for exercise stress testing. of 126 mg/dL (3.26 mmol/L), and HDL cholesterol level of
SO mg/dL (1.30 mmol/L) .
Which of the following should be done prior to the stress
An exercise treadmill test is administered for 8 min
test?
utes, 40 seconds. There are no electrocardiogram changes
(A) Stop hydrochlorothiazide at rest or with exercise. She does not have chest pain during
(B) Stop lisinopril exercise or recovery.
(C) Stop metoprolol Which of the following is the most appropriate manage
(D) Stop all medications ment?
1 31
Self-Assessment Test
Item 43
c:J
The left lower extremity is warm and without tenderness
or skin changes. The remainder of the examination is unre A 68-yea r-old m a n is eval ua ted i n t h e e mergency depart
markable. ment l'or a 24 -hour history of persisten t chest pai n. H e had
Which of the following is the most appropriate addition to a non -ST-elevation myocardial i n fa rction 1 week ago t ha t
his current therapy? was managed medically w i t h complete symptom recovery.
Yesterday, he developed recurrent chest pain that dilTers
(A) �-Blocker from his previous a ngina pai n. TI1e pain is constant but
(B) Cilostazol exacerbated when lea n i ng for.Nard a n d not associated with
(C) Clopidogrel o ther symptoms. Medications are low-dose aspi rin. clopido
(D) Warfarin grel , metoprolol. and a torvastat in .
On physical exami nation. vital signs are normal. There
is no j ugular venous distention . TI1e lungs are clear to a us
cultat ion. S 1 and S 2 are norma l , and there is no S,1 or S,1 • A
H--0--/�f\r\��
� ���
���
ITEM 42
1 32
Self-Assessment Test
Cl shows normal sinus rhyt hm and heart rate of' 80/ m in . There
Seru 111 t ropon in level is elevated . Elect roca rel iogram rate is 16/min. Oxygen saturation breathing ambient air is 98%.
There is no jugular venous distention. Lungs are clear. Cardiac
CONT.
are nonspec i fic ST-T wave abnorma lities but no ST-segment examination reveals a regular rate and a grade 3/6 apical holo
eleva tion or depression. systolic murmur that radiates to the axilla. There is no lower
Cardiac ca lheterization is sign i f ican t for preserved left extremity edema.
ventricular systolic fu nction and two-vessel coronary artery Electrocardiogram shows normal sinus rhythm and
disease. Percuta neous coronary i n tervention of t h e micl left evidence of left atrial enlargement. Echocardiogram shows
an terior clescencling a rtery ancl p rox i m a l righ t coronary severe eccentric mitral regurgitation with marked calcifi
a rt e ry is performed w i t h p lacement of drug - eluting stents. cation of the valve leaflets; left ventricular systolic function
is normal.
In addition to continuing aspirin, which of the fo llowing
is the most appropriate management of this patient's anti Which of the following is the most appropriate treatment?
platelet regimen?
(A) Bioprosthetic mitral valve replacement
(A) Continue t icagrelor f'or 30 days (B) Mechanical mitral valve replacement
(B) Continue t icagrelor f'or l yea r (C) Oral vasodilator therapy
( C) Con t i nue t icagrelor inclefi n i lely (D) Percutaneous mitral valvuloplasty
(0) Stop ticagrelor. s tart clopiclogrel
Item 47
Item 45 A 7S-year-old woman i s evaluated for a 3-month history
A 42-year-old woman is evaluated for a routine outpatient of progressive exertional dyspnea and decreased exercise
medical assessment. She was diagnosed with a ventricular tolerance. She does not have chest pain. She has a history
septa! defect at age 6 months. Evaluation was performed of hypertension and COPD. She has a SS-pack-year tobacco
early in life and observation was recommended. She has no use history but quit 3 years ago. She has no history of alcohol
symptoms and is taking no medications. use. Medications are lisinopril, tiotropium, and as-needed
On physical examination, blood pressure is 100/60 albuterol.
mm Hg, pulse rate is 70/min and regular, and respiration On physical examination, blood pressure is 136/78 mm
rate is lS/min. BM! is 28. The estimated central venous Hg, pulse rate is 88/min, and respiration rate is 16/min. The
pressure is normal. The apical impulse is normal. There is central venous pressure is estimated at 9 cm H20. There are
no parasternal impulse. S1 and S2 are masked by a loud holo decreased breath sounds throughout both lung fields, but
systolic murmur noted at the left lower sternal border. The no crackles are detected. An S4 is heard on cardiac examina
rest of the examination is unremarkable. tion. There is trace bilateral lower extremity edema.
An electrocardiogram is normal. The heart size is nor Laboratory studies, including thyroid function studies,
mal on the chest radiograph. An echocardiogram demon are normal. Electrocardiogram is shown (see top of next
strates normal left ventricular size and function with an page) . A chest radiograph shows changes consistent with
ejection fraction of 60%. A membranous ventricular septa! COPD, mild vascular congestion, and blunting of the costo
defect is noted with a small left-to-right shunt. The right phrenic angles bilaterally. Echocardiogram shows a left ven
heart chambers and valve function are normal. The esti tricular ejection fraction of30% and an a kinetic anterior wall.
mated pulmonary artery pressure is normal. The patient is started on furosemide.
Which of the following is the most appropriate manage Which of the following is the most appropriate diagnostic
test to perform next?
ment?
1 33
Self-Assessment Test
.
n�� I - . . _ aVR _ • -
n� �11 - . . -- aVL
• - - ' ·-
n ·- :------
Ill
- .iv------�--"'l!--
.
- .-,11 aVF
-
�--:-- � �
_
_ f\ _ r (I- - · 1 -A- A
-rN . _ .. :-:-: � �A�
V6 . _
-
n_�0· __
(L,\/-�
:� \/ J_ L �
__
�·
- IJ0\/(L,·
-- J_ L(L,11���-
� ____
rt;,�, -t-
11 \L\� �-=-=--
__ -�
- � _� ___
ITEM 47
Medications are aspirin, lisinopril, simvastatin, metformin, Which of the following is the most appropriate manage
metoprolol, and long-acting nitroglycerin. ment?
On physical examination, the patient is afebrile, blood
(A) Mitra! valve repair
pressure is 132/72 mm Hg, pulse rate is 68/min, and respira
tion rate is 16/min. BM! is 28. The remainder of her physical (B) Repeat TIE in 6 months
examination is normal. (C) Start lisinopril
Electrocardiogram is unchanged from the time of her (D) Transesophageal echocardiography
stress test.
1 34
Self-Assessment Test
Item 5 2
examination is normal. His left ventricular systolic function
is normal. as measured on transthoracic echocardiography
A 74-year-old woman is evaluated during a routine exam on the day after hospital admission.
ination. Her medical history is significant for hypertension
and obesity. She is a former smoker, stopping 5 years ago. Which of the following is the most appropriate adjustment
Medications are amlodipine, lisinopril, and aspirin. to his discharge medications?
On physical examination, she is afebrile, blood pres (A) Add diltiazem
sure is 136/78 mm Hg, pulse rate is 68/min, and respiration
rate is 1 5/min. BMI is 32.
The lungs are clear to auscultation,
(B) Discontinue ticagrelor, start clopidogrel
and no murmurs are noted. A bruit is heard over the left (C) Increase dose of metoprolol
femoral artery. (D) Start eplerenone
The right ankle-brachia! index is 1.2
and the left is 0.81. (E) Make no changes to his medications
II
f t . I
l-
Ill
1 :�1
t
I
· 11 t ! ,_ '
ITEM 5 1
1 35
Self-Assessment Test
Item 5 4 Item 5 6
A 54-year-old m a n is evaluated after a recent diagnosis A 77-year-old man with a 5-year history o f idiopathic
of systolic heart fa ilure. He initially presented with a cardiomyopathy is evaluated for progressive exertional
4 - month history of exertional dyspnea. He has not had fatigue and dyspnea. He has recently stopped carrying
prior regular medical care and had no known medical groceries in from the car because of his exertional dys
problems. His blood pressure was 164/96 mm Hg at the pnea. He had an implantable cardioverter-defibrillator
time of diagnosis. Echocardiography showed evidence placed 3 years ago. Medical history is also significant
of hypertensive cardiomyopathy with no regional wall for hypertension. Medications are lisinopril, 40 mgld;
motion abnormalities and a left ventricular ejection metoprolol succinate, 25 mg/d; furosemide, 40 mg/d; and
fraction of 30%. Cardiac stress testing showed no evi spironolactone, 25 mg/d.
dence of ischemia, and he exercised for 7 minutes and On physical examination, blood pressure is 94/60 mm
1 0 seconds to a peak heart rate of 142/min. He was Hg and pulse rate is 70/min. Estimated central venous pres
started on lisinopril and is now able to walk 6 blocks sure is 5 cm H20. There is no edema.
before experiencing dyspnea. Serum electrolyte levels and kidney function are nor
On physical examination, blood pressure is 110/72 mal. Electrocardiogram shows normal sinus rhythm, a PR
mm Hg, pulse rate is 84/min, and respiration rate is 14/min. interval of 210 ms, QRS duration of 160 ms, and a new left
Estimated central venous pressure is 6 cm H p . The lungs bundle branch block. His left ventricular ejection fraction 3
are clear. Cardiac examination shows the point of maximal months ago was 25%.
impulse is shifted to the left anterior axillary line. There is no
Which of the following is the most appropriate next step in
lower extremity edema.
management?
Laboratory studies, including electrolytes and kidney
function, are normal. (A) Cardiac resynchronization therapy
Which ofthe following medications is the most appropriate (B) Dobutamine therapy
addition to this patient's treatment regimen? (C) Increase furosemide dose
(A) Amlodipine (D) Left ventricular assist device placement
(B) Carvedilol
(C) Furosemide
Item 5 7
(D) Spironolactone
(E) No added therapy A 53-year-old man is evaluated for a 6-week history of
epigastric and chest discomfort. The onset of the pain has
a variable relationship to stress and exercise and spicy
food. The discomfort is relieved at times with antacids and
Cl IAt e63m-year-o
55
l d m a n is hosp i t a l ized fo l l owing a rece n t
with rest. He has hypertension and is a former smoker
(quit 2 years ago) . Medications are lisinopril and hydro
i n ferior myoca rd i a l i n fa rc t i o n . Perc u t a n eous coro n a ry chlorothiazide.
i n t e rve n t i o n was n o t su ccessfu l . A n echocardiogram On physical examination, he is afebrile, blood pres
o b t a i n ed fo l l owi ng t h e a t t e m pted coro n a ry i n t erve n t io n sure is 140/92 mm Hg, pulse rate is 78/min, and respiration
demons t ra ted a left ven t ri c u l a r eject i o n fra c t i o n o f 55% rate is 12/min. BM! is 29. Funduscopic examination is nor
w i t h i n ferior w a l l a k i n es i s a n d a d i l a ted and dysfu n c mal. Results of the cardiac examination are normal, with
t i o n a l righ t vent ric le . On t h e t h i rd clay a ft e r a d m issi o n , no S3 or S4.
t h e pat i e n t develops p rog ress ive o xygen desa t ur a t i o n Electrocardiogram is shown (see top of next page) .
a n d clyspnea despi te o xygen t he ra py w h i le u prigh t t h a t
i m proves w h e n s u p i n e . Which of the following is the most appropriate diagnostic
On physica l exa m i n a tio n . h i s blood pressure is 90/70 test to perform next?
m m Hg. pu lse ra te is 86 / m i n a n d reg u l a r, and res p i ra (A) Adenosine myocardial perfusion study
t ion ra te is 25 / m i n . Est i m a ted cen t ra l venous pressure is
(B) Cardiac magnetic resonance (CMR) imaging
markedly eleva t ed . The apical i m p u lse is norm a l ; t here is
a para s t e rn a l i m pu l se a t the left s t e rn a l border. The hea rt (C) CT angiography
sounds are d i s t a n t . There is a soft h o l osysto l i c m u r m ur a t (D) Dobutamine stress echocardiography
t he left s t e rn a l border t ha t i ncreases w i t h i nspira t i o n . The (E) Exercise stress test
oxygen saturation is 90% on oxygen acl m i nisterecl by mask
w h i le t h e pat ien t is s i t t i n g a n d i m p roves to 94% on re t u rn
t o h i s bed . ·n1e rem a i n der 0 1· t h e physical e x a m i n a t i o n
is norm a I .
Item 5 8
A 58-year-old m a n is eva luated for a 2-wee k history o f
CJ
Which o f t h e following i s the most likely diagnosis? mala ise and subjective fever. Medical h istory is sign i ficant
(A) Patent fora men ova le with right - to-lert s h u n t for wel l -con t rol led type 2 d i a betes mel l i tus a n d sinus node
dysfunction . A d u a l -cha m ber pacema ker was i m p l a n ted
M i t ra! regurgitat ion
( 13)
5 years ago. He does not have clyspnea or weig h t loss . None
(C) Severe left ventricular systolic dysfunction of h is fa m i ly mem bers have had a recen t v i ra l or febri le
(D) Ven tricu lar septa I defect i l lness.
1 36
Self-Assessment Test
III VF
a V3 V6
nn---r-Tl��r-1--r
flLi��-J�J.�J��L��_j�Lr
ll
nJ � V5
ITEM 57
Which of the fo ll owing is the most appropria te manage A 26-year-old woman with a mechanical mitral valve
me nt ? prosthesis visits to discuss anticoagulation management
during pregnancy. Her last menstrual period was 6 weeks
(A) B l ood c u l tures ago and her pregnancy was confirmed by laboratory test
(B) Pacemaker pocket aspira t i o n ing in the office. Her mitral valve was replaced 5 years ago.
(C) U l t rasonography of t h e pacemaker pocket Her medications are low-dose aspirin, metoprolol, and
warfarin (4 mg/d) .
(D) Repeat eva l u a L io n in 1 week
On physical examination, vital signs are normal. Car
diac auscultation demonstrates a normal mechanical S 1 •
There are no murmurs or added sounds. Her INR is 2.6.
Item 5 9
A 47-year-old man is evaluated during a routine examina Which of the following anticoagulation regimens will pro
tion. He has no symptoms. Medical history is significant for vide the greatest protection against thromboembolism
a bicuspid aortic valve. He is not taking any medications. during her pregnancy?
On physical examination, he is afebrile, blood pressure (A) Continue warfarin and aspirin
is 130170 mm Hg, pulse rate is 56/min, and respiration rate
is 15/min. Cardiac examination reveals a grade 1/6 diastolic (B) Stop warfarin and start dabigatran
murmur at the left lower sternal border. (C) Stop warfarin and start subcutaneous fixed-dose
Echocardiogram shows a bicuspid aortic valve with unfractionated heparin
moderate aortic regurgitation, normal left ventricular sys (D) Stop warfarin and start weight-based low-molecular
tolic function, and normal left ventricular chamber size. weight heparin
1 37
Self-Assessment Test
A 56-year-old man is being evaluated after his 18-year-old (B) Low-dose dopamine
son had a syncopal episode during a high school basketball (C) Percutaneous coronary intervention
game and was diagnosed with hypertrophic cardiomyop- (D) Temporary pacing
aVR
l
i �
l
.v V2
l
I ,
I . '
--�---'''�' '
I j ; �
ITEM 63
1 38
363
Self-Assessment Test
Cl IAt e78-year-old
m 66
m a n is evaluated in t h e emergency depart
pressure is 12S/64 mm Hg, pulse rate is 64/min, and respi
ration rate is 16/min. BMI is 26. Estimated central venous
ment because of a painful righ t foot. He h as a 1 -year h istory pressure is normal. Cardiac examination shows a regular
1 39
Self-Assessment Test
ITEM 68
rate with normal heart sounds and no murmurs. H e has no (C) Evaluation for left ventricular assist device placement
peripheral edema. (D) Home inotropic therapy
Pertinent laboratory findings include a negative tro
ponin test, a normal metabolic profile, and normal kidney
function studies. Item 7 0
Electrocardiogram is shown. Echocardiogram demon
A 7S-year-old woman is evaluated during a follow-up visit
strates no structural heart disease and shows normal left
for recently diagnosed atrial fibrillation that is adequately
ventricular function.
rate controlled on medication. Medical history is significant
Which of the following is the most appropriate management? for hypertension and end-stage kidney disease; she is on
hemodialysis. Medications are metoprolol, digoxin, lisino
(A) Cardiac magnetic resonance (CMR) imaging pril, and amlodipine. She has not yet been started on stroke
(B) Exercise treadmill stress test prevention therapy.
(C) Implantable cardioverter-defibrillator placement On physical examination, temperature is 3 6 . 8 °C
(D) Tilt-table test (98. 2 ° F) , blood pressure is 120/6S mm Hg, pulse rate is
72/min, and respiration rate is 16/min. BM! is 29. The pre
cordial cadence is irregularly irregular. There is no evidence
of pulmonary or peripheral congestion.
Item 6 9
A 68-year-old man is evaluated a t a follow-up appointment. Which of the following is the most appropriate treat
He has a 7-year history of heart failure secondary to isch ment?
emic cardiomyopathy. Over the past 6 months, he has had
(A) Apixaban
three hospitalizations for exacerbations of his heart failure.
He currently has exertional dyspnea while getting dressed, (B) Aspirin and clopidogrel
and his maximal activity level is limited to riding to the store (C) Dabigatran
with his wife but staying in the car. Medical history is signif (D) Dose-adjusted warfarin
icant for disseminated prostate cancer treated with andro (E) Rivaroxaban
gen deprivation therapy. Medications are aspirin, lisinopril,
carvedilol, furosemide, digoxin, spironolactone, rosuvasta
Cl
tin, and leuprolide. He is stable on his current medications. Item 7 1
On physical examination, blood pressure is 92/60 mm
A 62-year-old woman i s eval uated i n the emergency depart
Hg and pulse rate is 80/min. There is no jugular venous dis men t for sudden onset of severe chest . u pper abdom ina l .
tention. An S3 is heard on cardiac examination. The legs are
a n d back pai n of 2 h ours' duration . S h e h a s not had s i m i l a r
cool to the touch; there is no edema.
sym ptorns previously a n d notes no o t h e r sym p toms. Med
Laboratory studies are significant for a serum sodium
ical history is sign i fica n t for hypertension. She is a curren t
level of 132 mEq/L (132 mmol!L) and serum creatinine level
smoker w i t h a SS-pack-year h istory . Her medications are
ofl.8 mg/dL (1S9 µmol/L) .
a rn lodipine and benazepri l .
Which of the following is the most appropriate manage O n p hysical exa rn i n a tion . s h e is afebri le. b lood pres
ment? sure is 1 6S 1 00 m m Hg i n both arms, pu lse rate is 1 02 1111 i n .
and respira t ion ra te is 20/ m i n . Oxygen saturation is 98% on
(A) Add metolazone ambient a i r. Cardiac auscul tation reveals a n S , gallop but
(B) Cardiac transplantation evaluation no m u rrnu rs. Pulmona ry exa m ination is norm a l . Pulses are
1 40
Self-Assessment Test
Cl sym metric a ncl equa I in a l l e x l remi t ics. ·1 he rem a i ncler or l he tral venous pressure is elevated with a prominent a wave.
p hysical examinat ion is unrema rkable. The apical impulse is normal. There is a prominent paraster
CONT.
Laboratory studies reveal a D-di mer level o f 0 .8 pgi m L nal impulse at the left sternal border. The sl is normal; the s2
(0 . 8 mg/ L) < 111cl a serum crea t i n i n e level o r 2.'l mgtcl L is soft. A grade 4/6 late-peaking systolic murmur is heard at
(2 1 2 �t mol/ L) (baseline is < l mg/d L [ 88.4 p mol / L]) . I n i t ia l the left sternal border and second left intercostal space. An
carcl i <:ic l roponin T level is 0. 4 ng/ m L (0 .4 �tg/ L) . ejection click is not audible.
Elec l rocarcliogram shows Jell ven t ricular hypert ro An echocardiogram demonstrates a dysplastic pulmo
phy wi t h repol a riza l ion abnorma l i t ies. Chest radiograph nary valve with a peak instantaneous systolic gradient of 62
demonstrates an e n l a rged cmcl iac sil houe l l e. J\ magnetic mm Hg and mean systolic gradient of 45 mm Hg. There is
resona nce a ngiography s tudy demonstra tes aort ic dissec moderate pulmonary valve regurgitation. The right ventric
t ion origin<:i l i ng distal to t he Jen subclavi:rn artery extending ular size and function are normal, but there is right ventricu
to t he aortoi l iac bifurcat ion (max i m u m d i a meter 63 m m ) . lar hypertrophy. The left heart size and function are normal.
Bila teral renal arteries arise from the fa lse lumen.
Which of the following is the most appropriate manage
Trea t ment wi t h ana lgesics. a �-blocker. and sodium
ment for this patient?
ni lroprusside is start ed .
(A) Endocarditis prophylaxis
Which of the following is the most appropriate next step
in managem en t? (B) Exercise testing
(C) Pulmonary valve replacement
(/\) Aort ic repa i r
(D) Observation
( B) Coronary angiogra phy
Cl
(C) Con t i nue current med ica l t herapy
(D) I n t ravenous heparin Item 7 3
A 62-year-old m a n i s evaluated in t h e emergency depart
ment (ED) f'or a 3- hour h istory or d u l l . substernal chest clis
Item 7 2 com f'orl. He has type 2 diabetes mel l i t us. hypertension . and
An 18-year-old woman with Noonan syndrome is evaluated clysl i pidemia. H e does not smoke cigare ttes. Medica t ions are
for a heart murmur noted on a sports physical examination. low-close aspi rin. l isinopri l . and pravasta ti n . H i s younger
She is asymptomatic and her medical history is unremark sister was diagnosed w i t h coronary artery disease at the age
able. She takes no medications. of 50 years.
On physical examination, blood pressure is 120/70 mm Electroca rdiogram obta ined upon h is a rrival lo t h e ED
Hg, pulse rate is 70/min and regular, and respiration rate is shown . He is a d m i n istered aspirin and sublingual n i t ro
is 18/min. BM! is 18. The patient is of short stature and has glycerin . H is chest disco m lorl is rel ieved w i t h i n 1 5 m i n utes
hypertelorism, neck webbing, and a low hairline. The cen- or arriva l .
ITEM 73
141
Self-Assessment Test
1 42
Se lf-Assessment Test
Which of the following is the most appropriate next step Which of the following is the most appropriate next step in
in the management of her doxorubicin chemotherapy? treatment?
Item 7 8 Item 80
A 60-year-old man is evaluated fo r increasing shortness A 65-year-old man i s evaluated fo r a routine examination.
of breath. He noticed progressive exertional intolerance He is asymptomatic and is active, walking 2 miles on a
1 month ago. His symptoms have worsened, and he is now treadmill three times a week. He has hypertension and
short of breath with walking mild inclines. He does not dyslipidemia. He has a 15-pack-year smoking history but
have chest pain, orthopnea, paroxysmal nocturnal dyspnea, has not smoked since age 30 years. Current medications are
cough, wheezing, or lower extremity edema. He has a his hydrochlorothiazide, atorvastatin, and aspirin.
tory of atrial fibrillation but remains in sinus rhythm after On physical examination, his blood pressure is 134/76
his second catheter ablation procedure for atrial fibrillation mm Hg in the right upper extremity and 146/80 mm Hg in
1 year ago. Medical history also includes hypertension and the left. His pulse rate is 72/min. BM! is 23. He has a grade
hyperlipidemia but is negative for heart failure or left ven 2/6 midsystolic murmur heard loudest at his left sternal
tricular dysfunction. Medications are warfarin, metoprolol, border. Abdominal examination reveals a pulsatile mass in
ramipril, and atorvastatin. the epigastrium.
On physical examination, the patient is afebrile, blood An abdominal ultrasound reveals an aneurysm with
pressure is 132/78 mm Hg, pulse rate is 70/min, and res a maximum diameter of 4.7 cm not involving the renal
piration rate is 18/min. Pulse oximetry demonstrates 98% arteries.
oxygen saturation on ambient air. BM! is 30. Cardiac rate
Which of the following is the most appropriate manage
and rhythm are regular. He has bilateral breath sounds but
ment of this patient's abdominal aortic aneurysm?
no wheezes, crackles, or rhonchi. There is no prolongation
of the expiratory phase. (A) Refer for aneurysm repair
The electrocardiogram shows normal sinus rhythm. (B) Repeat abdominal ultrasonography in 6 to 12 months
A plain chest radiograph is normal, and pulmonary func
(C) Repeat abdominal ultrasonography in 24 to 36
tion tests demonstrate no obstruction. An echocardiogram
months
demonstrates normal left ventricular function with a left
ventricular ejection fraction above 55% and evidence of mild (D) No follow-up management is needed
diastolic dysfunction.
CJ
Which of the following is the most likely cause of this Item 8 1
patient's dyspnea? A 54-year-old man is evaluated in the emergency depart
ment for an episode of crushing substernal chest pain and
(A) Chronic thromboembolic disease
discomfort that began 30 minutes ago. He is obese and
(B) Intracardiac shunting
currently smokes 1 to 2 packs of cigarettes daily. He has
(C) Phrenic nerve injury dyslipidemia. TI1e patient"s medications are enteric-coated
(D) Pulmonary vein stenosis low-dose aspirin and simvastatin .
On physical examination, he is afebrile, blood pres
sure is 146/88 mm Hg. pulse rate is 88/min and symmetric
Item 7 9 bilaterally, and respiration rate is 18/ min . B M ! is 32. Cardiac
A 41-year-old man comes t o the office t o discuss man examination reveals a normal S, and S 2 and no S 3 : there is
agement of hypertrophic cardiomyopathy (HCM), which an S_, . TI1ere are no murmurs or rubs. The remainder of the
was diagnosed 2 weeks ago after a murmur was detected examination is normal.
incidentally on examination for another medical condition. Serum troponin levels are e levated . Hematocrit is 42%
HCM has since been diagnosed in his father and brother and platelet count is 220.000/µL (220 x 1 09/ L) . Electrocar
during family screening. There is no family history of sud diogram shows changes consistent with an i nferior ST
den cardiac death. He is asymptomatic. elevation myoca rdial infarction. Portable chest radiograph
On physical examination, vital signs are normal. A soft shows a normal cardiac silhouette and no infiltrate.
holosystolic murmur is heard, which decreases during both TI1e patient is treated wiU1 enteric-coated aspirin, nitrates,
handgrip and stand-to-squat maneuvers. and a �-blocker. The hospital does not have capabilities to
1 43
Self-Assessm ent Test
Cl and t he nearest pri 111ary PC! center is 111ore than 2 hours away.
perfor111 pri111ary percutaneous coronary in tervention (PC! ) , Which of the following is the most appropriate manage
ment?
CONT. ll1e patient is ad111 inisterecl in travenous tenecteplase .
1 44
Se lf-Assessment Test
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ITEM 86
Medications are metformin, lisinopril, and hydrochloro Which of the following is the most appropriate diagnostic
thiazide. test to perform next?
On physical examination, the patient is afebrile, blood
(A) Diagnostic coronary angiography
pressure is 132/78 mm Hg, pulse rate is 78/min, and respi
ration rate is 14/min. BM! is 28. The remainder of the exam (B) Exercise electrocardiography
ination is unremarkable. (C) Exercise nuclear perfusion study
Electrocardiogram is shown. (D) Pharmacologic nuclear perfusion study
Which of the following is the most appropriate diagnostic
test to perform next?
Item 88
(A) Coronary artery calcium scoring A 48-year-old woman i s evaluated during a follow-up visit.
(B) Exercise nuclear perfusion imaging She has a 5-year history of type 2 diabetes mellitus. She has
(C) Exercise treadmill stress testing no other significant medical history. Medications are ator
vastatin, metformin, and a multivitamin. She works as a
(D) Vasodilator nuclear perfusion imaging
mail carrier and has a walking route that takes 3 hours each
day. She consumes a diet high in fruits and vegetables and
does not smoke.
Item 8 7 On physical examination, the patient is afebrile, blood
A 49-year-old woman i s evaluated for intermittent sharp, pressure is 128/80 mm Hg, pulse rate is 70/min, and respi
nonradiating, substernal chest pain for the past 2 weeks. ration rate is 12/min. BM! is 26. The remainder of the exam
The pain occurs more frequently in the morning and is ination is unremarkable.
not associated with meals or exertion but may be ini Laboratory studies are significant for a serum LDL cho
tiated with emotional stress. The pain does not include lesterol level of 135 mg/dL (3.50 mmol/L) and serum HDL
any pleuritic or positional components, and she states cholesterol level of 37 mg/dL (0.96 mmol/L). Urinalysis is
that there are no aggravating factors. The pain often negative for albuminuria.
lasts for 10 minutes and subsides spontaneously. She has Her estimated 10-year cardiovascular risk by the Pooled
hyperlipidemia treated with pravastatin. Her mother had Cohort Equations is 2.7%.
a myocardial infarction and heart failure starting at the
age of 5 2 years. Which of the following is the most appropriate cardiovas
cular disease risk management?
On physical examination, blood pressure is 132/82 mm
Hg and pulse rate is 78/min. BM! is 28. Lungs are clear to (A) Aspirin
auscultation. Cardiac examination shows a normal S1 and S2 ; (B) Coronary artery calcium scoring
there is no S3, S4 , murmurs, rubs, or gallops. She has no lower
(C) Exercise stress testing
extremity edema. The remainder of the examination is normal.
Electrocardiogram shows a heart rate of 80/min. The (D) Folic acid supplementation
QRS axis is normal, and there are no ST-T wave changes. (E) No further testing or therapy
1 45
Self-Assessment Test
Item 9 1
Cl A 57 -yea r-o ld woman is evaluated i n t he hos p i t a l for
Item 8 9
A 64-year-old woman is evaluated for a 3-month history
c h ro n ic sys t o l i c heart fa i l ure. She was ad m i t ted w i t h of sharp chest discomfort that she experiences during gar
progressive clyspnea of 2 weeks' d ur a t ion . A fter 3 clays dening. Medical history is significant for hypertension and
o f aggressive d i ur e t i c t herapy w i t h weight loss o f 5 kg hyperlipidemia. The patient's father had a coronary artery
(11 l b) . she rem a i ned very dyspneic. and right heart ca t h bypass graft at the age of 68 years. Medications are losar
e teriza t ion w a s performed . Medica t ions a re l isinopri l , tan, hydrochlorothiazide, and atorvastatin, and she recently
digox i n . spironolactone. a n d i n t e rm i t tent furosemide started taking low-dose aspirin daily.
i n t ravenously. On physical examination, the patient is afebrile, blood
On physical examina tion . b lood pressure is 9 6/74 m m pressure is 136/84 mm Hg, pulse rate is 78/min, and respira
Hg. pulse rate i s 1 1 8/ min . a n d respiration rate i s 20/m i n . TI1e tion rate is 16/min. BM! is 26. The remainder of the physical
i n ternal jugular vein is not visible when the patient is in an examination is unremarkable.
upright posi tion . Lungs a re clear. An S.i is heard on cardiac Baseline electrocardiogram shows left ventricular
examination. There is b i lateral edema to the knees. Her hypertrophy with ST-segment depressions less than
serum creatinine level is 1 .7 m g/ell ( 1 5 0.3 pmol / L) . 0.5 mm in the lateral leads. During exercise stress test
Hemodynamic measurements: ing, the patient develops 1-mm ST-segment depressions
Right atrium pressure 4 mm Hg in leads II, III, and aVF. She exercised 5 minutes and
Pul monary capi llary 16 mm Hg 30 seconds of a Bruce protocol; her peak heart rate was
wedge pressure 129/min (85% predicted maximum) , and blood pressure
Cardiac output 3.1 U m in (normal, was 186/76 mm Hg.
4. 0- 8. 0 U m i n ) Which of the following is the most appropriate next step in
Cardiac i ndex 1 .8 U m i n/ m1 the management of this patient?
Systemic vascular 2050 dyne/s/cm2 (normal,
resistance 800- 1200 dynels/cm") (A) Add a �-blocker
(B) Cardiac catheterization
Which of the following is the most appropriate change in
(C) Cardiac magnetic resonance (CMR) imaging
this patient's thera py?
(D) Stress echocardiography
(A) Con t i nuous i n t ravenous furosem icle
(B) Dopamine i n fusion
Cl
(C) Esmolol drip Item 9 2
( D) N i t roprusside A 66 -year-old man i s evalua ted i n the emergency depart
ment for 45 m i n u tes ofsubsternal chest pain that radiates to
the left shoulder. The patient's medical histo ry is signi Acant
Item 9 0 for hy pert e nsi o n . type 2 diabetes mellitus. and hy pe rli pid
A 47-year-old man i s evaluated fo r a 3-month history of em ia . He has never had abnormal bleeding. Med icat ions are
fatigue, abdominal fullness, and lower extremity edema. low-close aspiri n . gli m e p i ride. l isinopr i l . and simvasta t i n .
Ten years ago, the patient had acute pericarditis with car He h a s no known drug al lergies.
diac tamponade; the tamponade was treated successfully On physical exami na tion. bl o od pressure is 174/ 92 m m
with pericardiocentesis, and the pericarditis resolved fol Hg a n d p ulse rate is 82/m i n. Cardiac examination shows a
lowing a course ofan anti-inflammatory medication. He has normal S1 and S2; there is no S,l' S4, m u rmur, or rubs. The
no history of significant alcohol consumption, hepatitis, or remainder of the physical exam ination is norm a l .
autoimmune disease, and takes no medications. Hemoglobin concent ra t ion is 13.4 g/d L ( 1 34 g/L) and
On physical examination, the patient is icteric. Vital serum crea t i n i ne level is 1.0 m g/d L (88.4 pmol 1 L) . Results
signs are normal; BM! is 30. The estimated central venous of serum troponin levels are pending. Electrocardiogram is
pressure is 12 cm Hp, and the jugular venous pulse shows shown (see top of next page) .
a prominent y descent. S1 and S2 are normal, and no mur Tiie pa t ie n t is given aspiri n . clopidogrel. u n fractionatecl
murs, rubs, or gallops are heard. There is dullness to percus hepari n , and a �-blocker. Transport to the nearest hos p i
sion at the right lung base. The remainder of the pulmonary tal w i t h primary percutaneous coronary intervention (PC!)
examination is normal. Both ascites and lower extremity capabi lities would take approximately 1 35 minutes.
edema are present.
Which of the following is the most appropriate manage
Transthoracic echocardiography is technically chal
ment?
lenging, and limited information is obtained. Fluid obtained
from abdominal paracentesis is transudative. (A) Administer tenecteplase and t ra ns fer to a PCI-capable
center
Which of the following is the most appropriate next step in
management? (B) A d m i t to the hospital and awa i t ca rdiac biomarker
results
(A) Hemodynamic cardiac catheterization (C) I n i tiate abciximab and transfer for u rgent coronary
(B) Liver biopsy angiography
(C) Measurement of B-type natriuretic peptide (D) Transfe r for primary percutaneous coronary i n ter
(D) Vigorous diuresis v e n t i on
1 46
Self-Assessment Test
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ITEM 92
1 47
Self-Assessment Test
(A) Aspirin
(B) Patent foramen ovale device closure
(C) Warfarin
Item 99
A 56-year-old man is eval uated i n t h e hosp i t a l for a 2-week
Cl
(D) No therapy history of' fevers and mala ise. M ed ical h ist ory is sign incant
for a bicuspid aortic valve . The patient t a kes no medica t ions.
On p hysica l exa m i na t ion . tem pera ture is 38 . 5 °C
Item 97 ( l O l . 3 ° F) . blood pressure is 1 4 0 1 50 mm Hg. p u l se ra te is
A 46-year-old man is evaluated i n follow-up for a bicus 9 8/ m i n . a n d res p i ra t ion rate is 16 m i n . ·n1ere is n o j ugu lar
pid aortic valve. He exercises regularly without any activi venous distent ion . ll1e l u n gs are clear. Cardiac exam i na
ty-limiting symptoms and feels well. His medical history is t ion revea ls a grade I 6 diastolic m u rmur. There a rc no
otherwise negative and he takes no medications. s igns of' periphera l embolic d isease . No lower e x t re m i t y
On physical examination, blood pressure is 138/85 mm edema is presen t .
Hg. BMI is 28. A systolic ejection click followed by a crescendo Electrocardiogram shows normal sinus rhy t h m . a P R
decrescendo murmur are noted at the left sternal border. interval of' 230 ms. and nonspecific T-wave changes. Except
No diastolic murmur is appreciated. 1he lower extremity for t he increased PR i n terval . t here a re no cha nges com
pulses are normal. The remainder of the examination is pared w i t h a prior t racing. A t ransthoracic echocardiogram
unremarkable. shows a 6 -m m vegetation on the aort ic valve wi t h m i l d to
Transthoracic echocardiogram shows a bicuspid aortic moderate aort ic regurgi ta t ion . A t ransesophageal echoca r
valve with systolic doming of the aortic valve and a valve cliogram con fi rm s t he va lve f i n d i ngs and suggests t he pres
area of 1.7 cm2• l11e mean gradient across the aortic valve ence o l ' a n area of' r l uicl around t h e aortic a n n u l us posterior
is 22 mm Hg. The ascending aorta is dilated at 4.5 cm; the to t he yegetation . i ndicat ive of a n aortic root abscess.
descending thoracic aorta is incompletely visualized. Chest Blood cul tures are posit i ve for Staphy lococcus a u reus
CT demonstrates a 4.6-cm aneurysm of the ascending aorta sensit ive to met h ici l l i n . Appropria te a n t ibiot ics are st a rt ed .
with no evidence of coarctation and no enlargement of the
Which of the following is the most ap prop ria te treatment?
descending aorta .
(A) A n t ibiotic t h erapy f'or 6 weeks and t hen reassess
Which of the following is the most appropriate next step in
management? ( B) A n t i b iotic t herapy for 3 mont hs and t hen reassess
(C) Aortic v;:i lve replacement a fter 6 w eeks of' a n t ibiotic
(A) Aortic valve replacemen t t h erapy
(B) Aortic valve replacement and ascending aortic repair
(D ) U rgent aort ic valve replacement
(C) Ascending aortic repair
(D) Repeat echocardiogram in 1 year
Item 1 0 0
A 26-yea r-o ld \Noman is evaluated i n t he emergency depart
CJ
Item 98 ment for palpitations and pounding in her neck. She of'ten
A 42-year-old woman i s evaluated for episodes of palpi gets t hese episodes and t hey typica l l y last sewral m i n u tes:
tations that last several seconds in duration. They occur however. t h i s episode has been going on for 30 m i n u tes.
once or twice a month and are accompanied by light She can usual ly stop t h e episodes by beari ng clown . but on
headedness and mild dyspnea. She has not experienced t h i s occasion t h i s has not· worked . She reports f'eel i ng a l i t t le
1 48
Self-Assessment Test
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ITEM 1 00
Cl s hort of brea t h , but she docs not have chest pain or loss o f' to t h e hospi t a l . he began receiving metoprolol, clopidogre l .
consciousness. She has no other sign i fie a n t med ica I history. and i n t ravenous hepari n .
CONT.
and her only medicat ion is an oral contracept ive. V i t a l signs are norm a l . and h is physical exa m i n a t ion is
On physica l exam i na t i o n , t h e p a t i e n t is af'e bri le. b l ood u nremarkable.
pressure is 1 20 /80 mm H g. pu lse ra te is 1 45 / m i n . a n d
In addition to continuing aspirin indefinitely, how long
respi r a t i o n r a t e is 1 8/ m i n . B M I is 25. Card iac e x a m i n a t i o n
should this patient's clopidogrel therapy be continued?
shows tachyca rdia b u t regu l a r r hy t h m . Lungs a re c l e a r t o
a uscu l ta ti o n . (/\) 2 weeks
The elec trocardiogram is shown. (B) I month
Which of the following is the most appropriate treatment? (C) I yea r
( D) Li f'c long
( A ) Adenosine
( 13) /\ m iodarone
(C) Cardioversion
(D) l b u t i l ide
Item 1 0 2
A 68-yea r-old man is eva luated for progressive shortness of
CJ
brea t h . He underwen t heart t ra nsplantation 10 years ago for
1 49
Se lf-Assess ment Test
IT'I TI1ere is no jugular venous disten t i o n. ·n1e l u ngs are clear. Which of the following is the most appropriate preopera
LI.I a n d t h e heart e x a m i n a t ion is unremarkable . T he rem a i nder tive device management?
CONT.
or the exam i n a ti on is norm a l . (A) Disable shock i ng function
Elect rocardiogram demons t ra tes s i n us tachycard i a.
( B) Proceed with surgery a n d i n terrogate t he device post
right bundle branch block. and no Q waves. Echocard io
opera tively
gram shows a left ven tricular eject ion fraction of 5 5 % . evi
dence or m i l d d iastolic dysfu nct ion . septa I wa l l t h ickness (C) Reprogram to async h ronous pacing and disable
of 0.9 cm . posterior wall t h ickness or 1 . 0 cm . and moderate shoc k i ng function
t ricuspid regu rgi t a ti on. (D) Advise aga i n s t surgery
Which of the following is the most appropriate diagnostic
test to perform next?
Item 1 0 4
(A) oro n a ry angiography A 38-year-old man is evaluated for gradualJy progressive
( B) Endomyocardial biopsy exertional dyspnea. He had one episode of atrial fibrillation
(C) Pulmonary function test 1 year ago but converted spontaneously in the emergency
department. No additional testing was performed at that
( D) Ven t i la t ion -perfusion lung scan
time, and no medical therapy was initiated. He is otherwise
healthy and has been active. His medical history is other
wise unremarkable. He takes no medications and has no
I i ll r
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ITEM 1 04
1 50
Self-Assessment Test
Cl An
Item 1 05
82-year-old man was admWed to the coronary care unit
(C) Add warfarin
(D) Cardiac resynchronization therapy
(CCU) 48 hours ago after a late presentation with anterior
ST-elevation myocardial infarction. -n1e patient underwent
. 11. 1 fect1ve
. endocard1t1s
. . CJ
coronary angiography and was found to have an occluded Item 1 0 7
proxi.rnaJ left anterior descending coronary artery but did not .
A 45-year-old man be111g treated for
undergo an attempt at revasculari.zation at the time of coro is seen for a follow-up examination. He was diagnosed with
nary angiography because of his late presentation and symp endocarditis 1 week ago after presenting with fatigue and
tomatic improvement. Today. he felt faint and lost conscious fever. Initial transthoracic echocardiogram showed a bicus-
ness while visiting with his family in the CCU. Medications pid aortic valve with a small vegetation but was otherwise
are aspirin. ticagrelor, metoprolol. lisinopril, and atorvastatin. normal. Blood cultures were positive for methicillin-sensitive
On physical examination. blood pressure is 72/54 mm Staphy lococcus a u reus, and intravenous nafcillin was ini
Hg and pulse rate is 108/min. Cardiac examination shows tiated. Blood cultures obtained 48 hours and 72 hours after
tachycardia with a normal S1 and S,. new holosystolic mur starting antibiotic therapy showed no growth.
mur heard best at the left lower sternal border that radiates On physical examination, temperature is 37.8 °C
to the apex, and a right ventricular heave. Crackles are (100.0 °F), blood pressure is 128/78 mm Hg, pulse rate is
heard at the bases of both lungs, one third of the way up. 88/min. and respiration rate is 16/min. BM! is 25. Physical
He has no lower extremity edema. ll1e remainder of the examination reveals no cutaneous or ocular stigmata or
examination is normal. bacterial endocarditis. Cardiac examination reveals a grade
Electrocardiogram shows persistent ST-segment eleva 2/6 early systolic murmur al the base of"the heart, unchanged
tion and Q waves in leads V1 through V,. Heart rate is UO/min. Crom previous examinations. -n1e remainder of the physical
Emergency transthoracic echocardiogram shows a lef't ven examination is normal.
tricular ejection fraction of 35'Yu with severe anterior-apical Electrocardiogram is unchanged from the time of diag
akinesis, a small pericardiaJ effusion, and a color flow jet nosis except for an increase in the PR interval from 120 to
across the ventricular septum. suggestive of"left-to-right flow. 210 ms.
Which of the following is the most appropriate manage Which of the following is the most appropriate next step
ment? in management?
Item 1 06 Item 1 08
A 72-year-old woman is evaluated for progressive heart A 58-year-old man is evaluated for a 3-month history of left
failure symptoms. She has a 10-year history of nonischemic upper extremity symptoms and dizziness. He is left-handed
heart failure. She currently experiences exertional dyspnea and works as a carpenter. He describes an aching sensation
with climbing one flight of stairs, which she was able to do and feeling of fatigue in his arm and occasional dizziness
without sho1iness of breath 3 months ago. Medical history that occur within 2 to 3 minutes of using a hand saw; these
is significant for hype1iension, and her medications are symptoms resolve several minutes after stopping activity. He
lisinopril, carvedilol, furosemide, digoxin, and spironolac is otherwise asymptomatic. Medical history is significant for
tone. The patient is black. hypertension, hyperlipidernia, and type 2 diabetes mellitus.
On physical examination, blood pressure is 134/72 mm He has a 40-pack-year smoking history but quit 1 year ago.
Hg and pulse rate is 66/min. BM! is 35. She has no jugular Medications are lisinopril, atorvastatin, and metformin.
venous distention. Cardiac examination reveals a grade 116 On physical examination, he is afebrile, left arm blood
holosystolic murmur but is otherwise normal. The1:e is . no pressure is 135/76 mm Hg, pulse rate is 68/min, and respi
lower extremity edema. The remainder of her exam111at10n ration rate is 16/min. BM! is 29. ·nie carotid upstrokes are
is unremarkable. normal. The chest is clear and the cardiac examination is
Laboratory studies are significant for normal elec normal. Examination of the left upper extremity is unre
trolyte levels and a serum creatinine level of 1.5 mg/dL markable, with palpable distal pulses and no evidence of
(133 µmol/L). distal ulceration or skin breakdown. 'llie remainder of his
Electrocardiogram shows normal sinus rhythm, a QRS physical and neurologic examination is unremarkable.
duration of 110 ms, and nonspecific ST-T wave changes. Which of the following elements of the physical examina
Echocardiogram shows a left ventricular ejection fraction tion would be most helpful in establishing the diagnosis?
of 38% and trace mitral regurgitation. (A) Ankle-brachia! index
Which of the following is the most appropriate treatment?
(B) Bilateral blood pressure measurement
(A) Add hydralazine and isosorbide di.nitrate ( C) Evaluation for pulsus paradoxus
(B) Add losartan (D) 111oracic outlet maneuvers
1 51
Self-Assessment Test
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ITEM 1 09
(A) Adenosine nuclear stress test A 52-year-olcl m a n is eval ua ted in t h e hosp i t a l for pro
(B) Dual-chamber pacemaker gressive chest pressure over t he past 3 wee ks. He has a
35-pack-year history of cigarette smoking. M ed ica l h i s tory
(C) Single-chamber pacemaker
is sign i f i c:rn t for h y p e rt ensi on a n d hy per li p i clem i a t rea ted
(D) No intervention is indicated w i t h aspi rin, hyd roc h l o ro t h iaz i d e . l is in op ri l a n d pravasta
.
tion 4 years ago and has hypertension and dyslipidemia. She gra m s hows 2 - m m ST-segment d epress i on i n l ea ds I. a\IL.
is a former smoker and consumes one alcoholic beverage a ncl \I 1 th rough v( ,.
daily. She has no limitations with physical activity and He is a d m i t ted to the coronary care u n i t a nd gi ve n a sp i
is able to exercise periodically. Medications are low-dose r i n . metopro l o l . n i t roglycerin paste. a n d e n o x a p a r i n . Over
aspirin, hydrochlorothiazide, metoprolol, and high-dose t he course of' the first 12 hours. h is chest pressure worsens.
atorvastatin. requ i ring i n t ravenous n i t roglycerin in fusion . Subsequently.
1 52
Self-Assessment Test
1 53
Self-Assessment Test
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ITEM 1 1 5
Electrocardiogram is shown. Transthoracic echocar hypertrophy. Echocardiogram shows a left ven tricular ejec
diogram shows no structural abnormalities. t ion fraction or 1 0% and no valvular regurgi tation.
Which of the following is the most appropriate next step in Which of t h e following i s t h e most appropriate di ag n ostic
management? test to perform next?
1 54
Self-Assessment Test
How frequently should this patient undergo surveillance est i m a ted cen t ra l venous pressure is eleva ted . The apical
imaging? i m pulse is di ffuse. T he S 1 and S 2 are soft . A n S 1 and S , a re
(A) Every 6 months prese n t . A soft holosystolic murmur is heard a t t he apex.
Crackles are heard over bot h l u ng fields. P i t t i ng edema is
(B) Every 12 months
noted to t he knees.
(C) Every 24 months An elect rocardiogram is shown. An echocardiogram
(D) Every 3 to 5 years reveals a global red uction in con trac t i l i ty and left ven t ricu
lar enlargement wit hout hypertrophy.
A 59-year-old man is evaluated for a 3-month history of (A) Acu te pulmonary embolism
intermittent exertional chest discomfort. He has hyperten (B) lschemic ca rd iomyopa t hy
sion treated with lisinopril and amlodipine. (CJ Peri part um cardiornyopat hy
On physical examination, the patient is afebrile, blood
( D ) S t ress-induced cardiomyopa t hy
pressure is 138/92 mm Hg, pulse rate is 82/min, and respi
ration rate is 14/min. BM! is 27. The remainder of the exam
ination is unremarkable. Item 1 2 0
Exercise electrocardiographic stress testing shows
A 37-year-old man is evaluated for a 6-month history of
1.5-mm ST-segment depressions in leads II, III, and a VF; in
exercise intolerance and shortness of breath when walking
addition, the patient developed chest pressure during this
up stairs. He has no significant medical history and takes no
test. He exercised 4 minutes and stopped because of chest
medications.
discomfort. Heart rate and blood pressure increased appro
On physical examination, blood pressure is 140/70 mm
priately. Duke treadmill score is - 11.5.
Hg, pulse rate is 62/ min, and respiration rate is 16/min. Car
Which o f the following i s t h e most appropriate next step in diac examination reveals an irregularly irregular rhythm.
management? An opening snap is heard after S 2 , followed by a grade 1/6
diastolic rumble at the apex.
(A) Begin aspirin, �-blocker, and statin and re-evaluate in
Electrocardiogram shows atrial fibrillation. Transtho
2 weeks
racic echocardiographic findings are consistent with rheu
(B) Cardiac catheterization matic valve disease, showing a mildly thickened mitral
(C) Dobutamine stress echocardiography valve with minimal calcification and mild restriction in
(D) Exercise myocardial perfusion imaging leaflet motion. ·n1e subchordal apparatus is mildly thick
ened, and there is mild mitral regurgitation and marked left
atrial enlargement. Mean gradient across the rnitral valve
CJ I t e m 1 1 9 is 13 mm Hg. Mitra! valve area is 1.2 cm2. Transesophageal
echocardiogram shows no left atrial appendage thrombus
A 26-year-old woman is evaluated i n t h e emergency depart and confirms transthoracic echocardiographic findings.
ment for progressive dyspnea . She is 2 weeks postpa rtum .
T he pregna ncy was com p l icated by preec l a m ps i a but In addition to anticoagulation therapy, which of the follow
resul ted in a norm a l del ivery. The i n fa n t is hea l t hy. She has ing is the most appropriate management?
no h istory of cardiovascular d isease. Her o n ly medicat ion is (A) Medical management; repeat echocardiogram in
prena t a l vitamins. 6 months
On physical exa m i n a t i o n . t h e patient is alebri le.
(B) Mitra! valve replacement
Blood pressure is 100/70 mm Hg i n bot h arms. pu lse rate is
! OS/ m i n and regular. and respi ra t ion rate is 25/ m i n . BMI (C) Percutaneous mitral balloon valvuloplasty
is 29. T h e oxygen satura tion on ambient a i r is 96%. ·n1e (D) Surgical mitral valve repair
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1 55
Answers and Critiques
Item 2 Answer: D
Bibliography
Educational Objective: Use high-sensitivity C-reactive
Ridker PM, Danielson E, Fonseca FA, el al: JUPITER Study Group. Rosuvastatin
protein level to guide treabnent and cardiac risk stratification to prevent vascular events in men and women with elevated (-reactive
in a patient at intermediate risk of cardiovascular disease. protein. N Engl J Med. 2008 Nov 20:359(21):2195-207. [PMID: 18997196]
1 57
Answers a n d C ritiq u e s
1 58
Answers a n d Critiques
flow tract obstruction and, therefore, should be a non-ST-eleva t ion myocardial in farction can not be excluded
based on t he electrocardiogram, it is less l i kely given his nor
avoided in patients with hypertrophic cariliomyopathy.
mal tropon i n I level : the t ropon i n level wou l d l i kely be elevated
arter 3 hours of ongoing cardiac ischemfa. TI1e patient also has
Bibliography
a low pretest probabi l i ty or pul monary embolism by Wel l s
Gersh BJ. Maron, BJ. Bonow. RO, et al: American College of Cardiology
Foundation/American Heart Association Task Force on Practice Guidelines. scoring. Given h i s c l i n ical a n d rad iograph ic presentation. nei
20ll ACCF/ AHA Guideline for the Diagnosis and Treatment of Hypertrophic t her al Lerna l ive d iagnosis to an acute aortic syndrome is l ikely,
Cardiomyopathy: a report of the American CoUege ofCardiology Foundation/
American Heart Association Task Force on Practice Guidelines. Developed a nd a n ticoagulation is not indicated in acute aortic syndrome.
in collaboration with the American Association for Thoracic Surgery, Mal perfusion syndromes a re uncommon w i t h an aor
American Society of Echocardiography, American Society of Nuclear
Cardiology, Heart Failure Society of America, Heart R hythm Society. Society tic penet rating a t herosclerotic u l cer but cou l d occur w i t h
for Cardiovascular Angiography and Interventions. and Society of Thoracic transformation to classic cl issecl ion. In such cases, emergency
Surgeons. J Am CoU Cardiol. 20U Dec l3;58(25):e212-60. [PMID: 22075469]
enclovascu lar ste n t i ng with or wit hout fenestration may be
necessary. This patient shows no evidence of l ower l i m b or
Cl Educational Objective:
Item 6 Answer: c
Treat a penetrating atheroscle
viscernl mal perfusion . and cat heterizal ion with endovascular
repa i r is not ind icated. Emergency open surge1y is rarely indi
rotic ulcer in the descending aorta. cated i n t h is setting w i t h t he advent ofendovascular therapies.
1 59
Answers a n d Critiques
Item 7 Answer: B that statin therapy be initiated in patients at high risk for
Educational Objective: Diagnose effusive constrictive
CHO. The intensity of the statin therapy should be tailored
to the CHO risk. Candidates for high-intensity statin therapy
pericarditis.
include:
The most likely diagnosis in this patient is effusive constric
tive pericarditis. Effusive constrictive pericarditis is a clini • Patients with known atherosclerotic disease (clinical CHO,
cal entity in which patients who have a pericardia! effusion, cerebrovascular disease, or petipheral artetial disease)
with or without cardiac tamponade, experience persistent • Patients with an LDL cholesterol level 190 mg/dL
symptoms and hemodynamic derangements after treat (4. 92 mmol/L) or greater
ment and relief of the pericardia! effusion. In some patients
• Patients with diabetes mellitus, an LDL cholesterol level
with constrictive pericarditis, pericardia! inflammation
below 190 mg/dL (4.92 mmol/L) , and calculated
results in an effusion, which is placed under pressure by
10 -year CHO risk of7.5% or higher
the inelastic pericardium. Symptoms of low cardiac output,
systemic congestion, and an elevated jugular venous pulse • Some patients without diabetes with an LDL cholesterol
are seen, as in constrictive pericarditis; however, in patients level below 190 mg/dL (4. 92 mmol/L) and calculated
with effusive constrictive pericarditis, a pericardia! knock 10-year CHO risk of7.5% or higher
is absent and the y descent of the jugular venous pulse may
Moderate-intensity statin therapy can be considered
be less prominent. Additionally, pulsus paradoxus may be
for:
present, which is not a typical finding of constrictive peri
carditis. T11is disorder is caused by pericarditis involving • Patients with diabetes who are not receiving high
the visceral layer of the pericardium. Thickening of the intensity therapy
visceral layer of the pericardium can be difficult to detect • Most patients without diabetes with an LDL cholesterol
with CT and other noninvasive imaging, and a high index
level below 190 mg/dL (4. 92 mmol!L) and calculated
of clinical suspicion is necessary to establish the diagnosis.
10-year CHO risk of7.5% or higher
In this patient, effusive constrictive pericarditis is suggested
by the persistently elevated right atrial pressure following • Some patients without diabetes with an LDL choles
pericardiocentesis. terol level below 190 mg/dL (4.92 mmol/L) and cal
Similar findings in the jugular venous pulse can occur culated 10-year CHO risk of 5 % or higher but lower
in patients with cor pulmonale or heart failure. With cor than 7 . 5 %
pulmonale, however, evidence of right ventricular dysfunc
This patient has diabetes, an LDL cholesterol level less
tion or chamber enlargement is usually seen on imaging.
than 190 mg/dL (4.92 mmol/L), and a calculated 10-year
The clear lungs on auscultation do not support the presence
CHO of 10%, and, therefore, should be considered for
of heart failure.
high-intensity statin therapy. Drugs and doses that consti
The presence of a normal electrocardiogram and the
tute high-intensity statin therapy include atorvastatin, 40
absence of the typical symptom of chest pain argue against
to 80 mg/d; rosuvastatin, 20 to 40 mg/d ; and simvastatin,
recurrent acute pericarditis as a diagnosis. Additionally,
80 mg/d. (The FDA has issued a warning regarding the inci
acute pericarditis cannot explain this patient's pulsus par
dence of muscle injury with products that contain 80 mg of
adoxus and elevated jugular venous pulse.
simvastatin and recommends that patients be switched to a
KEY POINT different statin rather than increasing the dosage of simvas
tatin to 80 mg/d.)
• Effusive constrictive pericarditis is a clinical entity in
Fluvastatin, 40 mgld; lovastatin, 20 mgld; p ravasta
which patients who have a pericardia! effusion expe
tin, 10 mg/d; and simvastatin, 10 mg/d, are all classified
rience persistent symptoms and hemodynamic
as low-intensity dosing and are inadequate to reduce this
derangements after relief of the pericardia! effusion.
patient's CHO risk.
level in a patient with diabetes mellitus and an elevated Stone NJ. Robinson JG, Lichtenstein AH. et al: American College of
Cardiology/American Heart Association Task Force on Practice
risk for coronary artery disease. Guidelines. 2013 ACC/AHA guideline on the treatment of blood choles
terol to reduce atherosclerotic cardiovascular risk in adults: a report of
The most appropriate management in this patient with a the American College of Cardiology/American Heare Association Task
Force on Practice Guidelines. Circulation. 2014 Jun 24;129(25 Suppl 2):5 1 -
coronary heart disease (CHO) risk equivalent is to switch
45. Erratum i n : Circulation. 2014 Jun 24:129(25 Suppl 2):S46-8. [PMID:
to atorvastatin, 40 mg/d. Current guidelines recommend 24222016]
1 60
Answers a n d Criti q u es
For patients with heart failure, the two most common atrial septa) aneurysm with patent foramen ovale.
causes of a nonproductive cough are volume overload No further evaluation or treatment is the appropriate manage
and ACE inhibitors. An ACE inh ibitor-induced cough ment approach for this patient with an incidentally discov
may occur at any time after an ACE in hibitor has been ered atrial septa! aneurysm. Atrial septa! aneurysm is redun
initiated, and it would not be surprising that she could dant atrial septa! tissue that is often associated with a patent
develop an ACE inhibitor cough after 9 months of therapy. foramen ovale. When atrial septa! aneurysm is identified inci
The most appropriate course of therapy, therefore, would dentally, no medical treatment or intervention is needed.
be to switch to an ARB. Although data regarding mortality Antiplatelet therapy is recommended for patients with cryp
outcomes in patients with heart failure taking ARBs are togenic stroke and an isolated atrial septal aneurysm.
limited, results thus far demonstrate equivalent mortality In patients with an atrial septa! aneurysm and recurrent
outcomes and fewer medication-related adverse events in stroke while taking antiplatelet therapy. anticoagulant ther
this setting. apy is recommended if no other cause of stroke is identified.
An echocardiogram evaluates left ventricular function Rarely, surgical excision of an atrial septa] aneurysm
and valvular abnormalities. It would not be helpful in a and defect closure is considered in patients in whom anti
patient with diagnosed heart failure to assess for volume platelet or warfarin therapy fails to prevent stroke recurrence
overload. Indications for repeating echocardiograms in or in patients with a large left-to-right shunt causing right
patients with heart failure include a decline in functional heart enlargement. Percutaneous device closure is rarely
status and to reassess function after uptitrating medications. performed in patients with atrial septal aneurysms, because
Additionally, in patients followed over time, repeating an a large device is required to plicate the atrial septa! aneurysm
echocardiogram every 2 to 3 years is indicated to assess for and close multiple fenestrations.
further left ventricular dilation and evaluate left ventric Atrial septa! aneurysms are most commonly detected by
ular ejection fraction for further decline. It is unlikely to transesophageal echocardiogram. However, when an inci
be helpful in this patient, who has no evidence of volume dental atrial septa! aneurysm is well visualized by trans
overload on examination and no pulmonary edema on chest thoracic echocardiogram, additional imaging with a trans
radiograph. esophageal echocardiogram is not needed.
B-type natriuretic peptide (BNP) level is useful for
KEY POINT
the assessment of acute dyspnea. Studies have shown that
levels of BNP are elevated in patients with heart failure. • When an atrial septa! aneurysm is identified inciden
Additionally, higher levels are associated with an increase tally, no further evaluation, medical treatment, or
in mortality. BNP has not been demonstrated to be useful intervention is needed.
to guide diuresis in patients with heart failure. In a patient
with a history of heart failure, a random BNP level would Bibliography
not help in the assessment of fluid overload being the cause Burger AJ. Sherman H B, Charlamb Ml. Low incidence of embolic strokes
with atrial septal aneurysms: A prospective. long-term study. Am Heart
of a cough.
J. 2000 Jan: 139(1 Pt 1 ) : 149-52. [PMID: 10618576]
Pulmonary function testing is useful primarily in the
evaluation of dyspnea or to assess for the presence of under
lying lung disease that might contribute to cough, such as
Item 1 1 Answer: B
cough-variant asthma. However, this patient has no clinical
history or increased risk for lung disease, is a never-smoker, Ed ucational Objective: Manage a patient with stable
and has a normal lung examination. 111erefore, pulmonary angina pectoris and a low-risk stress test result.
function testing would not be appropriate prior to a trial of In this patient with ongoing stable angina pectoris and a
medication adjustment. low-risk exercise stress test result (that is, a Duke tread
KEY POINT mil l score of +6) , initiation of a long-acting nHrate such as
isosorbide mononitrate is recommended by current guide
• An ACE inhibitor-induced cough may occur at any
lines. �-Blockers and nitrates improve functional capacity,
time after an ACE inhibitor has been initiated; the
delay onset of exercise-induced myocardial ischernia, and
best course of therapy is to switch to an angiotensin
decrease the frequency and severity of anginal episodes. Most
receptor blocker. patients with stable angina will require combination therapy
with these two classes of dmgs to achieve effective control of
1 61
Answers a n d Critiq u es
angina1 symptoms. Although this patient is at low risk, initi with congenital lesions such as aortic coarctation, interrupted
ation of medical therapy is also appropriate in patients who aortic arch, and Turner syndrome. More than 70% of patients
are at intermediate risk and high risk based on clinical risk with a bicuspid aortic valve will require surgical intervention
factors, symptom burden, and/or stress testing results. Those for a stenotic or regurgitant valve or aortic pathology over
patients with intermediate-risk stress test findings (Duke the course of a lifetime. The presence of a bicuspid aortic
treadmill score of -10 to +4) and high-risk stress testing find valve increases the risk for aortic stenosis or regurgitation,
ings (Duke treadmill score of less than - 11) have a 1 % to 3% and stenosis proceeds at a faster rate when the aortic valve is
cardiovascular mortality per year and a 3% or higher cardio bicuspid. The risk for infective endocarditis also is increased in
vascular mortality rate per year, respectively. these patients. In addition, bicuspid aortic valve is associated
Calcium channel blockers, such as diltiazem, are sec with aortopathy and a predisposition to aneurysm formation
ond-line therapy in patients with stable angina pectoris who and thoracic aortic dissection.
are intolerant of �-blockers or who have continued symp Adults with previously undiagnosed aortic coarcta
toms on �-blockers and nitrates. This patient is tolerating his tion may present with hypertension or a murmur. Pal
�-blocker well and is not yet taking a nitrate for his angina. pation of reduced femoral pulses and measurement of
Therefore, diltiazem is not indicated at this time. discrepant blood pressures during routine examination are
Because of the invasive nature of coronary angiography helpful in raising suspicion for the diagnosis. The murmur
and the inherent risks of vascular complications, it should associated with coarctation may be nonspecific but is usu
be reserved for patients with lifestyle-limiting angina despite ally a systolic murmur in the left infraclavicular area and
optimal medical therapy or high-risk criteria on noninvasive under the left scapula.
stress testing such as significant ST-segment depression at a The murmur associated with an atrial septa1 defect is a
low work load, ST-segment elevation. or hypotension. midsystolic flow murmur caused by the ejection of increased
Pharmacologic nuclear stress testing is not indicated right-sided volume, owing to the left-to-right shunt that
in this patient owing to the presence of stable symptoms, occurs initially with this defect. This murmur is best heard
lack of optimal medical therapy, and low-risk findings on over the pulmonic area of the chest and may radiate toward
exercise stress testing. the back, as with the murmur of pulmonary stenosis. The
most characteristic finding on auscultation in patients with
KEY POINT
an atrial septa! defect is a fixed split S 2 .
• First-line antiangina1 therapy for patients with stable The murmur of mitra1 stenosis is a diastolic low-pitched
angina pectoris is �-blockers and nitrates. decrescendo murmur heard best in the left lateral decubitus
position. With mitral stenosis. S 1 has increased intensity
Bibliography and S 2 is normal. The opening snap, which is due to forceful
Qaseem A. Fihn SD, Dallas P. Williams S. Owens DK, Shekelle P; Clinical opening of the mitral valve, occurs 1..vhen the pressure in the
Guidelines Committee of the American College of Physicians.
Management of stable ischemic heart disease: summary of a clinical left atrium is greater than the pressure in the left ventricle.
practice guideline from the American College of Physicians/ American As the severity of the mitra1 stenosis increases, the pressure
College of Cardiology Foundation /American Heart Association/American
Association for Thoracic Surgery/ Preventive Cardiovascular Nurses
in the left atrium increases, and the mitra1 valve opens ear
Association/Society of Thoracic Surgeons. Ann Intern Med. 2012 Nov lier in ventricular diastole.
20;157(10) :735-43. [PMID : 231656651
KEY POINT
• A bicuspid aortic valve is often discovered inciden
Item 1 2 Answer: C
tally; the murmur depends on the degree of valve dys
Educational Objective: Diagnose bicuspid aortic valve. function, with a systolic ejection murmur that may
This patient most likely has a bicuspid aortic valve. Bicuspid range from a minimal flow disturbance to findings
aortic valve is the most common congenital heart lesion, consistent with the murmur of aortic stenosis as the
occurring in approximately 0.5% to 2% of the general popu degree of outflow obstruction increases.
lation. It is the second most common cause of aortic stenosis
after ca1cific degeneration of a tricuspid aortic valve and the Bibliography
second most common cause of aortic regurgitation after aor Siu SC, Silversides CK. Bicuspid aortic valve disease. J Am Coll Cardiol. 2010
Jun 22:55(25):2789-800. [PMID: 20579534]
tic root dilation. Many patients with a bicuspid aortic valve
are asymptomatic, with the diagnosis being suggested based
on incidentally noted auscultatory findings. The presenting Item 1 3 Answer: c
murmur depends on the degree of valve dysfunction, with a
Educational Objective: Recommend influenza vaccina
systolic ejection murmur that varies in intensity, ranging from
tion for the secondary prevention ofischemic heart disease.
minimal flow disturbance to findings consistent with the
murmur of aortic stenosis as the degree of outflow obstruc Providing this patient with an annual influenza vaccination
tion increases. A diastolic murmur may occur if aortic valve would significantly reduce her risk of future cardiovascular
incompetence with regurgitation is present, as in this patient. events. A meta-analysis of randomized trials demonstrated
Bicuspid aortic valve has an increased prevalence associated that use of the influenza vaccine was associated with a 36%
1 62
Answers a n d Critiq u es
lower risk of major adverse cardiovascular events compared interval of 630 ms: a normal QTc is defined as 460 ms or less
with nonimmunized patients. Based on these data, the Amer in women and 440 ms or less in men. A QTc greater than 500
ican Heart Association and American College of Cardiology ms is associated wit h increased risk of torsades de pointes.
recommend influenza vaccination for the secondary preven ll1e cause of t his patient's QT prolongation is l i kely
tion of ischemic heart disease. In addition to influenza vac her pharmacot herapy. Many drugs have been impl icated in
cination as a preventive measure for cardiovascular disease, QT-interval prolongation . including antiarrhythmic agents.
this patient also qualifies for influenza vaccination according antibiotics (including some macrol ides and fluoroquino
to Advisory Committee on Immunization Practices (ACIP) lones ) , ant ipsychotic d rugs. and ant idepressants. A Ust of
guidelines that recommend all persons aged 6 months or QT-prolonging drugs is avai lable at http: //crediblemeds.org/.
older receive the influenza vaccination. The large degree of" QTc prolongation in t h is patient may be
Colchicine has anti-inflammatory properties, and caused by either the presence of two QT-prolonging med
observational studies of patients taking colchicine for gout ications or an underlying ion channel variant t hat is only
or familial Mediterranean fever suggest a decreased risk of evident in the setting of a QT-prolonging medication.
cardiovascular disease associated with treatment. However, A l t hough bot h medications may lead to her ECG find
its use for secondary prevention of cardiovascular disease ings. discontinuing either the moxifloxacin or amitriptyline
has not been established. i n isolation is insu fficient treatment given the sign ificant
Folic acid lowers homocysteine levels, which have degree of QT prolongation on t his patient's ECG.
been associated with increased cardiovascular disease in Carvedi lol does not prolong t he QT interval directly.
observational studies. However, clinical trials examining although it may increase t he risk of bradycardia-related
the effectiveness o f lowering homocysteine levels by folic arrhythmias in patients with acquired QT- i n terval prolon
acid supplementation have failed to show a reduction in ga tion by slowing t he heart rate. H owever, the most import
adverse cardiovascular events. Folic acid supplementa ant i ntervention in this patient is to reduce the risk of
tion for this purpose is therefore not recommended as torsades de pointes by d iscont i nu ing the oflending QT- pro
secondary prevention. longing agents.
Because inflammation and oxidative stress are involved
KEY POINT
in atherosclerosis, the use of antioxidant agents, including
vitamins E and C and P-carotene, has been proposed as both • A corrected QT interval greater than 500 ms is associ
a primary and secondary preventive intervention for cardio ated with increased risk of torsades de pointes;
vascular disease. Although supported by some basic science patients with this degree of QT-interval prolongation
and observational data, several large, randomized controlled should discontinue any potentially QT-interval pro
trials have failed to document benefit of antioxidant therapy longing drugs.
for either purpose. Therefore, the use of vitamin E supple
mentation would not be appropriate in this patient. Bibliography
Isbister GK, Page CB. Drug induced QT prolongation: the measurement and
KEY P O I NT assessment of the QT interval in clinical practice. Br J Clin Pharrnacol.
2013 Jul;76(1}:48-S7. [PMID: 231675781
• Influenza vaccine should be administered to patients
with established cardiovascular disease to reduce the
risk of future cardiovascular events.
Cl
Item 1 5 Answer: c
Bibliography
Educational Objective: Manage heart failure with
Udell JA. Zawi R, Bhatt DL. et al. Association between innuenza vaccination preserved ejection fraction with diuretics.
and cardiovascular outcomes in high-risk patients: a meta-analysis.
JAMA. 2013 Oct 23;310(16):1711-20. [PMID: 241504671 ll1is patient should be admitted to the hospital and given
intravenous furosemide. H is presentation is characteristic for
heart failure with preserved ejection fraction ( H FpEF) . He has
volume overload manifested by i ncreasing abdominal girth,
Cl Educational Objective:
Item 1 4 Answer: c
i ncreased exertional dyspnea, and progressive orthopnea. His
Treat acquired QT-interval
left ventricular ejection fraction is normal, but he has mild left
prolongation.
ventricular hypertrophy and a long histo1y of hypertension.
Both amitriptyline and rnoxifloxacin should be discontinued Additionally, he has a markedly elevated B-type natriuretic
in t his patient. Her electrocardiogram (ECG) demonstrates peptide level. ll1e etiology of h is acute exacerbation i nto heart
QT- i nterval prolongat ion. Many medications may prolong the failure is most l i kely acute atrial fibrillation, but because he is
QT i nterval. including amitriptyline and moxifloxacin: QT al ready on diltiazem and has a normal heart rate. he may have
prolongation may be markedly increased in patients taking been in atrial fibrillation for some t i me and not noticed it.
more than one medication with this effect. I n con trast to patients with a reduced ejection fraction,
The corrected QT i nterval (QTc) is most often deter no drugs have been shown to reduce mortal i ty rates in
mined using the Bazett formula (QT interval I v'R-R i nter patients with H Fp EF. I nstead, guidelines emphasize con
val ) . This patient's ECG at t he time of admission shows a QTc t ro l l i ng blood pressure and volume. Patients with H FpEF a re
1 63
Answers a n d Criti q u es
Bibliography
Cl Educational
Item 1 6 Answer:
Objective:
B
Treat low-pressure cardiac tam
Sagrista-Sauleda J. Angel J. Sambola A. Alguersuari J. Permanyer-Miralda G.
Soler-Soler J. Low-pressure cardiac tamponade: clinical and hemody
ponade. namic profile. Circulation. 2006 Aug 29:114(9):945-52. [PMID: 16923755]
1 64
Answers a n d Critiques
approximately 50% of the risk of developing acute Ml. The reasonable second-line therapy in patients who are
INTERHEART study assessed the prevalence of nine poten intolerant of �-blockers or who have continued symp
tially modifiable risk factors in more than 15,000 patients toms on �-blockers and nitrates. However, it would not
with first acute MI and almost 15,000 asymptomatic age- and be appropriate to switch to a calcium channel blocker,
sex-matched controls. Nine risk factors were strongly associ such as amlodipine, in this patient who currently toler
ated with acute MI in the 52 countries included in the trial. ates an effective dose o f a �-blocker.
In descending order, these are: dyslipidemia, smoking, psy Myocardial viability testing is performed with a radio
chosocial stressors, diabetes mellitus, hypertension, obesity, nuclide radiotracer that is taken up by viable myocardial tis
alcohol consumption, physical inactivity, and diet low in fruits sue. Viability testing may demonstrate hypoperfused regions
and vegetables. Results of the INTERHEART study suggest that of the heart that might show functional improvement if
these modifiable risk factors account for more than 90% of the revascularization is performed. However, information from
risk for acute Ml. a substudy of the Surgical Treatment of Ischemic Heart Fail
KEY POINT ure (STICH) trial demonstrated no relationship between the
results of viability imaging and the effectiveness of bypass
• Nine modifiable risk factors account for more than
surgery. Therefore, in this patient who remains symptomatic
90% of the risk for acute myocardial infarction; in
despite optimal medical therapy and is a reasonable surgical
descending order, these are: dyslipidemia, smoking, candidate, revascularization is indicated, and myocardial
psychosocial stressors, diabetes mellitus, hyperten perfusion testing would not contribute significant informa
sion, obesity, alcohol consumption, physical inactivity, tion regarding medical decision making. Myocardial perfu
and diet low in fruits and vegetables. sion testing is typically limited to use in patients at high risk
for revascularization surgery in whom assessing the degree
Bibliography of viable myocardium present may influence the risk-benefit
Yusuf S, Hawken S, Ounpuu S. et al: INTERHEART Study Investigators. ratio of surgical treatment.
Effect of potentially modifiable risk factors associated with myocardial
infarction in 52 countries (the INTER H EART Study) : case-control study. KEY POINT
Lancet. 2004 Sep ll- 17;364(9438):937-52. [PM I D : 15364185]
• Coronary artery bypass grafting is recommended for
patients who remain symptomatic with optimal med
ical therapy and have specific angiographic findings
Item 1 8 Answer: B
(either left main disease or multivessel disease with
Educational Objective: Manage a patient with diabetes
involvement of the proximal left anterior descending
mellitus and three-vessel coronary artery disease.
artery) , concomitant reduced systolic function, or dia
The appropriate management of this patient is coronary artery betes mellitus.
bypass grafting (CABG). He has type 2 diabetes mellitus and
multivessel coronary artery disease (CAD) with moderate to Bibliography
severe symptoms despite optimal medical therapy. In multi Farkouh ME. Domanski M, Sleeper LA. et al: FREEDOM Trial Investigators.
ple observational studies and randomized controlled trials, Strategies for multivessel revascularization in patients with diabetes.
N Engl J Med. 2012 Dec 20:367(25):2375-84. [PMID: 23121323]
performing CABG compared with percutaneous coronary
intervention (PC!) as the initial revascularization strategy in
patients with a clear indication was associated with improved Item 1 9 Answer: C
outcomes, including reduced rates of death, myocardial
Educational Objective: Evaluate coronary artery disease
infarction (Ml) , and stroke. The FREEDOM trial evaluating
in a patient with COPD.
management of multivessel CAD in patients with diabetes
showed that the composite endpoint of death, MI, and stroke This patient should undergo dobutamine stress echocardi
was significantly lower in patients treated with CABG versus ography. She has a history of coronary artery disease (CAD)
PC!. This difference was driven by a statistically significant with new atypical, but exertional, symptoms suggestive of
reduction in the occurrence of death and MI in CABG patients, cardiac ischemia. Because she has baseline electrocardiogram
although stroke rates were higher in the CABG group than the abnormalities (left ventricular hypertrophy with repolariza
PC! group. CABG is recommended for patients who remain tion abnormalities) that will make interpretation of ST-seg
symptomatic despite optimal medical therapy and have spe ment changes difficult, she should undergo stress testing with
cific angiographic findings (either left main disease or mul imaging, with either stress echocardiography with dobuta
tivessel disease with involvement of the proximal left anterior mine or myocardial perfusion imaging with a vasodilator.
descending artery) , concomitant reduced systolic function, or With stress echocardiography, regional myocardial
diabetes mellitus. function is assessed in real time. Stress images are obtained
�-Blockers are first-line antianginal agents because at peak or immediately after stress, before cardiac func
of their ability to reduce heart rate, myocardial contrac tion returns to baseline. Wall motion abnormalities indicate
tility, and blood pressure, resulting in reduced myo either infarction (seen on stress and rest images) or isch
cardial oxygen demand. Calcium channel blockers are emia (seen on stress images only) . For patients who cannot
1 65
Answers and Critiques
exercise, such as this patient, pharmacologic stressors such associated with the procedure. Additionally, this patient
as dobutamine in combination with imaging can be used in is asymptomatic, so there is no indication for intervention
place of exercise and imaging. at present.
Because of her COPD with active wheezing on exam Surgical aortic valve replacement is indicated for
ination, pharmacologic testing with vasodilators should be symptomatic patients with severe aortic stenosis, asymp
avoided. Pharmacologic vasodilators, such as dipyridamole, tomatic patients with severe aortic stenosis and LV systolic
adenosine, and regadenoson, can cause bronchospasm and dysfunction (LV ejection fraction <50%), and patients with
are therefore contraindicated in a patient who is actively severe aortic stenosis who are undergoing coronary artery
wheezing. These agents can be used with caution in a patient bypass graft or surgery on the aorta or other heart valves.
with a history of bronchospastic airways disease, but the This patient is asymptomatic with normal LV systolic func
presence of active wheezing in this patient precludes the use tion, and he does not have any other cardiac procedures
of a vasodilator. Therefore, stress testing with dobutamine is planned.
the correct choice. Transcatheter aortic valve replacement (TAVR) is indi
This patient has no symptoms to indicate an acute cor cated for patients with symptomatic severe aortic steno
onary syndrome that would prompt cardiac catheterization sis who are considered unsuitable for conventional surgery
as the initial diagnostic test. Evaluation of the extent and because of severe comorbidities. Currently, TAVR should not
severity of disease would be the first step in deciding man be performed in patients with intermediate or low surgical
agement in this patient. If she has a small perfusion defect, risk, and no therapeutic intervention is currently indicated
she could be treated medically with more intensive antiangi in this asymptomatic patient.
nal therapies.
KEY POINT
KEY P O I NT • I n patients with asymptomatic severe aortic stenosis,
• Pharmacologic vasodilators, such as dipyridamole, close clinical follow-up with echocardiography every
adenosine, and regadenoson, can cause broncho 6 to 12 months is appropriate.
spasm during cardiac stress testing; these agents can
be used with caution in a patient with a history of Bibliography
COPD but are contraindicated i n a patient who is Manning WJ. Asymptomatic aortic stenosis in the elderly: a clinical review.
JAMA. 2013 Oct 9;310(14):1490-7. [PMID: 241043731
actively wheezing.
Cl
Bibliography Item 2 1 Answer: c
Qaseem A. Fihn SD, Williams S. Dallas P. Owens DK. Shekelle P; Clinical
Guidelines Committee of the American College of Physicians. Diagnosis Educational Objective: Diagnose stress cardiomyopathy
of stable ischemic heart disease: summary of a clinical practice guideline (takotsubo cardiomyopathy) .
from the American College of Physicians/American College of Cardiology
Foundation/American Heart Association/American Association for ·n1is patient's clinical history and presentation are consis
Thoracic Surgery/Preventive Cardiovascular Nurses Association/Society
of Thoracic Surgeons. Ann Intern Med. 2012 Nov 20;157(10) :729-34. tent with stress cardiomyopathy (takotsubo cardiomyopa
[PMID: 23165664] thy) . ll1e absence of coronary artery stenosis and t he pres
ence of hypokinesis of t he mid and apical left ventricle on
Item 2 0 Answer: B ventriculography confirm this diagnosis. ll1is patient with
takotsubo cardiomyopathy without evidence of cardiogenic
Educational Objective: Manage asymptomatic severe
shock should be administered metoprolol and captopril. ll1e
aortic stenosis.
treatment of stress cardiomyopat hy is supportive. including
Although this patient has severe aortic stenosis based on t he use of �-blockers and ACE inhibitors. and most patients
quantitative echocardiographic findings, he is asymptom have resolution of symptoms and recovery of left ventricular
atic with normal left ventricular (LV) systolic function; function within 7 days.
therefore, follow-up echocardiography in 6 to 12 months Takotsubo cardiomyopat hy often mimics non-ST
is the most appropriate management. Appropriate fol elevation myocardial infarction (NSTEMl) or ST-elevation
low-up in patients with asymptomatic severe aortic ste myocardial infarction (STEM!) . Patients present with chest
nosis includes a clinical evaluation and echocardiography pai n or short ness of breat h, e lectrocardiographic cha nges
every 6 to 12 months. Patients should also be educated to consistent with anterior and/or lateral ST-segment eleva
identify and report possible aortic stenosis-related symp tion . and elevated cardiac biomarkers. Although not required
toms, such as dyspnea, reduced exercise tolerance, exer for diagnosis, many patients develop symptoms following a
tional chest pain, lightheadedness, and syncope, before stressful or emotional event. The diagnosis of stress cardiomy
scheduled follow-up. opathy requires (1) ST-segment elevation on electrocardiogra
Balloon valvuloplasty, although important in the phy. (2) transient wall motion abnormalities of the mid and
treatment of the pediatric patient with severe aortic ste apical left ventricle. (3) t h e absence of significant obstrnctive
nosis, has a more limited role in adults owing primarily coronary artery disease. and (4) the absence of other causes
to its limited efficacy and the high rate of complications of transient left ventricular dysfunction. such as myocarditis.
1 66
A nswers a n d Critiques
Cl
CONT.
E nd omyocard ial biopsy is genera l ly not indicated for
t he i n i t ial evaluation of heart failure unless a speciAc diag
feet equally and a loud early systolic murmur heard at the
left sternal border related to the right ventricular outflow
nosis that wou ld i nfluence ma nagement or prognosis is tract obstruction. The pulmonic component of the S 2 is
suspected based on clinical data or noninvasive testing. This generally soft or absent depending on the degree of pul
patient's presentation, with acute onset following a st ress fu l monary valve stenosis. Right ventricular hypertension but
event, ST-segment elevation, and hypokinesis of the cardiac not pulmonary hypertension is present; the pulmonary
apex. is cha racteristic of takotsubo cardiomyopathy. a nd an vasculature is protected by the presence of pulmonary
en domyo ca rd ia l biopsy is not indicated as a n i nitial d iag valve stenosis.
nostic test. Myocarditis has a variable presentation , but focal A ventricular septa! defect can cause pulmonary
ST-segment changes and apical hypokinesis are not typical. hypertension if it remains open beyond age 2 years. Clin
More than 95% of patients with stress cardiomyopathy ical features include cyanosis and clubbing that affect the
recover ventricular function with conservative supportive hands and feet equally, a parasternal lift, and increased S 2 .
care (�-blockers and ACE inhibitors) . This patient does not Differential clubbing and cyanosis would not occur in a
have evidence of hemodynamic compromise. and intra-aor patient with pulmonary hypertension related to a ventric
tic balloon pump implantation is not indicated. ular septa! defect.
In t h is patient, the lack of coronary artery obstructive
K EV P O I N T
disease i n the presence of ST- segment elevation and elevated
cardiac biomarkers eliminates STEM! or NSTEM I as poss i • A patent ductus arteriosus with Eisenmenger syn
ble etiologies for this presentation . Therefore. t h rombolytic drome is characterized by differential cyanosis and
therapy is not indicated. clubbing affecting the lower body.
KEV POINT
Bibliography
• Stress cardiomyopathy presents similarly to myocar Srinivas SK. Manjunath CN. Differential clubbing and cyanosis: classic signs
dial infarction, with ST-segment elevation and, often, of patent ductus arteriosus with Eisenmenger syndrome. Mayo Clin Proc.
2013 Sep;88(9):el05-6. [PMID: 24001503]
elevated cardiac biomarkers; however, coronary angi
ography demonstrates an absence of significant
obstructive coronary artery disease.
Item 23 Answer: A
Educational Objective: Treat a patient with acute
Bibliography
decompensated heart failure and evidence of low cardiac
Prasad A, Lerman A. Riha! CS. Apical ballooning syndrome (Tako-Tsubo or
stress cardiomyopathy): a mimic of acute myocardial infarction. Am output.
Heart J. 2008 Mar:l55(3):408-17. [PMID: 18294473]
This patient should be started on dobulamine for proba
ble cardiogenic shock. Cardiogenic shock is present when
1 67
Answers a n d Critiq u e s
Cl Educational Objective:
Item 24 Answer: B
Manage type A aortic dissection.
Cl
Item 2 5 Answer: D
The most appropriate management for this patient with an
Educational Objective: Manage antibiotic prophylaxis
acute type A aortic dissection is emergency surgical inter
in a patient with a mechanical aortic valve prosthesis.
vention. The abrupt onset of severe chest and back pain is
typical of an acute aortic syndrome. A diastolic murmur con For t h is patient with a mechanical valve preparing for hernia
sistent w i l h aortic valvu lar i nsufficiency increases the clin ical repai r su rgery. antibiotic prophylaxis to prevent bacterial
suspicion for a prox imal (type A) aortic dissection t hat has endocarditis is not i ndicated. Prophylaxis to prevent bacte
d isrupted normal valve leaflet coapta l ion. Acute aortic dissec rial endocard i t is is appropriate before certain dental proce
t ion is the most common l i fe-threatening disorder aflecting d u res for patients with specific i ndica tions placing them at
the aorta. In the Stan ford classi ficat ion. type A dissections h igh ris k for an adverse outcome from i n fective endocarditis
involve t he ascending aorta. and type B d issections are those (class I la recommendation) . These i nd ications include pre
t hat do not involve the ascending aorta. Type A dissections vious endocard i t is. a h i story of cardiac transplantation . a
require emergency surgical repair, whereas medical therapy, prosthetic valve, and specific forms of complex congenital
consisting of a �-blocker to decrease t he heart rate to below heart d isease. However. prophylaxis is not recom mended for
6 0/min plus add i t ional medications as needed to control nondental proced u res. i ncluding t ra nsesophageal echocar
hypertension, is usually t he initial st rategy for acute type B cliography and genitourinaty or gastrointestinal procedures
dissections. Therefore. pursuing medical managemen t alone (such as upper endoscopy. colonoscopy, or hernia repai r) .
wou ld not be appropriate i n this pat ient. ·n1e immediate mor i n the absence o f active i n fection (class I l l recommenda
tality rate in aortic dissection is as high as l 'Y., per hour over tion) . Dental procedures for which a n t i biotic prophylaxis
the First several hours, making early diagnosis and treatment is reasonable i nclude t hose t hat i nvolve manipulation o f
crit ical for survival. gi ngival t i ssue or t he periapical region of tee t h or perfora
A l t hough most p at i e n t s w i t h d i ssec t i o n have underly i n g t i o n of' t h e oral m ucosa. Prophylaxis is n o t reco m m en ded
hypertension. only a t i ny fraction of all persons with hyper fo r rou t i ne dental procedu res, inclu d i ng radiographs a nd
tension ever have a d issect ion . Syncope occurs in approx i ort hodon t ics.
mately IO'X. of patients w i t h an acute aortic dissection a n d is When antibiotic prophylaxis is indicated. it shou ld be
more commonly associated with prox imal dissect ion . Pulse given as a single dose 30 to 60 minutes before t h e dental
deficits occur in less than 20% of type A d issections. Abnor procedure. I f' t he prophylactic medica tion is inadvertently
mal aortic contour or widening of the aortic s i l houette may not admin istered, it may be given u p to 2 hours a fter the
be an important clue to the d iagnosis of' aortic d issect ion . procedu re. Options include amoxici l l i n , 2 g oral ly. o r ampi
However. a normal chest radiograph is seen in nearly I S'Y.. c i l l i n . 2 g i ntravenously. For patients allergic to pen ici l l i n or
of patients with acute aortic dissection . The 1 0-year survival amoxicill i n, alternatives include clindamycin. 600 mg orally;
1 68
Answers and Criti q u es
CJ azit h romycin. 500 mg orally; or cetazo l i n /ceftriaxone, 1 g not be used for the diagnosis of PAD. Moreover, this patient
i n t ram uscu larly or i n t ravenously.
CONT. has chronic kidney injury, and use of contrast for either
KEY POINT study should be avoided if possible.
Bibliography Bibliography
Bonow RO, Carabello BA, Chatterjee K, et al; American College of Cardiology/ Aboyans V, Criqui M H , Abraham P. et al: American Heart Association
American Heart Association Task Force on Practice Guidelines. 2008 Council on Peripheral Vascular Disease; Council on Epidemiology and
focused update incorporated into the ACC/AHA 2006 guidelines for the Prevention; Council on Clinical Cardiology; Council on Cardiovascular
management of patients with valvular heart disease: a report of the Nursing; Council on Cardiovascular Radiology and Intervention, and
American College of Cardiology/American Heart Association Task Force Council on Cardiovascular Surgery and Anesthesia. Measurement and
on Practice Guidelines (Writing Comminee to revise the 1998 guidelines interpretation of the ankle-brachia! index: a scientific statement from
for the management of patients with valvular heart disease). Endorsed the American Heart Association. Circulation. 2012 Dec 11;126(24):2890-
by the Society of Cardiovascular Anesthesiologists, Society for 909. Erratum in: Circulation. 2013 Jan 1;127(l):e264. [PM I D : 23159553]
Cardiovascular Angiography and Interventions. and Society of Thoracic
Surgeons. J Am Coll Cardiol. 2008 Sep 23;52(13):el-142. [PMID: 18848134]
Cl
Item 2 7 Answer: D
Educational Objective: Manage atrial fibrillation in a
Item 2 6 Answer: D
patient following percutaneous coronary intervention for
Educational Objective: Diagnose peripheral arterial treatment of refractory angina.
disease in a patient with uninterpretable ankle-brachia}
This pal ient should be treated w i t h aspirin. clopiclogrel, and
index testing.
warfarin ("lriple therapy") . He has new-onset atrial fibrillation
The most appropriate diagnostic test to perform next in this in the set t i ng of' recent bare metal slent placement for medi
patient is a toe-brachia! index. The ankle-brachia! index (AB!) cally rcf'raclory a ngi na. Palienls with a bare metal stent should
is obtained by measuring the systolic pressures in the dorsalis be lrealed w i t h dual anliplatelet t herapy for at least l month
pedis and posterior tibialis arteries on both sides. The AB! for to al low endothelial iz<Jtion of t he stenl; with clrug-eluling
each leg is the highest ankle pressure for that side divided slents. t he requ i rement for dual antiplalelet t herapy is longer
by the highest brachia! pressure (regardless of side) . An AB! and depends upon the lype of' stcnt implanted. ·1 11is patient is
of 0. 90 or lower establishes a diagnosis of peripheral arterial also a t h igh risk or t h romboembolic d isease associated w i l h
disease (PAD) . In this patient, the right AB! is greater than alrial fibril lalion . He has a CH1\DS1-VASc score of 5 (2 points
1.40. An AB! above 1.40 suggests noncompressible vessels, for age >75 years . I poin t each for diabetes melli t us, hyper
which may reflect medial calcification but is not diagnostic tension. and vascular disease) . ll1erefore, oral <Jnticoagu lanl
of flow-limiting atherosclerotic disease. An AB! greater than t herapy is also i ndicated. Although l riple t herapy w i t h two
1 .40 is associated with worse cardiovascular outcomes than antiplalelel agents and systemic a n t i coagulation is associated
a normal AB!. In such patients, an appropriate next step is to wilh a significant increase in bleeding risk, t h is regimen is
either measure great toe pressure or calculate a toe-brachia! appropriate t reatment i n this pat ient !b r at leasl 1 mo nt h u n t i l
index (systolic great toe pressure divided by systolic brachia! stent endothelialization can b e assured. at which t i m e h e can
pressure) . Vessels within the great toe rarely become noncom be transitioned to only aspirin ancl an oral anlicoagulant to
pressible, and a great toe systolic pressure below 40 mm Hg or decrease bleeding risk but provide adequate t hromboembolic
a toe-brachia! index of less than 0.70 is consistent with PAD. prophylaxis. I f' warfarin is used as an anticoagulant during
An AB! obtained immediately following symptom triple t herapy. careful maintenance of' t he I N R w i t h i n lhe rec
limited exercise is useful when a high clinical suspicion ommended range of 2 .0 lo 2.5 in patients without mechanical
for PAD remains despite a normal (1.00-1 .40) or borderline valves may reduce the overall bleeding risk.
resting AB!. A decrease of the AB! by 20% compared with Aspirin and clopiclogrel are i n ferior to oral an ticoagula
the resting AB! is consistent with significant PAD. This lion for the prevenlion of stroke in patients w i t h an i n d ica
patient's resting AB! is above the normal range; therefore, tion for a n t icoagu lat ion for th romboembolism prophylaxis
exercise AB! would not help to establish the diagnosis. i n a lrial fibri l lat ion .
In patients with an established diagnosis of PAD and Treatment with aspirin and dabigatran is not opti m a l
indications for revascularization, further vascular anatomic f C i r two reasons. Firsl. i n the Random ized Eva luation of
data can be obtained noninvasively using gadolinium Long Term A n t i coagu lant ·1 herapy ( R E-LY) trial, t here was
enhanced magnetic resonance angiography or contrast a n u meric excess of' myocard ial i n farclions observed w i t h
enhanced multi-detector CT angiography. However, these dabigatran . More i m portanl ly, no dala are ava i lable regard
tests should be reserved for planning intervention in patients ing l he e f ficacy of aspi rin and dabigal ran for l h e p revention
who have not benefited from medical therapy; they should of' stenl t h rombosis fol low i ng an acute coronary syndrome.
1 69
Answers a n d Critiques
1 70
Answers and Criti ques
KEY POINT . ·
Management of Cardiovascular Diseases during Pregnancy of the
European Society of Cardiology (ESC). Eur Heart J. 2011 Dec-32(24):3147-
'
• Fixed splitting of the S2 throughout the cardiac cycle 97. [PM l D : 21873418)
and a right ventricular heave are characteristic clinical
features of atrial septal defect.
Item 3 1 Answer: A
Bibliography
Educational Objective: Manage abdominal aortic aneu
Baumgartner H. Bonhoeffer P, De Groot N M , et al; Task Force on the
rysm with referral for repair.
Management of Grown-up Congenital Heart Disease of the European
Society of Cardiology (ESC); Association for European Paediatric
Cardiology (AEPC); ESC Committee for Practice Guidelines (CPG) ESC The most appropriate management is to refer this patient
Guidelines for the management of grown-up congenital heart di�ease for abdominal aortic aneurysm (AAA) repair. AAA is a
(new version 2010). Eur Heart J. 2010 Dec·31(23):291S-S
' 7 . [PMlD·. common and potentially life-threatening condition, and
20801927)
management of detected aneurysms is based on size or rate
of expansion. Elective repair to prevent rupture in asymp
Item 3 0 tomatic patients is optimal management in those meet
Answer: C
ing criteria for intervention. Once an aneurysm reaches
Educational Objective: Manage a patient with mitral
5 . 5 cm in men and 5 . 0 cm in women, repair is generally
valve stenosis considering pregnancy.
warranted. Repair may be performed by an open approach
This patient has clinical and echocardiographic features of or an endovascular approach, if the anatomy of the aneu
severe mitral valve stenosis as indicated by mitral valve mean rysm is amenable; the mode of therapy should be decided
gradient, valve area, and pulmonary artery systolic pressure by the surgeon, the internist, and the patient after a com
greater than SO mm Hg. Planned pregnancy is a class I indi prehensive discussion of risks and long-term benefits. Ran
cation for intervention in patients with severe rnitral steno domized trials show that endovascular aneurysm repair
sis despite the absence of baseline symptoms. Most young (EVAR) is associated with lower perioperative morbidity
patients will be candidates for mitral balloon valvuloplasty. and mortality compared with open AAA repair, but EVAR
In severe rnitral valve stenosis, negative chronotropic does not completely eliminate the future risk of AAA rup
drugs such as �-blockers allow increased diastolic filling ture. Open repair is associated with higher perioperative
time of the left ventricle and may improve symptoms. If morbidity and mortality than EVAR, but it provides a more
atrial fibrillation develops (even if paroxysmal), chronic definitive repair.
anticoagulation therapy with warfarin is indicated to reduce The optimal surveillance schedule for patients once
the risk of thromboembolism, which is much higher than an AAA has been identified has not been clearly defined.
in nonvalvular atrial fibrillation. ACE inhibitors provide no Annual surveillance is recommended, but larger aneu
particular benefit to patients with mitral stenosis and are rysms expand faster than small ones and may require
contraindicated in pregnant patients. Dabigatran is approved more frequent surveillance. Aneurysm diameter is the
for prevention of systemic embolism in adults with nonval most important factor predisposing to rupture, with risk
vular atrial fibrillation; however, its effectiveness in pre increasing markedly at aneurysm diameters greater than
S . S cm. For asymptomatic patients, the risk of AAA rup
venting embolism in patients with valvular heart disease is
unknown, and it is not recommended in this setting. ture generally exceeds the risk associated with elective
Cardiac magnetic resonance imaging will not add AAA repair when aneurysm diameter exceeds 5 . 0 cm in a
incremental information to determine therapeutic strategy woman and 5 . 5 cm in a man. This patient's AAA is 5 . 7 cm
for this patient with rheumatic mitral stenosis considering in diameter; therefore, she should be referred for repair,
pregnancy. rather than continuing surveillance.
Pregnancy is associated with a marked increase in blood Although controlling risk factors for cardiovascular
volume and cardiac output. Patients with severe mitral ste disease is essential in patients with AAA , there is little com
nosis and moderate pulmonary hypertension often develop pelling evidence for treating hypertension in these patients
symptoms during pregnancy and should receive interven with a specific agent, including �-blockers, to prevent aneu
tion prior to pregnancy. rysm expansion. As this patient's blood pressure is well con
trolled, no change in antihypertensive therapy is indicated.
KEY POINT
K E Y P O I NT
• Planned pregnancY. iS a class I indication for mitral
• An abdominal aortic aneurysm larger than 5.5 cm in
valve intervention in patients with severe mitral valve
men and 5 . 0 cm in women is an indication for referral
stenosis despite the absence of baseline symptoms.
for repair.
Bibliography
European Society of Gynecology (ESG); Association for European Paediatric Bibliography
Cardiology (AEPC); German Society for Gender Medicine (DGesGM). Buck DB. van Herwaarden JA, Schermerhorn ML, Moll FL. Endovascular
Regitz-Zagrosek V, Blomstrom Lundqvist C, Borghi C, et al; ESC treatment of abdominal aortic aneurysms. Nat Rev Cardiol. 2014
Committee for Practice Guidelines. ESC Guidelines on the management Feb;ll(2) :112- 23. Erratum in: Nat Rev Cardiol. 2014 Feb;l l (2 ) : i. [PMl D :
of cardiovascular diseases during pregnancy: the Task Force on the 24343568]
1 71
Answers a n d Critiq u es
Bibliography
Haley JH, Tajik AJ, Danielson GK, Schaff HV, Mulvagh SL, Oh JK. Transient
constrictive pericarditis: causes and natural history. J Am Coll Cardiol. Item 34 Answer: A
2004 Jan 21;43(2):271-5. [PMID: 147364481
Educational Objective: Manage discharge of a patient
with heart failure to prevent read m iss ion .
1 72
Answers a n d Critiq u es
1 73
Answers and Critiques
Item 38 Answer: C
Item 37 Answer: A Educational Objective: Manage medication use prior to
Educational Objective: Diagnose a murmur heard in a stress testing.
pregnant woman.
This patient's metoprolol should be withheld for 48 hours
The most likely cause of this woman' s murmur is a bicus before stress testing. In this intermediate-probability patient
pid aortic valve, the most common congenital heart abnor with a normal electrocardiogram, exercise stress testing is
mality. The characteristic finding of a bicuspid aortic valve is appropriate. Exercise stress is preferred to pharmacologic
an aortic ejection sound associated with either a systolic or stressors because it provides a gauge of functional capacity
diastolic murmur. While the murmur associated with aortic and a contextual understanding of symptoms, and it records
stenosis usually radiates to the carotid arteries, the murmur hemodynamic response to exercise. The sensitivity of the
1 74
Answers and Criti ques
study to detect obstructive coronary artery disease (CAD) is studies in this clinical setting and would allow for further
lowered, however, if patients are taking certain medications. stratification of this patient's risk for ischemic heart disease.
P-Blockers and nondihydropyridine calcium channel block Proceeding directly to cardiac catheterization may be
ers can blunt the maximal heart rate that can be achieved appropriate in patients at very high risk for ischemic heart
with exercise and may limit a patient's ability to reach 85% of disease in whom noninvasive testing would not be expected
the maximal predicted heart rate. However, dihydropyridine to significantly change the pretest probability of disease.
calcium channel blockers do not need to be withheld prior However, in this patient with an intermediate risk of disease,
to testing. Similarly, digoxin can limit the maximal heart rate adequate noninvasive testing would be helpful in evaluating
and should be withheld. Nitrates are effective antianginal for the presence of ischemic heart disease, and coronary
agents but may minimize the ischemic response on stress angiography would not be indicated as a next diagnostic test.
testing; therefore, they should also be withheld. If, however, Switching the patient's antihypertensive medication to
a patient has known CAD and the goal of testing is to deter an agent with antianginal properties would not be indicated
mine whether symptoms are related to ischemia or to assess without establishing the presence of ischemic heart disease,
adequacy of antianginal therapy, there is no need to stop any particularly with adequate control of her blood pressure on
of the medications. her current regimen.
There is no evidence that ACE inhibitors, such as lisin Because an inadequate exercise ECG exercise test is
opril, or angiotensin receptor blockers alter the sensitivity unable to assess for the presence of ischemic heart disease in
of exercise stress testing in the diagnosis of CAD, and these this patient at intermediate risk, clinical observation without
agents do not need to be discontinued before testing. For this further evaluation would not be appropriate.
patient on several medications for hypertension, an appro
KEY POINT
priate strategy would be to continue hydrochlorothiazide
and lisinopril and discontinue metoprolol. • Exercise stress testing is recommended as the initial
test of choice for patients with intermediate risk of
KEY POINT
ischemic heart disease who are capable of exercising
• In patients undergoing stress testing to diagnose coro and have a normal resting electrocardiogram,
nary artery disease, P-blockers should be withheld for although advanced imaging is indicated if the exercise
24 to 48 hours before testing. stress test is inadequate or indeterminate.
Bibliography Bibliography
Fihn SD, Gardin JM. Abrams J, et al; American College of Cardiology Fihn SD. Gardin JM, Abrams J. et al: American College of Cardiology
Foundation; American Heart Association Task Force on Practice Foundation/American Heart Association Task Force. 2012 ACCF/AHA/
Guidelines; American College of Physicians; American Association for ACP/ AATS/PCNA/SCAl/STS guideline for the diagnosis and management
Thoracic Surgery; Preventive Cardiovascular Nurses Association: Society of patients with stable ischemic heart disease: a report of the American
for Cardiovascular Angiography and Interventions: Society of Thoracic College of Cardiology Foundation/American Heart Association task force
Surgeons. 2012 CCF/AHA/ACP/AATS/PCNA/SCAI/STS Guideline for the on practice guidelines. and the American College of Physicians. American
diagnosis and management of patients with stable ischemic heart dis Association for Thoracic Surgery. Preventive Cardiovascular Nurses
ease: a report of the American College of Cardiology Foundation / Association. Society for Cardiovascular Angiography and Interventions.
American Heart Association Task Force on Practice Guidelines, and the and Society of Thoracic Surgeons. Circulation. 2012 Dec 18;126 (25) :e354-
American College of Physicians. American Association for Thoracic 47l. Erratum in: Circulation. 2014 Apr 22;129(16) :e463. [PMI D : 23166211]
Surgery. Preventive Cardiovascular Nurses Association. Society for
Cardiovascular Angiography and Interventions. and Society of Thoracic
Surgeons. J Am Coll Cardiol. 2012 Dec 18;60(24):e44-el64. [PMID:
23182125]
Item 40 Answer: c
Educational Objective: Manage cardiovascular risk in
an older woman with diabetes mellitus.
Item 39 Answer: B
This patient should start atorvastatin, discontinue simvas
Educational Objective: Manage a patient with new
tatin, and continue her other medications. The most recent
onset exertional angina pectoris.
cholesterol guidelines recommend a moderate- or high
This patient with an intermediate risk of ischemic heart dis intensity statin, such as atorvastatin, in patients aged 40 to
ease and an inadequate exercise electrocardiographic (ECG) 75 years with diabetes mellitus who have a 10-year cardio
stress test should undergo pharmacologic stress testing. vascular risk greater than or equal to 7.5%. This patient's
Although exercise ECG stress testing is the preferred diagnos 10-year cardiovascular risk is above 10%. A cardiovascu
tic study in patients with an indication for testing who have an lar risk calculator based on the Pooled Cohort Equations
interpretable resting ECG and are able to exercise, in patients for the purpose of managing cholesterol levels is ava ilable
who are unable to meet the minimal criteria for adequacy on at http: //my.americanheart.org/professional/Statements
this study (achievement of at least 85% of the age-predicted Guidelines/PreventionGuidelines/Prevention-Guidelines_
maximal heart rate and maximal metabolic demand) , addi UCM_ 457698_SubHomePage.jsp. In patients with diabetes
tional testing is indicated to appropriately evaluate for isch in this age group with a 10-year risk below 7.5%, a moder
emic heart disease. Dobutamine stress echocardiography or a ate-intensity statin (such as simvastatin 20-40 mg/d) would
vasoclilator nuclear medicine stress test would be appropriate be recommended. While there are multiple options for a
1 75
Answers and Critiques
high-intensity statin, the fact that atorvastatin has a generic tension in patients with PAD because of the possibility of
alternative makes it a more attractive choice. loss of p-receptor-mediated vasodilation causing worsening
I ncreasing simvastatin from 10 mg/d to 80 mg/d is claudication, this has not been supported by study data.
incorrect, as the FDA issued a black box warning against the Therefore, p-blockers may be used in patients with PAD for
use of simvastatin 80 mgld because of a heightened risk of blood pressure control. However, this patient's hypertension
muscle adverse effects. is wel l controlled, and P-blockade is not indicated as therapy
Continuing the patient's current medications is incor for claudication symptoms.
rect because this patient's cardiovascular risk warrants Clopidogrel or another thienopyridine should be added
change to a moderate- or high-intensity statin. to aspirin therapy in all patients following an acute coronary
The addition of clopidogrel to this patient's drug regi syndrome and in those undergoing coronary stent place
men is incorrect because dual antiplatelet therapy (such as ment. However, there is no benefit in adding clopidogrel to
aspirin plus clopidogrel) increases the risk of bleeding and is aspirin in patients with PAD for treatment of the vascular
not routinely recommended for patients for primary preven occlusion or reducing the risk of cardiovascular events.
tion of cardiovascular events. In the Warfarin Antiplatelet Vascular Evaluation (WAVE)
Despite a negative exercise stress test, aspirin therapy trial among patients with PAD, the combination of an oral
would be recommended in this woman who has several risk anticoagulant and antiplatelet therapy was not more effec
factors for cardiovascular events and stroke, including hyper tive than antiplatelet therapy alone in preventing major
tension, type 2 diabetes me!Jitus, hyperlipidemia, and her age. cardiovascular complications and was associated with an
In the Women's Health Study of 40,000 healthy women, 100 increase in life-threatening bleeding.
mg/d of aspirin decreased the risk of stroke, myocardial infarc
KEY POINT
tion, and cardiovascular death in patients older than 65 years.
• Cilostazol has been shown to be effective at improving
KEY POINT pain-free walking and overall walking distance in
• A moderate- or high-intensity statin is recommended patients with claudication.
for patients aged 40 to 75 years with diabetes mellitus
who have a 10-year cardiovascular risk greater than or Bibliography
equal to 7.5%. Pande RL. Hiatt WR. Zhang P, Hittel N. Creager MA. A pooled analysis of the
durability and predictors of treatment response of cilostawl in patients with
intermittent claudication. Vase Med. 2010 Jun:l5(3):181-8. [PMID: 20385711]
Bibliography
Stone NJ, Robinson JG, Lichtenstein AH. et al: American College of
Cardiology/American Heart Association Task Force on Practice
Cl
Guidelines. 2013 ACC/AHA guideline on the treatment of blood choles Item 42 Answer: E
terol to reduce atherosclerotic cardiovascular risk in adults: a report of
the American College of Cardiology/American Heart Association Task Educational Objective: Manage an accelerated idioven
Force on Practice Guidelines. J Am Coll Cardiol. 2014 Jul 1:63(25 Pt tricular rhythm following myocardial infarction.
8):2889-934. Erratum in: J Am Coll Cardiol. 2014 Jul 1:63(25 Pt 8):3024-
3025. [PMID: 24239923]
111is patient requires no further intervention at this time. She
developed a wide complex rhythm shortly after percutaneous
Item 4 1 Answer: B coronary intervention and reperfusion of her infarct-related
artery. The electrocardiogram (ECG) shows a regular wide
Educational Objective: Manage claudication with phar
complex rhythm at 92 min with no clearly discernible atrial
macologic therapy in a patient with peripheral arterial
activity findings consistent \.\'ith accelerated idioventricular
disease.
rhythm (AIVR) . A l \I R is postulated to result from abnor
The most appropriate treatment is the addition of cilostazol. mal automaticity in the subendocardial Purldnje fibers. It is
Cilostazol is an oral phosphodiesterase-3 inhibitor that has observed in up to 1 5°!.. of patients who undergo reperfusion
demonstrated increases in pain-free walking and overall of an infarct-related artery. 111e rate is almost always less
walking distance in patients with claudication in randomized than 120 min and usually less than 1001 min. �ost studies
clinical trials. Cilostazol is contraindicated in patients with have shown that it is a benign rhythm when it occurs within
heart failure or a left ventricular ejection fraction below 40%. 24 hours of reperfusion. ·n1is patient is tolerating the rhythm
This contraindication exists because cilostazol has a similar vvell and is already on a �- blocker for post-myocardial i n farc
pharmacologic action to the inotropic drugs milrinone and tion care: therefore. no intervention is required.
amrinone, which demonstrated increased mortality rates with Neither amiodarone nor lidocaine is indicated because
long-term use in patients with heart failure. In the absence of A IVR is a benign ventricular arrhythmia and usually does
heart failure, a therapeutic trial of cilostazol should be consid not recur. Studies of prophylactic lidocaine after acute cor
ered in all patients with lifestyle-1.imiting claudication. onary syndromes have demonstrated potential harm. and
Antihypertensive therapy is recommended for reduc amiodarone has been associated with decreased survival
tion of cardiovascular events in patients with peripheral after myocardial infarction.
arterial disease (PAD) . Although concern has been raised in Cardioversion is not indicated because Al\IR is a tran
the past regarding use of P-blockers for treatment of hyper- sient rhythm and. in this patient. it is well-tolerated.
1 76
Answers a n d Critiq u es
CJ reperfusion
CONT.
A I VR usua l ly ind icates successful (or at least part i a l )
and i s co nsidered a reversi bl e arrhyt h m ia .
K EY P O I N T
I mplantable cardioverter-defibri l l a tor ( !CD) placement is not • Anti-inflammatory therapy with aspirin or other
indicated a l t h is time given t he patienl·s recent revascular NSAIDs, such as ibuprofen, is indicated in patients with
ization and nature of t he arrhy t h m ia . I f t h e left ventricular acute pericarditis; when the pericarditis is associated
ejection fract ion remains low despite med ical t herapy. ! CD with myocardial infarction, only aspirin should be used
placement m ight be i n dicated in the fu ture. because other NSA!Ds can impair myocardial healing
and increase the risk of mechanical complications.
K EY P O I NT
• Accelerated idioventricular rhythm is a common Bibliography
complication following coronary reperfusion a n d Lotrionte M. Biondi-Zoccai G, lmazio M, et al. I nternational collaborative
does not require intervention when it occurs within systematic review of controlled clinical trials on pharmacologic treat
ments for acute pericarditis and its recurrences. Am Heart J. 2010
24 hours of reperfusion. Oct;l60(4):662-70. [PMID: 209345601
CJ Educational Objective:
Item 43 Answer: A should co ntin ue taking ticagrelor for l year. He also should
Treat a patient with acute cont i nue taking aspirin i ndeftni tely. T he American College of
pericarditis with high-dose aspirin. Cardiology American Hean Associat ion percutaneous coro
nary intervention ( PC ! ) guideli nes recommend at least l year
This patient should receive high-dose aspi ri n . He has acute
of dual anti platelet t herapy for patients undergoing PCI with
pericard i t is in the setting of a recent myoca rdial i n farct ion .
a drug-elut i ng stent ( DES) .
ll1e typical chest pa in , physical exa m i nation fi ndi ngs. and
A n t i pla telet d rugs i nd icated for patients who h ave
abnormal electrocardiogram (ECG) are a l l consistent with this
received a d rug- e l u t i ng stenl following an acute coronary
diagnosis. especially t he fi ndi ngs of concave upward ST-seg
syndrome are clopidogre l . t icagre lor. and prasugre l . In the
ment elevation and PR- segment depression in a l l leads. except
PLATe let i n h i b i t ion and pat ient Outcomes (PLATO) t r i a l .
aVR. on t he ECG. Anti-inflam matory t herapy with aspi1in or
t i cagrelor was found to b e superior Lo clopi dogrel i n reduc
other N SA ! Ds. such as ibuprofen. is indicated in patients with
ing the i ncidence of cardiovascular dea t h . myocardial in farc
acute pericard i tis. I n those whose pericarditis is associated
t io n . and st roke fol lowing an acute coronary syndrome.
with myocardi a l i n farction. such as t his pat ient. only aspirin
Dual a n t iplatelet t herapy for patients who have under
should be used because ibuprofen and other NSAI Os can
gone pl acement of a bare metal sten t fol lowing a n acute
i mpair myocardia l healing and increase t he risk or mechani
coronary syndrome i s ind icated for a t least 4 weeks and up
cal compl ications. ll1e a n t i-inflammatory medication should
to l yea r. Fol lowing placement o f a DES. h owever, a 30-day
be given i n relatively high doses to ach ieve an a n t i -in flamma
duration of t icagre lor i s insu fficient.
tory e f fect and t hen tapered slowly over 2 to 4 weeks lo reduce
Despite numerous studies about t he risk o f very late
t he risk of recurrent pericard i tis. Colchicine (0. 5- t .2 mg d)
stent t h rombosis. defined as the occurrence of stent t h rom
also has been shown to be effective as adjunctive t herapy to
bosis greater than I ye<Jr after placemen t . t here is no recom
anti-inflammatory agents i n patients with acute pericarditis.
mendation for l i fe l ong dual a n l iplatelet therapy in current
fw 1her red ucing t he risk of recurrent pericarditis and t reat
trea t ment guidel i nes.
ment fai lure. Colch icine is not recommended for pat ients
Stopping ticagrelor and starting clopidogrel is i nconect
with post-i n farction pericardi tis. Colchicine may be associ
because there is no i ndication to stop t icagrelor. an agent that
ated with gast rointestinal side effects. l iver tox icity. and bone
appears to be superior to clopidogrel i n i mproving cardiovascu
ma rrow suppression but is genera l ly well tolerated .
lar outcomes following an acute coronary syndrome. Cost and
N i t roglycerin is an e f fect ive t herapy for chest pain
adverse events (i ncluding bleeding. dyspnea. and bradycardia)
caused by myocardial ischem ia but is not e ffec t ive for symp
are t he most common reasons for discontinuation of ticagrelor.
toms caused by pericard i t i s.
G l u cocorticoids. such as pred n isone. are reserved for KEY POINT
patients w i t h con t ra i ndications to N S A I Ds or t hose w i t h • At least 1 year of dual antiplatelet therapy is recom
refractory acute pericarditis. primarily because t here is evi mended for patients undergoing percutaneous coro
dence t hat t heir use i s associated with an increased risk of
nary intervention with a drug-eluting stent (DES) ;
recurrent pericarditi s. As t h is pa tient has no apparent con
therapeutic options for agents to be taken with aspirin
t ra i nd ication to aspir i n u se. t reatment w i t h glucocorticoids
are clopidogrel, ticagrelor, and prasugrel.
i s not in d icated.
1 77
Answers a n d Critiq u es
1 78
Answers a n d Critiques
bundle branch block and her echocardiogram demonstrates mellitus presenting with stable angina pectoris not con
an akinetic left wall, both of which suggest CAD. Patients trolled with optimal medical therapy.
with heart failure and multiple risk factors or symptoms This patient with stable angina pectoris with symptoms that
of CAD should be evaluated by either a stress test or car are not adequately controlled on optimal medical therapy
diac catheterization. The reason to evaluate for CAD is that should undergo left heart catheterization for further evalu
revascularization by either percutaneous coronary inter ation and potential revascularization. Her myocardial perfu
vention (PCI) or coronary artery bypass graft surgery may sion imaging results are consistent with ischemic coronary
improve her left ventricular ejection fraction and reduce her artery disease (CAD) ; however, these findings alone would
symptoms of heart failure. Noninvasive exercise testing is not be an indication for left heart catheterization. In patients
often performed initially to provide information about the with stable angina pectoris, coronary revascularization has
possible presence of ischemic heart disease but also to assist not been shown to improve morbidity or mortality, and thus
in risk stratification and prognosis. Cardiac catheterization is not indicated in patients whose symptoms are able to
may be helpful in patients with suggestive findings on non be controlled with optimal medical therapy. However, in
invasive testing or may be an appropriate initial study in patients with coronary ischemia who fail to respond to ade
selected patients. quate antianginal therapy, such as this patient, coronary
Cardiac magnetic resonance (CMR) imaging is not part angiography is indicated to evaluate for possible revascular
of the routine evaluation of new-onset heart failure but ization to control her angina symptoms. Catheterization may
may be used if an infiltrative or an inflammatory process is allow for percutaneous intervention to address a coronary
suspected, such as myocarditis, hemochromatosis, Wilson occlusion leading to her angina symptoms, or assessment for
disease, or sarcoidosis. If the patient's evaluation for CAD the need for surgical revascularization if extensive or com
as a cause of her heart failure is normal and myocarditis is a plex CAD is present.
consideration, CMR imaging may be a reasonable test. CT angiography is an emerging technology for the non
Coronary artery calcium scoring is a method of mea invasive evaluation of the coronary arteries. Although it may
suring vascular calcification in the coronary arteries, be able to confirm the diagnosis of CAD in this patient, it
with increased levels of calcium being associated with an would not allow the opportunity for percutaneous coronary
increased burden of atherosclerotic plaque and cardiac intervention, if possible. The use of CT angiography to esti
events. Its optimal use may be in providing additional infor mate the need or benefit of coronary artery bypass grafting
mation for making therapeutic decisions in asymptomatic also has not been established. Therefore, this study would
patients at intermediate risk for atherosclerotic cardiovas not be indicated in this clinical setting.
cular disease. However, its role in evaluating patients with Dobutamine stress echocardiography is typically used
heart failure believed to be caused by CAD has not been to evaluate for ischemic CAD in patients who are unable to
established. exercise. However, in this patient with documented coro
Endomyocardial biopsy is indicated in patients with nary ischemia established by a nuclear medicine myocardial
heart failure that progresses despite medical therapy and perfusion study, there would be no benefit to performing
those with malignant arrhythmias to evaluate for giant cell this alternative diagnostic study for ischemia.
myocarditis, as well as in those in whom amyloidosis or Because this patient remains symptomatic with restric
hemochromatosis is suspected. Endomyocardial biopsy is tions on her quality of life, continuing her current medi
not indicated in this patient with evidence of heart failure in cal therapy without additional intervention would not be
whom CAD has not been evaluated. appropriate.
1 79
Answers and Critiques
Bibliography KEY P O I NT
Fihn SD, Gardin JM, Abrams J, et al ; American College of Cardiology
• In patients with mitral regurgitation, transthoracic
Foundation/American Heart Association Task Force. 2012 ACCF/AHA/
ACP/AATS/PCNA/SCAl/STS guideline for the diagnosis and manage echocardiography (TTE) is used to evaluate the
ment of patients with stable ischemic heart disease: a report of the
degree of m i t ral regurgitation and assess the causa
American College of Cardiology Foundation /American Heart
Association task force on practice guidelines. and the American College t ive valve abnormalities, t hereby providing essential
of Physicians. American Association for Thoracic Surgery, Preventive
information for guiding therapy; if visualization is
Cardiovascular Nurses Association. Society for Cardiovascular
Angiography and I nterventions. and Society of Thoracic Surgeons. inadequate with TTE, transesophageal echocardiog
Circulation. 2012 Dec 18: 126(25) :e354-471. Erramm in: Circulation. 2014
raphy is indicated.
Apr 22;129(16) :e463. lPM ID: 231 66211]
Bibliography
Item 49 Answer: D Solis J, Piro V, Vazquez de Prada JA. Loughlin G. Echocardiographic assess
ment of mitral regurgitation: general considerations. Cardiol Clin. 2013
Educational Objective: Use appropriate imaging to May:3l(2):165-8. [PMID: 23743069]
evaluate mitral regurgitation.
1 80
Answers a n d Critiq u es
Patients are encouraged to perform their normal activities a x i s 1 23 d egrees ) : howeve r. t h ere is a lso evidence or le f'l pos
while wearing the monitor. Patients keep a symptom diary terior l�1 scic u l a r bloc k (sm a l l r w:wes a n d deep S waves i n
or trigger a marker on the continuous reading that correlates leads I a n d ci \/ L: q R complexes in leads 1 1 . 1 1 1 . ci nd a \/ F) . T h us .
with symptoms. Ambulatory ECG monitors can also be use t hese fe<i t u res a re most consistent w i t h aberra n t cond u c t i o n
ful to detect asymptomatic arrhythmias, such as asymptom i n t he set t i ng of' a t ri a l f i bri l l a t ion ra t h e r t h a n ven tricu l a r
atic atrial fibrillation. This patient's symptoms occur about tac hyca rd ia .
once a week, and a 24- or 48-hour ambulatory monitoring I n t ravenous proc a i n a m i d e wou l d be t h e <1gen t ol' choice
period is likely to miss the symptomatic episodes. i f t h i s tachycard i a were pre-excited (Wol ff'- Pa rki nson -Wh i te
1 81
Answers a n d Critiques
intervention. CT angiography (CTA) i s rapid and easily available support t he use of an aldosterone a ntago nist such as epler
but requires the administration of intravenous contrast dye. enone. Based on the EPH ESUS ( Eplereno ne Post-A M I Heart
While CTA compares favorably with digital subtraction (inva Fai lure Effi cacy and Surviva l ) t r i a l . the 2007 American Col
sive) angiography for the detection of occlusive arterial disease, lege of Cardiology /American Heart Association gu idel i nes
imaging is not needed at this time because the patient does not recommend the a d m i nistra t ion of an aldosterone an tago
require surgical inte1vention. nist to all pat ien t s fol l owing a non- ST-elevation myoca rd i a l
Lower extremity segmental pressure measurement can i n fa rction (NSTEM I ) w h o a re receivi ng a n A C E i n h i bi tor,
help determine the level and extent of PAD. Using special have a left ven tricu lar ejection fraction of 40'Y., or below. and
ized equipment in the vascular laboratory, blood pressures have e i t her heart fai lure sym ptoms or d iabetes mel l itus.
are obtained at successive levels of the extremity, localizing ACE i nh i b i tors i nh i b i t postinfarction remodel i ng. hel p
the level of disease. Many vascular laboratories use air pleth i ng to preserve ven t ricular function . ACE i n h i bi tors shou l d
ysmography to measure volume changes within the limb, in b e cont i n ued indefi n itely.
conjunction with segmental limb pressure measurement.
KEY POINT
Lower extremity segmental pressure measurement is not
needed at this time because localization of disease is not • Long-term therapy following myocardial infarction
needed to guide therapy. such as would be required if surgi includes aspirin, a �-blocker, an ACE inhibitor, and a
cal intervention were being planned. statin; a P2Y 12 inhibitor (clopidogrel, prasugrel, tica
grelor) should be continued for at least 1 year for
KEY POINT
patients undergoing coronary percutaneous interven
• Patients with peripheral arterial disease should be tion with stent placement.
treated with a moderate- or high-intensity statin.
Bibliography
Bibliography Makki N, Brennan TM, Girotra S. /\cute coronary syndrome. J Intensive Care
Berger JS. Hiatt WR. Medical t herapy in peripheral artery disease. Med. 2013 Sep 18. [PMID: 24047692]
Circulation. 2012 Jul 24:126(4):491-500. [PMID: 228254 11]
Item 54 Answer: B
Cl Educational Objective:
Item 53 Answer: E Educational Objective: Treat heart failure with reduced
Choose appropriate antiplatelet ejection fraction with a �-blocker.
agents following acute coronary syndrome and percutane
This patient with a recent diagnosis of heart failure with
ous coronary intervention.
reduced ejection fraction (HFrEF) should be started on a
No changes should be made to this pat ient"s medicat ions at �-blocker, such as carvedilol. Standard therapy for patients
t he t i me o f hospital discharge. with HFrEF includes an ACE inhibitor and a �-blocker. This
Calcium chan nel blockers . wi t h t he except ion o f n i fed patient is already on an ACE inhibitor for treatment of his
i p i ne. can be used in patients with co n t ra i nd icat ions to blood pressure and for afterload reduction for his heart fail
�- blockers and i n t hose wi t h cont i n ued angina despi te opti ure. ACE inhibitors are typically started first in patients with
mal doses of �- blockers and n i t ra tes. l l 1 is patient has no heart failure because of their positive hemodynamic effects.
i n d i cations for a calcium channel blocker such as cl i l t iazem. An angiotensin receptor blocker (ARB) would be another
ll 1ere is no evidence t o support a change from t icagre treatment option, particularly if an ACE inhibitor were not
lor to clopidogrel a fter percutaneous coro n a ry i n tervention tolerated. A �-blocker should then be started in stable, euvole
(PCI) for acute coronary synd rome. In the PLATO ( PLATelet mic patients with heart failure, either at the time of diagnosis
i n h i bi t ion and pat ient Outcomes) t r i a l . t h e use of t i cagrelor or after acute decompensation is treated. �-Blockers have sev
was associated w i t h a 1 . 9"/., absolute risk reduct ion i n the eral beneficial effects and have been shown to prolong overall
occurrence o r cardiovascu lar dea t h . myocardial i n farct i o n . and event-free survival.
�nd stroke w h e n comp<Jrecl w i t h clopidogre l . A P2Y1:1 i n h i b The P-blockers that have been shown to provide benefit
itor (clopidogre l . prasugre l . t i cagrelor) should be con l i n ued in patients with HFrEF are metoprolol succinate, ca1vedilol,
for <Jt least I year for pat ients undergoi ng PCI with stent and bisoprolol. The P-blocker dosage should be increased
placement . slowly-at 1- to 2-week intervals- to the maximal dose.
Oral � blockers should be given to a l l pat ients w i t h acute Like ACE inhibitors, there are data that suggest improved
coronary syndrome w i t hout a con t ra i ndic<Jtion (decom outcomes on higher doses of �-blockers (increased ejection
pensated heart fai l ure, advanced a triovent ricular block. or fraction, reduced symptoms, lower mortality rates) ; there
severe reactive a i 1-vvays disease) and con t i nued incleAni tely. fore, attempting to up-titrate to maximally tolerated doses
ll1 i s pat ient is a l ready bradyca rd ic. and an i n c rease i n t h e is important.
dosage o f metoprolol may b e associated w i t h sym ptomatic Although dihydropyridine calcium channel block
bradyca rd ia. ers, such as amlodipine, are effective antihypertensive and
I n t h i s patient w i t h an acute coronary syndrome and antianginal medications, they do not provide the same ben
preserved left ven t ricular function. t here i s no evidence to efits as ACE inhibitors, ARBs, or P-blockers, and would not
1 82
Answers and Critiq u es
be appropriate add-on therapy in this patient who is not tion: however. the presentation generally is characterized
currently on a P-blocker and has controlled blood pressure by acute dyspnea and pulmonary edema rather than platy
without angina. pnea-ort hodeox ia . Physical exa mination fi ndi ngs include a
This patient has clear lungs, no significant jugular systolic murmur at t he apex that increases during expiration
venous distention, and no peripheral edema. He has no rather t han inspiration.
evidence of volume overload and therefore does not need Severe left ventricular systolic dysfunction generally
a diuretic, such as furosemide. Diuretics have no mortality does not cause arterial oxygen desaturation. In addition. the
benefit and are only used for symptom relief in the setting initial assessment of left ventricular function was normal.
of volume overload. ·n1e clin ic<Jl picture in this patient is more compatible with
Spironolactone has been demonstrated to decrease right ventricu lar dysfunction.
mortality rates in patients with New York Heart Associa Ven t ricular septa! defect is a recognized complication
tion (NYHA) functional class II to IV heart failure (dyspnea a fter transmural myoca rd ial in farction; however, the pre
with activities of daily Living) . 1his patient has good exer senta t ion generally includes acute dyspnea and pulmonary
cise capacity and has NYHA class II heart failure. However, edemJ rather than ox')'gen desaturation. The left-to-right
candidates for spironolactone therapy should already be on shunt associated with the ventricular septa! defect causes
standard medical therapy, including an ACE inhibitor and a left heart volume overload. rather than the right heart vol
P-blocker. ume overload caused by right-to-left shunting seen in this
Making no changes in this patient's treatment regimen patient. Physical examination findi ngs in patients with ven
would not be appropriate because he is not being treated t ricular septa! defect following myocardial i n farction include
with medications associated with improved outcomes in a holosystolic murmur at the left sternal border that does not
patients with systolic heart failure. change with respirat ion .
Bibliography Bibliography
Parikh R, Kadowilz PJ. A review of current therapies used in the treatment Kubler P, Gibbs H. Garrahy P Platypnoea- orthodeoxia syndrome. Heart.
of congestive heart failure. Expert Rev Cardiovasc Ther. 2013 2000 Feb;83(2):221 -3. [PMID: 10648502]
Sep;ll(9):ll71-8. [ PMID: 23980607]
Item 5 6 Answer: A
Cl Ed ucational Objective:
Item 55 Answer: A Educational Objective: Manage heart failure with car
Diagnose platypnea diac resynduonization therapy.
orthodeoxia syndrome.
1his patient with symptomatic heart failure and a reduced
ll1e most likely diagnosis in this patient is a patent foramen left ventricular ejection fraction with evidence of significant
ovale with right-to-left shunt. He presents with features of conduction system disease should undergo placement of a
platypnea-orthodeoxia syndrome. characterized by posilionaJ biventricular pacemaker (cardiac resynchronization therapy
symptoms of cyanosis and dyspnea that generally occur when [CRT]) . He has progressive heart failure symptoms while on
the patient is sitting and resolve in the supine position. Right appropriate medical therapy and has New York Heart Associ
to-left shunting across an atrial septa! defect or patent fora ation (NYHA) functional class III symptoms. With his ejection
men ovale may rarely cause cyanosis and dyspnea owing to fraction less than 35% and left bundle branch block (LBBB) ,
defom1ation oft he atrial septum and redirection of shunt flow he is a candidate for a biventricular pacemaker, which has
that result from increased right atrial pressure in the upright been demonstrated to reduce mortality and symptoms in
position. ll1is patient had an i n ferior and right ventricular patients with NYHA functional class III and IV heart failme
myocardial infarction with associated right heart enlargement by improving cardiac hernodynamics. The 2013 American
and dysfunction and clinical features of hypotension. T he College of Cardiology Foundation/American Heart Associa
right hean enlargement causes a n nular d i latation and t ricus tion/Heart Rhythm Society(ACCF/AHA/HRS) guideline rec
pid regurgitation . The foramen ovale stretches and becomes ommends CRT therapy in patients with an ejection fraction
patent. ll1e preferential cyanosis is caused by the hemody of 35% or below, NYHA functional class III to IV symptoms
namic alterations and preferential t ransfer of right atrial blood on guideline-directed medical therapy, and LBBB with QRS
across the patent foramen in the upright position. duration greater than or equal to 150 ms. ·n1is patient already
Mitra! regurgitation due to papillary muscle injury or has an implantable cardioverter-defibrillator, which is indi
rupture is a recognized complication after myocardial i n fa rc- cated for patients with NYHA functional class II to Ill heart
1 83
Answers a n d Critiques
failure and an ejection fraction less than 3 5 % . Now that he has are present (left bundle branch block [LBBB] , left ventric
developed a LBBB and an increase in symptoms, it is reason ular hypertrophy, paced rhythm, Wolff-Parkinson-White
able to proceed with placement of a biventricular pacemaker pattern) , results may be indeterminate. This patient has
as well. none of these conditions, and therefore exercise stress
lnotropic therapy, such as dobutamine, is reserved for testing is a reasonable option. In patients who can exercise,
patients with end-stage heart failure, either as a bridge to exercise stress is preferred to phannacologic stress because
transplantation or for palliative care. Patients in this cate of the functional and prognostic information exercise stress
gmy often have recurrent hospitalizations for heart failure, provides. Persons who can exercise have a better prognosis
have evidence of end-organ compromise such as worsening than those who are unable to exercise and require pharma
kidney and liver function, and have very poor exercise tol cologic stress testing.
erance. Although this patient has progressive symptoms, Among patients with resting ECG abnormalities that
he has not reached this stage yet, and has no indication for limit ST-segment analysis, the addition of imaging aids diag
inotropic therapy. nostic accuracy and provides improvement in localizing the
The patient has no evidence of volume overload on site and extent of ischemia. In patients with LBBB, exercise
examination and a borderline low blood pressure; there stress may result in abnormal septa! motion due to conduc
fore, increasing his diuretic dose would not be expected to tion delay with falsely positive septa! abnormalities; this
improve his symptoms and may worsen them by lowering abnormality is lessened with use of vasodilator (such as
his cardiac filling pressures and cardiac output. adenosine) stress imaging. This patient does not have ECG
The patient is fairly symptomatic but has not yet had abnormalities that warrant adenosine myocardial imaging
optimal therapy, as he has an indication for CRT and has not study and the added expense and radiation exposure that
yet received it. Left ventricular assist devices (LVADs) are this procedure would require.
reserved for patients with end-stage refractory heart failure Cardiac magnetic resonance (CMR) imaging can be
as a bridge to heart transplantation or as destination therapy used to evaluate aortic pathology, pericardia! diseases, and
in selected patients who are not candidates for transplan myocardial diseases, as well as to evaluate the extent of myo
tation. However, prior to being considered for either an cardial fibrosis. CMR imaging may be useful in determining
LVAD or heart transplantation, a patient must be on optimal the extent of myocardial infarction and potential viability.
medical therapy. This patient is asymptomatic; therefore, CMR imaging is not
indicated.
KEY POINT
CT angiography a llows determination of the pres
• Cardiac resynchronization therapy is recommended ence and extent of coronary artery d isease. I f this
in patients with an ejection fraction of 35% or below, i ntermediate-risk patient was unable to exercise or
New York Heart Association functional class III to IV the ECG was uninterpretable, CT a ngiography could be
symptoms on guideline-directed medical therapy, and performed. I f, however, obstructive disease was found,
left bundle branch block or QRS duration of 150 ms or the patient would then need to undergo coronary angi
greater. ography to perform a percutaneous intervention, thus
performing two procedures that require contrast agents
Bibliography and radiation exposure.
Epstein AE. Di Marco JP. Ellenbogen KA, et al; American College of Cardiology For patients unable to exercise because of physical lim
Foundation; American Heart Association Task Force on Practice
itations or physical deconditioning, pharmacologic stressors,
Guidelines; Heart Rhythm Society. 2012 ACCF/AHA/HRS focused update
incorporated into the ACCF/AHA/HRS 2008 guidelines for device-based such as dobutamine, can be used. These agents, which are
t herapy of cardiac rhythm abnormalities: a report of the American recommended if the patient cannot achieve at least five
College of Cardiology Foundation/American Heart Association Task
Force on Practice Guidelines and the Heart Rhythm Society. J Am Coll metabolic equivalents, increase myocardial contractility and
Cardiol. 2013 Jan 22:61 (3):e6-75. [PMID: 23265327] oxygen demand. This patient can exercise, and dobutamine
stress echocardiography is not indicated.
Item 5 7 Answer: E KEY POINT
Educational Objective: Evaluate a patient with cardio • Exercise electrocardiographic testing is the standard
vascular risk factors and atypical chest pain and a normal stress test for the diagnosis of coronary artery disease
resting electrocardiogram.
in the absence of conditions that limit ST-segment
1he most appropriate diagnostic test for this patient is analysis.
exercise stress testing. He has an intermediate pretest prob
ability of coronary artery disease (CAD) based on his age, Bibliography
sex, and symptoms. He should undergo stress testing to American College of Cardiology Foundation Appropriate Use Criteria Task
Force: American Society o f Echocardiography; American Heart
determine if his symptoms are related to obstructive CAD. Association: American Society of Nuclear Cardiology; Heart Failure
Exercise electrocardiographic (ECG) testing is the standard Society of America; Hean Rhythm Society; Society for Cardiovascular
Angiography and Interventions; Society of Critical Care Medicine;
stress test for CAD diagnosis in patients with a normal Society of Cardiovascular Computed Tomography; Society for
baseline ECG. If abnormalities limiting ST-segment analysis Cardiovascular Magnetic Resonance, Douglas PS. Garcia MJ. Haines DE.
1 84
Answers a n d C ritiques
Bibliography
Cl Ed ucational Objective:
Item 58 Answer: A
Baddour LM. Cha YM. Wilson WR. Clinical practice. Infections of' cardiovas
Manage infection of an cular implantable electronic devices. N Engl J Mee!. 2012 Aug 30:
implanted electronic cardiac device. 367(9):842- 9. Erratum in: N Engl J Med. 2012 Oct I I ; 367(15): 1474. N Engl
J Med. 2012 Sep 27:367(13): 1272. [PMID: 22931318]
T l 1 i s pa t i ent has signs a nd symptoms conce rn i n g !or possi
ble i n fect ion of an i m p l a n ted cardiac elec t ro n i c device. He
should undergo laboratory eva l u a t ion i nclud i ng assess ment
Item 59 Answer: B
of' a complete blood cou n t with d i f frre n t i a l . two peripheral Ed ucational Objective: Manage asymptomatic moder
blood cul tures from separate ph lebotomy sites . and an e ryth ate aortic regurgitation.
rocyte sed i mentat ion rate to assess for t he possibi l i ty or a
This patient with moderate aortic regurgitation should be
device- related i n fect io n .
reassessed clinically in 1 year. Patients with moderate aortic
Pa t i e n t s w i t h a n i m p l a nte d ca rd i a c dev ice can deve l o p
regurgitation should be evaluated on a yearly basis and echo
e i t h er a l o ca l i zed t i ssue i n fe c t i o n a t t he i m p l a n t s i t e
cardiography performed every 1 to 2 years.
( pocket i n fect i o n ) o r a system i c i n fe c t i o n w i t h bactere m i <l
Aortic valve replacement is indicated for symptomatic
( to r ex a m p l e. e n d oca rcl i t i s ) . T h ese i n f e c t i o n s can occ u r
patients with chronic severe aortic regurgitation irrespec
a ft e r i n i t i a l i m p l <rn ta t i o n . l a t e <lfter i m p l a n t a t i o n . o r fol
tive of left ventricular ( LV) systolic function, asymptomatic
l ow i ng a device b a t tery re p l acement or rev i s ion . I f' l e f't
patients with chronic severe aortic regurgitation and LV
u n t re a ted . i m p l a n ted ca rd i ac device i n fec t i o n s w i l l prog
systolic dysfunction (LV ejection fraction :<:;SO%) , and patients
ress to e n doca rd i t is a ncl seps i s a n d u l t i ma t e l y dea t h . 'J h e
with chronic severe aortic regurgitation undergoing coro
fa t a l i ty ra t e fo r an u n t reated device i n fe c t i o n a p p roaches
nary artery bypass graft (CABG) or surgery on the aorta or
75% to 100% . A n l i b i o t i c t h erapy a l o n e is i n su f f i c i e n t .
other heart valves. TI1is patient is not a candidate for aortic
C ura t i ve t he rapy req u i res a n t i b i o t ic t h erapy a n d comp lete
valve replacement.
h a rdware remova l .
Endocarditis prophylaxis is not recommended for
T h e most common pat hogens a re coagulase n eg<l l i ve
patients with bicuspid a011ic valves in the absence of another
Staphy lococcus species a n d S. a u re us. Pat ie n t s m,1y prese n t
speciAc indication such as a prior episode of infective endo
w i t h fever or m <1 l aise: many a lso have loC<ll f- i n d i ngs sugges
carditis, previous valve replacement, prior cardiac trans
t i ve of' i n fect i o n . such as ery t h em<l or vva rmt h a t t h e i m p l a n t
plantation with valvulopathy, and certain forms of complex
s i t e. 'J hese p a t i e n t s s hou lcl u n dergo a laboratory eva l u a t i o n
congenital heart disease.
for s i g n s of' i n lec t i o n . E l evated ery t h rocyte sed i me n t a t i o n
Medical therapy for chronic aortic regurgitation is
rate. Jeu kocytosis w i t h a left s h i r t . a n d <rne m i a a rc suggest ive
limited. ACE inhibitors or angiotensin receptor blockers
of' i n fect ion . All pa t ie n t s w i t h poss i b l e device-related i n fec
may be used in patients with chronic severe aortic regur
t i o n ( w i t h or w i t hout fever) s h o u l d have a m i n i m u m of two
gitation and heart failure as wel l as in patients with aortic
b lood c u l t u res d rawn f'rom separate s i tes. Once t here is sus
regurgitation and concomitant hypertension, but these
p i c i o n tor a device i n lec t i o n , re lerral to the patient ' s electro
agents, as well as dihydropyridine calcium channel block
physiologist or an i n lecrious d i sease spec i a l i s t is manda to ry.
ers, have not been shown to delay surgery in asymptomatic
Pacem<1ker pocket aspi ra t i on s h o u lcl never be per
patients without hypertension. There is no established
lormed . as it can seed a steri le pocket a n d lead to i n fect i o n .
benefit in medical therapy for this patient with moderate
espec i a l ly i f . t here i s superAci<l l cel l u l i t i s w i t hout deeper
aortic regurgitation without other specific indications for
t i ssue i nvolveme n t .
treatment.
U l t rns o n ogra p h i c ex a m i n a t io n or <I pace m a ker o r
d e fi b r i l l a t o r pocket mJy be h e l p f'ul in p a t i e n t s w i t h sus KEY POINT
pected i rn p Lrnted card i::ic device i n lect ion s. I Jowevcr. pocket • Asymptomatic patients with moderate aortic regurgi
f l u i d may not a lways represe nt i n lect i o n . <ll1cl steri l e sero mas tation should be evaluated on a yearly basis and have
a re someti mes en cou n tered . Tl1erefore. pocket u l t rnsou n d echocardiography performed every 1 to 2 years.
h < l S I i m i ted - i r a ny -d iagnost ic va l ue.
::ven
1 t h ough t h e prese n t i ng sy m pt o m s or a dev i ce
Bibliography
re l a ted i n le c t i o n may be n o n spec i l i c a nd d ifficult to
Helms AS, Bach DS. Heart valve disease. Prim Care. 2013 Mar:40(t) : 9 1 - 108.
d i s t i ng u i s h rro m ot her com m o n . ben ign i n le c t i o n s . d e l ayed [PMID: 23402463]
1 85
Answers and Crit i q u es
Item 60 Answer: A emergency PCI. ·n1 rombolytic t hernpy l�1 i l ure. w h ich occurs
in up to 30% or patienrs. remains d i l f ic u l t to diagnose. Chest
Educational Objective: Manage anticoagulation therapy
pain resol u t i on . ST- segme n t elevat ion i m p roveme n t . a n d
in a pregnant woman with a mechanical valve prosthesis.
reperrusion a rrhyt h m ias (most co m mo n ly a n acce lerated
The anticoagulation regimen that will provide the greatest i d i ove n t ri c u l a r rhy t h m ) i n d icate success fu l L h rombo ly
protection against thromboembolism in this patient is warfa sis. A l t hough complete ST- segment elev•ll i o n resol u t ion is
rin therapy. Low-dose aspitin therapy should also be contin associated w i t h coron<iry p<itency. il occurs in a m i nority
ued. Women with mechanical valve prostheses cany a high o f P•l l ien ts. I m provement i n ST- segment elevation greater
tisk of valve thrombosis, bleeding, and fetal morbidity and t h a n SO% on a n electrocardiogram ( ECG) obta i ned 60 m i n
mortality duting pregnancy, and the optimal anticoagulation utes alter t h e :.id rn i n is l ra t ion or t h romboly t ic t herapy i s t h e
strategy has not been established. Options i nclude warfa1in , most com mo n ly used criterion l o ind icate successfu l reper
unfractionated heparin (UFH) , a n d low-molecular-weight fusio n. Cont i nued chest pain . lack of i m p rovement in ST-seg
heparin (LMWH). Although warfarin poses an increased risk menl eleva t i o n . hemodyna m i c i nstabi l i ty. a n d t he absence
of teratogenicily and fetal loss, it appears to be the most effec o r reperfusion a rrhy t h m ias most l i kely i n d icate l�1 i l ure or
tive option for reducing thromboembolism tisk in the mother. L h romboly t i c t herapy and ind icate the need !or rescue PC I .
1he current dose ofwarfatin (4 mg/cl), used to achieve a thera 1 h is pa t ient has clear evidence or t a i led reperfusion o r reoc
peutic INR, is associated vvith a low risk of warfarin embryop clusion (worseni ng or ST - seg me n t eleva t i o n . persistence or
athy and a low risk of fetal complications. sympto ms) and now has signs o f cardiogenic shock ( low
TI1e novel oral anticoagu lants, such as dabigatran, blood pressure. p u l monary edema ) . I n pat ients with t h rom
bivalirudin, rivaroxaban, and apixaban, do not adequately bolyl ic l herapy f a i l u re. guideli nes recom mend i m mediate
protect patients with mechanical valve prostheses against t ransfer tor rescue P C I . In m u l t iple t ri a ls or t h rornbolytic
thromboembolism and should not be used in pregnant or t he rapy fa i l u re. patients who underwent rescue PCI had a
nonpregnant patients with mechanical valve prostheses. sign i ficant i m p rovement in t h e rate or rei n f a rction when
I ntravenous UFH is the anticoagulant treatment of co mpared with t hose receiving conservat ive care. b u l no
choice around the time of del ivery. Intravenous UFH can i m p rovement i n mort a l i ty.
also be used during the first trimester. TI1e dose effect must 'n1e use or g lycopro t e in l i b I l la i n h i bi tors has been
be measured by activated partial thromboplastin time and tested i n m u l ti p l e scenarios i n pa t ients w i t h STE M ! . Based on
the dose adjusted to a therapeutic level. Fixed-dose subcuta t hese stud ies. t he i r use has been l i m i ted ow i ng lo excessive
neous UFH may not provide adequate anticoagulation. bleed i ng events. In pat ients i n whom t h romboly t i c t herapy
LMWH can be used as an anticoagulant during preg has !ai led. rescue PCI w i t hout the use of a glycoprolein I l b
nancy, but for patients with a mechanical valve prosthesis, Illa in h i bitor o r addi t i onal L h romboly t i c agents i s prelerred.
a weight-based regimen has been demonstrated to be inad A meta -a nalysis p u b l i shed i n 2007 co mpared repeat
equate. The LMWH dose must be adjusted to anti -factor Xa t h rombolytic t herapy w i t h con ervative t h e rapy in pa t i ents
activity in order to provide adequate anticoagulation. i n whom i n i t ia l t h rombolyt ic t he rapy fa i l ed. T h is analysis
KEY POINT showed no sign i ficant difference i n mort a l i ty rates or re i n
t a rc t ion between the two groups, a n d ou tcomes in t h ese
• Anticoagulation strategies for pregnant women with a
groups were i n ferior to rescue PC! .
mechanical valve prosthesis include warfarin, dose
adjusted unfractionated heparin, and dose-adjusted K EY P O I N T
low-molecular-weight heparin; of these options, war • Patients with thrombolytic therapy failure following
farin poses a lesser risk of maternal thromboembo an ST-elevation myocardial infarction should be
lism but a greater risk of fetal embryopathy. immedia tely transferred for rescue percutaneous cor
onary intervention.
Bibliography
Nishimura RA, Otto CM. Bonow RO. et al: American College of Cardiology/ Bibliography
American Heart Association Task Force on Practice Guidelines. 2014
Sutton AG. Campbell PG. Graham R, et al. A randomized trial of rescue
AHA/ACC guideline for the management of patients with valvular heart
angioplasty versus a conservative approach for failed fibrinolysis in
disease: executive summa1y: a report of the American College of
ST-segment elevation myocardial infarction: the Middlesbrough Early
Cardiology/A merica n Heart Association Task Force on Practice
Guidelines. J Am Coll Cardiol.2014 Jun 10;63(22):2438-88. Erratum in: J
Revascu la rizat i on to Limit lNfarction (MERLI N) trial. J Am Coll Cardiol.
2004 Jul 21 :44(2):287-96. [PM ID: 152619201
Am Coll Cardiol. 2014 Jun 10:63(22):2489. [ PMID: 24603192]
Cl Item 61 Answer: D
Educational Objective: Treat a patient with thrombo
Item 62 Answer:
Educational Objective: Screen patients with a family
c
lytic failure following an ST-elevation myocardial infarction. history of hypertrophic cardiomyopathy for the disease at
appropriate intervals.
1 l1 is patient w i t h ST-elevation myocard ial in f a rction (STEM I )
should b e t nrn s ferrecl to t h e nearest hospital \Ni t h p r i m a ry TI1 is patient should again be screened for hypertro
percutaneous coronary i n tervent ion ( PC I ) capabi l i t ies !or phic cardiomyopathy (HCM) i n 5 years. All first-degree
1 86
Answers and Criti q u es
l11ese recommendations are for relatives of patients t h e p a t i e n t develops he mocly n a m i c a l ly signi fica n t brady
with HCM in whom genetic testing is negative, inconclusive, c a rd ia . dop<1 111 i n e i n fusion cou l d be used to stabi l i ze h i m
or not performed. Genetic testing of probands can be used u n t i l coro n a ry reperfusion a n d tem porary paci n g cou l d be
to identify pathologic mutations, which can then be used acco mpl ished .
to screen family members and, if negative, may obviate the Aclva ncecl a l riove n t ricu l a r block i n t h e se t t i ng o f' a n
need for continued imaging. l11e yield of genetic testing, acute coronary syndrome of'Len req u i res tempora ry or per
which can be costly, varies according to the phenotypic m a n e n t paci ng. I n t h is patient. tem porary paci ng is not i ncl i
expression and familial nature of HCM. Thus, referral to a cated because he is hemody n a m i ca l ly stable a n d h i s block
cardiovascular specialist or a genetic counselor is recom is not advanced . Decisions o n perm a ne n t paci ng should be
mended for clinical decision-making based on genetic test de layed u n t i l a patient has been revascu la rized a n d st<lb i l i zecl
• All first-degree relatives of patients with hypertrophic • Patients with acute coronary syndrome and related
cardiomyopathy should u ndergo screening for the Mobitz type 1 second-degree atrioventricular block ·
disorder with a comprehensive physical examination, should undergo urgent reperfusion therapy as the
electrocardiogram, and echocardiogram; lifetime treatment of choice for this conduction block.
screening of those in whom the disorder has not yet
been diagnosed is indicated. Bibliography
Epstein AE, DiMarco JP, Ellenbogen KA, et al; American College of
Cardiology/American Heart Association Task Force on Practice
Bibliography Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002
Gersh BJ, Maron. BJ, Bonow. RO, et al; American College of Cardiology Guideline Update for Implantation of Cardiac Pacemakers and
foundation/ American Heart Association Task Force on Practice Antiarrhythmia Devices): American Association for Thoracic Surgery:
Guidelines. 2011 ACCF/AHA Guideline for the Diagnosis and Treatment Society of Thoracic Surgeons. ACC/AHA/HRS 2008 Guidelines for
of Hypertrophic Cardiomyopathy: a report of the American College of Device-Based Therapy of Cardiac Rhythm Abnormalities: a report of the
Cardiology Foundation/American Heart Association Task Force on American College of Cardiology/American Heart Association Task Force
Practice Guidelines. Developed in collaboration with the American on Practice Guidelines (Writing Committee to Revise the ACC/AHA/
Association for Thoracic Surgery, American Society of Echocardiography, NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers
American Society of Nuclear Cardiology. Hearl Failure Society of and Antiarrhythmia Devices) : developed in collaboration with the
America. Heart Rhythm Society, Society for Cardiovascular Angiography American Association for Thoracic Surgery and Society of Thoracic
and I nterventions, and Society of Thoracic Surgeons. J Am Coll Cardiol. Surgeons. Circulation. 2008 May 27;117(2l) :e350-408. Erratum in:
2011 Dec l3;58(25):e212-60. [PMID: 22075469] Circulation. 2009 Aug 4: 120(5):e34-5. [PMID: 18483207]
1 87
Answers and Critiques
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� ��
Item 65 Answer: D
Cl
Educational Objective: Manage coronary artery disease Item 66 Answer: D
risk in an asymptomatic patient with diabetes mellitus. Educational Objective : Manage acute limb ischemia.
This patient should continue his current therapy; no addi ll1c most appropriate ma nagem ent !or t his pa t i ent with an
tional testing is indicated at this time. The leading cause of ische m ic but viable e x t re m i ty (severe acute l i mb ische m i a )
1 88
Answers a n d Critiques
c::J is u rgent a ngiograp hy to defi ne t he anatomic level o f oc lu c to moderate LY dysfunction (ejection fraction of 30%-60%
s 1 o n and assess appropriate treat ment options. wh ich may and/or LY end-systolic dimension ;:::40 mm). Mitral valve repair
CONT
include su rgica l or percutaneous revascularizal ion or t hrom is the operation of choice when the valve is suitable for repair
bolytic therapy in se l ected pat ients. He has several risk factors and appropriate surgical skill is available and is recommended
lor at herosclerotic peripheral arterial d isease ( P A D) . a nd the for most patients. Mitra] valve replacement, especially with
claudicat ion t hat he has experienced for t he past year has chordal preservation, is appropriate for patients with severe
progressed to severe rest ing l i mb pain. ll1e limb i s v ia bl e as mitral regurgitation in whom the valve is not repairable or a less
indicated by t he presence or pa in . slow but present capi l la ry than optimal result would be obtained. Recently, a percutane
refi l l. and t he presence o f Dopple r vascular s ig nals. Acute
'
ously placed mitral valve clip has been introduced that is placed
ischemia can be caused by remote embol izat ion but may also to better approximate the edges of the anterior and posterior
resul t from in-situ t h rombosis. Because of' t h is. ant icoagu la leaflets of the valve and may be a therapeutic option in patients
t ion is crucial once a d i agn o s is of acute a rterial occlusion has who are at a prohibitive risk for m.itral valve surgery.
been made by h i s tory and physica l exam ination. The next step Yasodilator therapy, such as with ACE inhibitors or
in management is to fu r t her eva l uate t he l i mb ischemia and angiotensin receptor blockers, has not been shown to
plan fOr t reatment. Digi tal subt raction angiogra p hy provides improve outcomes in patients with severe mitral regurgita
the most hel p fu l i n formation and is t he p re f erred i maging tion who are asymptomatic. Additionally, vasodilator ther
modal i ty for acute l i m b i sc h e m i a : d e l ayi ng angiography could apy may mask the development of more severe left ventric
lead to l i m b necrosis a nd loss of' limb funct ion ing. ular dysfunction due to regurgitant volume. Therefore, these
Ca t he te r- d i re ct e d t h ro m bo ly t i c therapy may be a n agents should not be used as a substitute therapy for surgery
option i n some p a t i e nt s with acute l i m b ischem ia w i t h a when the patient is thought to be a surgical candidate.
viable o r ma rg i na l ly t h reatened l i m b as an a l ternat ive to a Mitra! balloon valvuloplasty or valvotomy is indicated
surgical approach . part icularly i r t he duration or acute l i m b for patients with severe mitral stenosis in whom there is a
ischem ia is less t ha n I day. However. ini t i a t i ng t h ro mbo reasonable likelihood of success and in whom there are no
l y l i c t he ra py in t h is patient before furt her evaluat ion of' t h e contraindications (such as moderate to severe mitral regurgi
nature o f the occ l u s i o n wou l d not be appropria te. tation or left atrial thrombus) . TI1is patient has severe mitral
for a nonviable extrem i ty. su rg ica l a m putation wit hout regurgitation, and repair, not valvotomy, is indicated.
a ngiography is i n clica t ecl because or t h e i ncreased risk or Serial echocardiography may be helpful in follow-up of
t issue necrosis and i n fect i o n . I l owevc r. t h is pat ien t's loot the asymptomatic patient in whom worsening of LY systolic
shows evidence of' viabi l i ty. mak i ng i m med i,1te amputation function or increase in chamber size may help facilitate deci
i na pp ro p r i a t e
. sion for surgery. This patient's LY function is compromised
Warfarin has not been shown to be an ef lecl ive t herapy and intervention is indicated.
lor managing stable Pr\ D. and a l t hough a n ticoagu lat ion is
K EV P O I N T
ind icated in ma naging acute l i mb ischemia pend ing fur
t he r eva l ua t i o n . i n i tiat ion of' long-term ant icoagulation with • Mitra! valve repair is the operation of choice for severe
wa rfarin i n t h is p<1 l iem with a viable but t h reatened l i m b mitral regurgitation when the valve is suitable for
w i t hout fu rt her intervention wou l d not b e a p pro pria te .
repair.
KEV POINT
Bibliography
• Patients with an ischemic but viable extremity on clin Nishimura RA, Oito CM, Bonow RO. et al: American College of Cardiology/
ical examination should undergo urgent angiography American Heart Association Task Force on Practice Guidelines. 2014
AHA/ACC guideline for the management of patients with valvular heart
to plan surgical or percutaneous revascularization. disease: executive summary: a report of Ihe American College of
Cardiology/American Hean Association Task Force on Practice
Guidelines. J Am Coll Cardiol. 2014 Jun 10;63(22):2438-88. Erratum in: J
Bibliography Am Coll Cardiol. 2014 Jun 10:63(22):2489. [PMID: 24603192)
Creager MA. Kaufman JA. Conte MS. Clinical practice. Acute limb ischemia.
N Engl J Med. 2012 Jun 7:366(23):2198-206. [PMID: 2267090S l
Item 68 Answer: c
Item 6 7 Answer: D Educational Objective: Manage Brugada syndrome.
Educational Objective: Manage asymptomatic severe
This patient should undergo placement of an implantable
mitral regurgitation with reduced left ventricular function.
cardioverter-defibrillator (!CD). He had an episode of abrupt
Mitra] valve repair is the most appropriate option for this patient syncope that is concerning for a cardiac etiology, specifically
with asymptomatic severe mitral regurgitation and moder an arrhythmia. He has a structurally normal heart on echo
ate left ventricular (LY) dysfunction. Surgery is indicated for cardiogram, but his electrocardiogram (ECG) shows right
patients with symptomatic acute severe mitral regurgitation, bundle branch block and 2-rnm ST-segment elevation in the
those with symptomatic chronic severe mitral regurgitation precordial leads. These findings are consistent with a type 1
with LY ejection fraction greater than 30%, and asymptom Brugada pattern (coved or descendant ST-segment elevation
atic patients with chronic severe mitral regurgitation and mild followed by negative T waves) on ECG. The presence of a type
1 89
Answers and Crit i q u es
1 Brugada pattern and symptoms (cardiac syncope) or vent1ic pitalizations, poor kidney function, diuretic dependence to
ular arrhythmia is diagnostic for Brugada syndrome. Brugada maintain fluid balance, and hypotension. Tiie two possible
syndrome can be genetically heterogeneous, but it is often options for therapy in a patient with this degree of heart
caused by mutations in the SCNSa sodium channel, which failure are placement of an LVAD and heart transplantation.
are believed to cause alterations in the ventricular refracto1y Because of his diagnosis of disseminated prostate cancer,
period and are responsible for the characteristic ECG findings however, the patient is not a candidate for transplantation.
and predisposition to sudden cardiac death. LVADs are indicated either as a b1idge to hea11 transplantation
Because this patient with Brugada syndrome is at risk or as destination therapy in selected patients who are not can
for sudden cardiac death, particularly given his recurrent didates for transplantation. Newer LVAD devices are smaller
episodes of near-syncope and syncope, an !CD should be and easier to maintain than earlier versions. making their
implanted. Patients with precordial ST-segment abnormal long-tenn use as destination therapy possible. Although this
ities should be referred to a cardiologist or electrophysiolo patient might otherwise be a candidate for transplantation,
gist. Once Brugada syndrome is diagnosed, first-degree fam his diagnosis of disseminated prostate cancer is an absolute
ily members should be referred to an inherited arrhythmia contraindication because of the required long- term post
clinic (electrophysiology clinic specializing in genetic disor transplant immunosuppression. However, placement of an
ders) for counseling and screening. Patients who have a Bru LVAD would be an appropriate consideration in this patient if
gada pattern but are asymptomatic often do not require !CD he is expected to survive for longer than 1 year.
placement. Tiie incidence of B rugada syndrome is higher in Other contraindications to cardiac transplantation
patients of Asian ethnicity. include medical problems associated with a reduced life
Cardiac magnetic resonance (CMR) imaging would not expectancy (rheumatologic disease, severe pulrnona1y dis
be helpful given the nonnal echocardiogram and diagnosis ease, liver failure) , fixed severe pulmona1y hypertension,
of Bmgada syndrome. CMR imaging would be helpful if diabetes mellitus with end-organ manifestations, age greater
occult structural heart disease was suspected, such as car than 65 to 70 years, severe pe1ipheral arterial or cerebro
diac sarcoid. amyloidosis, or arrhythmogenic right vent1ic vascular disease, and advanced kidney disease. Although
ular dysplasia. several of these factors are also associated with poorer out
An exercise treadmill stress test can be valuable for comes with LVAD use (such as advanced age and degree
identifying an exercise-induced arrhythmia. Brugada syn of comorbid disease), assist devices are a viable option for
drome often presents with nocturnal arrhythmias and is treatment in patients who are clearly not candidates for
usually not adrenergically driven. A stress test would not aid transplantation.
in this patient's diagnosis. Metolazone inhibits sodium reabsorption in the distal
Tilt-table testing should be reserved for patients with tubule and may be particularly effective in inducing diuresis
recurrent syncope without known heart disease or those when used in combination with a loop diuretic in patients
with heart disease in whom a cardiac cause of the syncope with volume overload who have not responded adequately
has been excluded. Tilt-table testing may also be helpful in to high doses of a loop diuretic. However, this patient does
evaluating patients in whom documenting neurocardiogenic not have signs of volume overload (no jugular venous dis
syncope is important (such as in high-risk occupational tention or edema) and therefore would not be expected
settings) . In this patient, the ECG is diagnostic for Brugada to benefit from the addition of metolazone to his current
syndrome and tilt-table testing is not needed. regimen.
Home inotropic therapy is associated with a mortality
KEY POINT
rate of approximately 90% at 1 year and should be consid
• The presence of 2-mm or more coved precordial ered as a palliative care option only. Use of this therapy is
ST-segment elevation (leads V1 through V ) on electro associated with worsening hea11: failure, infection, and
cardiogram and symptoms (cardiac syncope) or ven arrhythmias. In a patient who is a candidate for either
tricular arrhytlunia indicates the presence of Brugada LVAD or heart transplantation, this should not be con
syndrome. sidered as an alternative therapy. Occasionally, patients
require supportive inotropic therapy until t hey receive a
Bibliography transplant. Tiiis should be managed by their transplant
Mizusawa Y. Wilde AA. Brugada syndrome. Circ Arrylhm Electrophysiol. cardiologist.
2012 Jun 1 :5(3):606- 16. [PM!D: 22715240]
K EY P O I N T
• Placement of a left ventricular assist device is an
Item 69 Answer: c
option for patients with end-stage heart failure who
Educational Objective: Manage end-stage heart failme are not candidates for hea11: transplantation.
with a left ventricular assist device.
1 90
An swers and Criti q u es
1 91
Answers a n d Criti ques
despite the absence of symptoms. Surgical valve replacement i n i t ial ECG. A l though an early i nvasive strategy (defi ned as
is recommended given the dysplastic valve features and pres w i t h i n 2-1 hours o f hospital adm ission) has been proved lo
ence of coexisting moderate pulmonary valve regurgitation. be efTect ive in t reatment o f NSTE M I . t here is no evidence
Most patients with severe pulmonary valve stenosis are treated t hat earlier angiography (<6 hours or at hospi tal admission)
with balloon valvuloplasty, but this patient has a dysplastic ofTers incremental beneAt lo these patients.
valve related to Noonan syndrome and moderate pulmonary J n pat ients with a low TI M I risk score ( 0- 2) . i n d ica t i ng
valve regurgitation; thus, pulmonary valve replacement is a low i n - h o sp ital risk of de<1 t h or recurrent ischem ia 'in farc
recommended. tion. predischarge st ress testing may be warran ted to further
Patients with pulmonary valve stenosis without a his define a large ischemic burden and guide revascul a rizat ion
tory of endocarditis or pulmonary valve replacement do not decisions. ll1 is patient has a T I M I risk score o r 4 (:2:3 t ra
require endocarditis prophylaxis. d i t ional card i ovascu lar risk factors. ST-segment de\·i a t ion .
Exercise testing could help determine whether the d a i ly aspirin use. elevated cardiac b iomarkers) . p laci ng h i m
patient has exercise limitation related to her pulmonary a t i ntermediate risk. 'l l1ese patients have i mproved c l i nical
valve stenosis but would not change the recommendation for outco mes w i t h an early i nvasive strategy. Exercise stress
intervention; thus, it is not required. test ing is not appropriate and may b e da nge rous .
Clinical observation, with continued participation in Cl i n ical decisio n - making should not be affected by t h e
competitive sports, is not advised in patients with severe resu lts of t he second set o f card iac b i o m arkers ·n1e pres.
pulmonary valve stenosis regardless of symptom status ence of an elevated t ropo n i n level drawn i n t he emergency
because of the risk of progressive right heart failure. department is p rognost i cal ly sign i ficant and warrants hospi
tal adm ission . t rea t ment of NSTE M I . and risk stra l i ficat i on .
KEY POINT
• In patients with severe pulmonary valve stenosis, KEY POINT
valve intervention is recommended regardless of the • Initial therapy o f non-ST-elevation myocardial infarc
presence or absence of symptoms. tion is medical, with antiplatelet and anticoagulant
medications, antianginal medications, and cardiopro
Bibliography tective medications; early angiography (<6 hours or at
Burch M. Sharland M. Shinebourne E. Smith G. Patton M. McKenna W. hospital admission) does not provide incremental ben
Cardiologic abnormalities in Noonan syndrome: phenotypic diagnosis
and echocardiographic assessment of ! l 8 patients. J Am Coll Cardiol.
efit but an early invasive strategy (defined as within
1993 Oct:22(4) : l l89- 92. [PMlD: 8409059] 24 hours of hospital admission) may improve outcomes.
Bibliography
Cl Educational Objective:
Item 73 Answer: A
Amsterdam EA, Wenger NK, Brindis RG, et al; ACC/AHA Task Force
Manage a patient with a non Members. 2014 AHA/ACC guideline for the management of patients with
ST-elevation myocardial infarction with antiplatelet and non-ST-elevation acute coronary syndromes: executive summary: a
report oft he American College of Cardiology/American Heart Association
anticoagulant medications. Task Force on Practice Guidelines. Circulation. 2014 Dec 3:130(25) :2354-
94. [PMID: 25249586]
ll1 is patient should be given a P2Y 1 2 i n h i b ito r. such as clopido
grel. and an a n ticoagulant. such as the low-molecu la r-weigh t
heparin enoxapari n . a nd b e adm itted to I he hospital. Once Item 74 Answer: E
the d iagnosis of a non -ST-elevation myocardial i n farction
Educational Objective: Evaluate change in clinical sta
(NSTEM I ) has been con Armed by the presence or ischemic
tus of a patient with mitral regurgitation.
chest pai n. ST segm en t de p ressi o n on the electrocardiogram
-
( ECG) . a nd or abnormal cardiac biomarkers. the use of' a n t i Transthoracic echocardiography (TIE) in a patient with val
platelet a nd ant icoagu lant medications. a n tianginal medica vular heart disease is appropriate when there is a change in
t ions. a nd cardioprotective medications is imperative. ll1is clinical symptoms. This patient has had worsening dyspnea
patient was given aspi rin a nd n i t roglycerin prior to the diag on exertion for the past 3 weeks that may be a result of
nosis of NSTEM I. and he takes daily ACE i n hibitor a nd statin worsening mitral regurgitation. In patients with myxomatous
med icat ions. T he addi t ional t herapies t hat are warra nted in mitral valve disease, rupture of a primary or secondary chor
t h i s situat i on i ncl ude a P2Y12 i n h i bi to r ( c l op i cl ogre l . prasu dae may cause an acute change i n the degree of mHra l regur
grel. t icagrelor) . an ant icoagu lant (unfract ionated hepa rin o r gitation and change in clinical status; this is the likely cause
l ow- m ol ecu l a r we i ght h e pa ri n ) . a nd a �- b locke r. ·n1e use of'
- of this patient's worsening symptoms. Other causes of her
clopidogrel. in addi t ion lo aspirin. is the besl -stucliecl combi progressive shortness of breath could also be evaluated with
nation of antiplatelet medications. an echocardiogram. For example, new wall motion abnor
I n patients w i t h an ST-elevation myocardial i n farction malities could signal recent silent myocardial infarction, and
(STEM I ) . repe rfusion . preferably ,·ia percuta neous coronary changes in overall ejection fraction would prompt evaluation
i rnervernion. should be performed as quickly as poss i b l e for new cardiomyopathies.
from symptom onset. T h is patient does n o t have evidence Exercise stress testing may be appropriate if TTE does
or ST-segment elevation or lef't bundle bra nch block on t he not reveal a structural cause of her shortness of breath.
1 92
Answers and Critiq u es
In addition to evaluating for obstructive coronary artery patients with mitrai valve regurgitation include a holosys
disease, stress echocardiography could be used to evaluate tolic murmur, heard best at the apex, that radiates to the
changes in mitral regurgitation and pulmonary pressures axilla.
with exercise. However, a TTE at rest should be obtained Recurrent aortic coarctation occurs in about 20% of
before deciding whether a stress test is warranted. patients with previous coarctation repair. Clinical features
111is patient' s mitral regurgitation increases her risk for include hypertension that is difficult to control with medical
atrial fibrillation. However, her baseline electrocardiogram therapy. lll i s patient has hypertension that is controlled with
demonstrates sinus rhythm, so paroxysmal atrial fibrillation medical therapy, which occurs in up to 75% of patients with
as a cause of her dyspnea is less likely. In addition, she has repaired coarctation. Other features of recurrent coarctation
had progressive dyspnea on exertion that has not waxed not demonstrated in this patient include a radial artery-to
and waned, as might be expected if she was in and out of femoral artery pulse delay and a systolic murmur over the
atrial fibrillation. 111e fact that she presents with worsening left anterior or posterior chest.
symptoms and is in sinus rhythm makes paroxysmal atrial
KEY POINT
fibrillation less likely. 111erefore, 24-hour ambulatory elec
trocardiographic monitoring would not be the best choice • A bicuspid aortic valve is present in more than 50% of
for this patient. patients with aortic coarctation; more than 70% of
lll i s patient does not appear to have active asthma patients with a bicuspid aortic valve will require car
symptoms or changes in pulmonary status that would indi diac surgical intervention for valve dysfunction or
cate a need for spirometry at this time. aortic pathology over the course of a lifetime.
Transesophageal echocardiography may be helpful in
further defining the anatomy, particularly if surgical inter Bibliography
vention is planned, but should not be the first diagnostic Tanous D. Benson LN, Horlick EM. Coarctation of the aorta: evaluation and
study. If the cause of the valvular disorder or degree of management. Curr Opin Cardiol. 2009 Nov;24 (6):509- 15. [PMID:
19667980]
regurgitation is unclear from the TIE, then transesophageal
echocardiography may be appropriate.
1 93
Answers a n d Criti q u es
ure regimen that already includes an ACE inhibitor would s i derable i nc l i v i c l u a l va r i a t i o n i n closes t h a t lead to tox ic
tional benefit, and this medication combination has been 'l h i s pa t i e n t developed carclio tox i c i ty despi t e a low
shown to increase risk of hyperkalemia and kidney injury. r i s k ( I 'X, ) . ll1erelore. n e i t her co n t i n u i ng doxoru b i c i n nor
Because this patient's heart rate is 56/min, indicating decrea s i n g t he dose i s an appropriate t reatmen t . Aclcl it ion
adequate p-blockade, and his blood pressure is at a desired a l ly. bec<i u se C<ircl iotoxici ty is associated wi t h t h i s class o f
level, no benefit would be expected by increasing his dose agents. subst i t u t i ng a n o t h e r a n t h racyc l i ne for t h e cloxoru
Indications for mitral valve replacement, an invasive I n t h i s pa t i e n t . <rn t h rncyc l i ne t he rapy m u st be d i scon t i n ued
procedure that carries risks, include the presence of severe i ndefi n i tely to reverse the carcli otox ic eflect s and p reve n t
mitral regurgitation and NYHA class III or IV symptoms t hem from recurri ng.
attributed to the valve disease. None of these are present ACE i n h i b i tors are i n d icated i n p<:l t i e n t s w i t h asymp
in this patient. This patient's mitral regurgitation is "func toma t i c or sym p toma t ic syst o l i c dysfun ct io n . ll1ere a re l i m
tional, " meaning it is more likely to be a result of his dilated i ted d a ta on t he use of t hese age n t s to h e l p preve n t ca r
cardiomyopathy and not the underlying cause. d i otox i c i t y re l a t ed to chemot herapy. b u t t h e i r use for t h is
i nd i cation i s not rou t i nely recom mended.
KEY POINT
KEY POINT
• Implantable cardioverter-defibrillator placement is
indicated for patients with heart failure and a left ven • Anthracyclines, such as doxornbicin, can cause carcli
tricular ejection fraction less than or equal to 35% and otoxicity in patients being treated with chemotherapy.
New York Heart Association functional class II or III
heart failure while on optimal medical therapy. Bibliography
Swain SM, Whaley FS, Ewer MS. Congestive heart failure in patients treated
with doxorubicin: a retrospective analysis of three trials. Cancer. 2003
Bibliography Jun 1 ;97(11):2869-79. [PMID: 12767102)
Bardy GH. Lee KL. Mark DB, et al; Sudden Cardiac Death in Heart Failure
Trial (SCD-HeFT) I nvestigators. Amiodarone or an implantable cardio
verter-defibrillator for congestive heart failure. N Engl J Med. 2005 Jan
20;352(3):225-37. Erratum in: N Engl J Med. 2005 May 19;352(20):2146. Item 78 Answer: D
[PM ID: 156597221
Educational Objective: Evaluate a patient with dyspnea
with a history of catheter ablation for atrial fibrillation.
Cl Educational Objective: Treat cardiotoxicity due to che
Item 7 7 Answer: B
lhe most likely cause of dyspnea in this patient is pulmonary
vein stenosis. He has progressive, unexplained dyspnea and
motherapy with an anthracycline.
a history of multiple catheter ablation procedures for atrial
-n1e doxoru b i c i n s h ou l d b e d i sco n t i n u ed i n d e fi n i t e ly i n L h i s fibrillation. During catheter ablation of atrial fibrillation,
pat i e n t . T h e e f'fi cacy o f a n t h racyc l i n e based c h e m o t herapy the tissue around each of the pulmonary veins is cauter
reg i m e n s lor breast a d e n oca rci no m a has been. docu men led ized to achieve electrical isolation and prevent ectopic foci
by m u l t ip le s t u d ies. H ovveve r. doxoru b i c i n . d a u n o ru b i c i n . from triggering recurrent atrial fibrillation. Following this
a n d ot h e r a n t h racyc l i nes have k n ov; n c a rd io t ox i c i ty. w h i c h procedure, approximately l % to 3% of patients can develop
i s depe n d e n t o n dosage . age . conco m i t a n t t rea t m e n t w i t h symptomatic pulmonary vein stenosis. The risk is higher
o t h e r carcl i o t o x i c age n ts. chest rad i a t i o n . a n d p re e x i s t i ng after multiple procedures, but it can occur after a single
c a r d i a c d i sease . ll1e i nc i d e nce of' card i o t ox i c i t y l ( i r doxo procedure. Patients present with progressive dyspnea, but
ru b i c i n or d a u n o r u b i c i n is less t h a n 1 'X, k i r c u m u l a t ive more severe pulmonary vein stenosis can be accompanied by
dosages of' less t h a n 400 m g m 2 bu t 26'X, lor c u m u l a t ive cough, hemoptysis, or chest pain. The image (shown on top
dosages grea t e r t h a n or eq u a l to 550 mg ' 111 2 . Cll"cl i o tox of next page) depicts a three-dimensional cardiac magnetic
i c i t y from a n t h racyc l i nes usu<J l ly develops w i t h i n seve rn l resonance angiogram showing the pulmonary venous return
m o n t h s a ft er i n i t i a t i on or chemo t h erapy. m a n i test i n g as to the left atrium (LA) , with stenosis of a pulmonary vein at
e i t her systo l i c o r d i a s t o l i c h e a r t l'a i l u re. a l t hough t h ere ca n its ostium where it empties into the LA ( a rrow) .
be a long l a te n cy period . O n ce h e a r t f a i l ur e is prese n t . a s lhe most important step in the diagnosis of pulmonary
in t h i s pat i e n t . a n t h racyc l i n e s s h o u l d be clisco n t i n u ecl. I n vein stenosis is maintaining a high degree of suspicion when
t h e absence o r h e a r t fa i l u re. a n t h racyc l i nes a l s o s h ou l d encountering a patient with dyspnea and prior atrial fibril
be cl isco n t i n u ecl w h e n new left ve n t ricu l a r dys fu nc t i o n lation ablation. Several diagnostic modalities can be used to
is cl e t ec t ecl . Ro u t i n e su rvei I l a n e e vvi t h s e r i a l echoca rel i make the diagnosis noninvasively, including CT angiography,
ography is reco m me n d e d i n p a t i e n ts b e i n g t re<l l ecl w i t h magnetic resonance angiography, and nuclear lung perfu
a n t h rncyc l i nes. w i t h t i m i n g i n terva l s based on t h e pa t i e n t ' s sion scanning. Each method has advantages and disadvan
base l i n e fu n c t i o n . r i s k p ro l l le. dose. ;rnd c l i n i cal s u s p i c i o n tages, and the preferred method of diagnosis often differs
lo r tox ici t y. T h e m a x i m u m c u m u l a t ive d o s e !O r t h ese c l rugs from institution to institution.
1 94
Answers and Critiques
1 95
Answers a n d C ritiques
risk increasing with progressive increases in aneurysm size. for adm i n istration during PCI . rat her t ha n "up front" in the
For aneurysms smaller than 4.0 cm in diameter, the annual emergency departrnenl. Large studies have shown no clear
rupture risk is below 0.5%, whereas for aneurysms between mortal i ty benefit and sign i fi can t ly h igher rates o f' major
5.0 and 5.9 cm in diameter, the annual rupture risk is 3% to bleeding in patients u ndergoing fibrinolysis treated w i t h
15%. For AAAs with a maximum diameter less than 3.5 cm, abcixi mab versus placebo . U s e o f ' platelet glycoprotein l i b/
repeat surveillance with ultrasonography may be repeated I l l a i n h ibi tors in pat ien ts u ndergo i ng t h rombolysis is not
every 3 to 5 years. However, larger aneurysms require more cu rrently recom mended.
frequent surveillance because of their tendency to expand For STE M I patients u n dergo i ng primary PCI . both pras
faster than smaller anemysms and their increased risk of ugrel and ticagrelor have shown superior efficacy compared
rupture, with reevaluation on a 6- to 12-month basis recom with clopidogre l . However. use of t hese agents in patients
mended. Because this patient's aneurysm is 4.7 cm in diam t reated with t h rombo lytic t herapy has not been wel l studied.
eter, he should undergo surveillance every 6 to 12 months. and l ittle evidence exists to reco mmend t he use or either of
Elective repair should be considered for AAAs of t hese agents i n patien ts receiving t h rombolyt ic t herapy.
5 . 5 cm in diameter in men and 5.0 cm in women, for AAAs
KEY POINT
that increase in diameter by more than 0.5 cm within a
6-month interval, and for those that are symptomatic (ten • Patients with ST-elevation myocardial infarction
derness or abdominal or back pain) . undergoing thrombolysis should be given adjunctive
AAA rupture has an exceedingly high mortality rate, antiplatelet therapy with clopidogrel.
and the risk of rupture is 0.5% to 5% annually for aneurysms
between 4.0 cm and 4.9 cm in diameter. Therefore, no fur Bibliography
ther management would not be appropriate in this patient Sabatine MS. Cannon CP. Gibson CM, et al; CLARITY-TIMI 28 Investigators.
Addition of clopidogrel to aspirin and fibrinolytic therapy for myocardial
with a 4.7-cm aneurysm. infarction with ST-segment elevation. Engl J Med. 2005 Mar
24:352(12):1179-89. [PM ID: 15758000]
KEY POINT
• An abdominal aortic aneurysm smaller than 5 . 5 cm in
Item 82 Answer: D
men and 5 . 0 cm in women is managed conservatively,
with routine surveillance by abdominal ultrasonogra Educational Objective: Manage iron-deficiency anemia
phy every 6 to 12 months. in a patient with cyanotic congenital heart disease.
CJ Item 81 Answer: B
1 96
Answers and Critiq u es
Although heart-lung transplantation is an option for In this active construction worker who is asymptom
end-stage cardiopulmonary disease in patients with Eisen atic, the pretest probability of finding ASCVD is low; because
menger syndrome, this option is not indicated without fur of the increased rate of false-positive test results in low-risk
ther trial of standard medical therapy with iron. patients, stress testing is not recommended. The use of stress
testing to diagnose ASCVD in asymptomatic persons does
KEY POINT
not reduce mortality. Appropriate risk factor modification,
• Most patients with cyanotic heart disease have com however, does have the potential to reduce cardiovascular
pensated erythrocytosis with stable hemoglobin levels. risk and mortality.
This patient's blood pressure is adequately treated, and
Bibliography there is no evidence that further lowering of blood pres
Tay EL, Peset A, Papaphylactou M, et al. Replacement therapy for iron defi sure results in decreased cardiovascular risk. Therefore, an
ciency improves exercise capacity and quality of life in patients with
cyanotic congenital heart disease and/or the Eisenmenger syndrome. Int increase in his lisinopril dosage would not be beneficial.
J Cardiel. 2011 Sep 15;151(3):307-12. [PMID: 20580108] Although studies of the mechanisms of atherosclerosis
suggest that antioxidant therapy might be protective against
development of ASCVD, studies have failed to show a benefit
Item 83 Answer: A of antioxidants as a primary prevention intervention. The
Educational Objective: Use coronary artery calcium use of vitamins A, C, or E, alone or in combination, is there
score to clarify cardiovascular risk in an intermediate-risk fore not recommended to decrease cardiovascular risk.
patient.
KEY POINT
The most appropriate option for this patient with interme • Coronary artery calcium scoring can improve cardio
diate cardiovascular risk is to obtain an additional factor to vascular risk assessment in intermediate-risk patients
help clarify risk to guide therapy, such as a coronary artery in whom therapy may be affected by a reclassification
calcium (CAC) score. Assessment for risk of atherosclerotic of risk.
cardiovascular disease (ASCVD) is an important component
of primary prevention. Several risk assessment tools for
Bibliography
ASCVD are available, with the Framingham risk score being Greenland P, Alpert JS, Beller GA, et al; American CoUege of Cardiology
the most commonly used. The Pooled Cohort Equations are Foundation; American Heart Association. 2010 ACCF/AHA guideline for
a new risk assessment instrument developed from multiple assessment of cardiovascular risk in asymptomatic adults: a report of the
American College of Cardiology Foundation/American Heart Association
community-based cohorts (including the Framingham study) Task Force on Practice Guidelines. J Am Coll Cardiel. 2010 Dec
that includes a broader range of variables and endpoints than 14;56(2S):e50-103. [PMID: 21144964]
1 97
Answers a n d Critiques
Patients with stable claudication progress to critical this patient, CMR imaging and multidetector CT scanning
limb ischemia and limb loss at a rate of less than 5% annu are not indicated because the aortic root and ascending aorta
ally. For most symptomatic patients, therefore, noninvasive are adequately visualized by transthoracic echocardiogra
therapy with exercise and medication is appropriate. If con phy. In addition, annual multidetector CT scanning would
servative therapy fails or patients have symptoms limiting needlessly expose the patient to excessive radiation.
their lifestyle or employment, revascularization by an endo Transesophageal echocardiography is an invasive pro
vascular or surgical approach should be considered. cedure and would be considered if transthoracic imaging
was inadequate to measure aortic root size and evaluate the
KEY POINT
ascending aorta.
• Supervised exercise therapy can effectively treat clau Because of the risk of aortic dissection and rupture,
dication, with increases in pain-free walking time reassurance and clinical observation are inadequate fol
and maximal walking time, and is recommended as low-up for bicuspid valve-related aortopathy.
part of the initial treatment regimen for intermittent
KEY POINT
claudication.
• I n patients with a bicuspid aortic valve, serial evalua
Bibliography tion of ascending aortic diameter should be per
Murphy TP, Cutlip DE, Regensteiner JG, et al; CLEVER Study Investigators. formed by transthoracic echocardiography (or by CT
Supervised exercise versus primary stenting for claudication resulting angiography or magnetic resonance angiography if
from aortoiliac peripheral artery disease: six-month outcomes from the
claudication: exercise versus endoluminal revascularization (CLEVER) not adequately visualized by echocardiography) .
study. Circulation. 2012 Jan 3:125(1):130-9. [ PM I D : 220901681
Bibliography
Morcli I, Tzemos N. Bicuspid aortic valve disease: a comprehensive review.
Item 85 Answer: D
Cardiol Res Pract. 2012;2012:1 96037. [PM I D : 22685681]
Educational Objective: Select proper imaging surveil
lance for a patient with a bicuspid aortic valve and aor
topathy. Item 86 Answer: D
Educational Objective: Diagnose obstructive coronary
This patient with a bicuspid aortic valve should have annual
artery disease in a patient with left bundle branch block.
transthoracic echocardiography. Bicuspid aortic valve occurs
with other cardiovascular and systemic abnormalities. Specif This patient with a left bundle branch block (LBBB) on her
ically, ascending aortic dilation may occur in persons with a baseline electrocardiogram (ECG) should undergo vasodilator
bicuspid aortic valve, in combination with aortic valve disease nuclear perfusion imaging. In patients with baseline ECG
or as an independent condition. Previously considered a sec abnormalities such as pre-excitation, LBBB, a paced rhythm,
ondary event caused by abnormal aortic valve function, the or baseline ST-segment depression greater than 1 mm, ECGs
aortopathy associated with a bicuspid aortic valve is now rec obtained during stress testing cannot be appropriately inter
ognized to result from intrinsically abnormal connective tis preted, and standard exercise treadmill testing is therefore
sue. As a result, serial evaluation of ascending aortic diameter not appropriate. Instead, these patients must undergo stress
should be performed by transthoracic echocardiography (or testing with additional imaging, such as nuclear perfusion
by CT angiography or magnetic resonance angiography if not imaging or stress echocardiography.
adequately visualized by echocardiography) . The frequency When myocardial perfusion imaging is used to evaluate
of surveillance depends upon aortic root and ascending aorta patients with LBBB, a vasodilator study using an agent such
size. Expert consensus guidelines recommend reassessment as adenosine or dipyridamole is necessary instead of a n
of the aorta if the aortic root or ascending aorta dimension is exercise study. Th i s i s because perfusion defects that are not
greater than or equal to 4.0 cm, with the evaluation interval related to obstructive coronary artery disease (CAD) can be
determined by degree and rate of aortic dilation and by family seen in the septum with exercise. Radiotracers are distrib
history. Annual evaluation should occur if the aortic diame uted with blood flow, and when the coronary arteries fill
ter is greater than 4.5 cm. New or changing symptoms and during diastole, the delay in contraction of the septum with
pregnancy are indications for earlier imaging of the aorta. For LBBB can impair filling and create a defect in the septum in
this patient, in view of the similar findings on transthoracic the absence of obstructive CAD. However, vasodilators pro
echocardiogram and chest CT scan, serial transthoracic echo duce hyperemia and a flow disparity between myocardium
cardiography is reasonable. Moreover, transthoracic echo supplied by the stenotic vessel as compared with the unob
cardiography is more cost effective than both CT and cardiac structed vessel that is not affected by the delay in septa! con
magnetic resonance (CMR) imaging, and serial CT scans can traction related to LBBB. For this reason, an exercise nuclear
result in significant cumulative doses of radiation in this perfusion study would not be appropriate in this patient.
young patient. Coronary artery calcium scoring quantifies the amount
CMR imaging and multidetector CT are appropriate for of calcium in the walls of the coronary arteries and correlates
further assessment of aortic pathology when transthoracic well with plaque burden in the coronary arteries. It is an ana
or transesophageal echocardiography is not conclusive. In tomic study with fairly high sensitivity for detecting occlusive
1 98
Answers a n d Criti q u es
CAD, although the frequency of false-negative results (signif to warrant immediate coronary angiography as the initial
icant CAD with a low CAC score) is not known. Therefore, diagnostic test.
CAC scoring is more frequently used for risk stratification in The sensitivity and specificity of noninvasive stress test
patients with intermediate risk for atherosclerotic cardiovas ing for the evaluation of chest pain are lower in women
cular disease. Another anatomic study, coronary er angiog than in men. However, the routine use of exercise testing
raphy, is an emerging technology that has high correlation with either nuclear perfusion imaging or echocardiography
with findings on invasive coronary arteriography and may be to assess left ventricular regional wall motion or perfusion
increasingly useful in evaluating for occlusive CAD. imaging is not recommended for women or men in the
absence of baseline ECG abnormalities. Although the addi
KEV POINT
tion of noninvasive imaging increases diagnostic sensitivity
• In patients with suspected coronary artery disease tor coronary artery disease, use of exercise nuclear perfusion
with baseline electrocardiographic (ECG) abnormali testing as the initial test has not been found to reduce cardio
ties such as pre-excitation, left bundle branch block, a vascular events compared with exercise ECG testing alone.
paced rhythm, or ST-segment depression greater than Pharmacologic stress testing with imaging is indicated
1 mm, ECGs obtained during stress testing cannot be for patients who are unable to exercise. In addition, patients
interpreted; therefore, stress testing with additional with. left bundle branch block undergoing nuclear stress
imaging is required. testing should be administered a pharmacologic stressor
even if they are able to exercise because of the potential for
Bibliography a false-positive test owing to a septa] perfusion abnormality
Fihn SD, Gardin J M , Abrams J. et al: American College of Cardiology that may occur with exercise. Pharmacologic stress testing is
Foundation: American Heart Association Task Force on Practice not indicated because this patient is physically able to exer
Guidelines: American College of Physicians: American Association lor
Thoracic Surgery: Preventive Cardiovascular Nurses Association: Society cise and does not have a left bundle branch block.
for Cardiovascular Angiography and Interventions: Society of Thoracic
Surgeons. 2012 CCF/AHA/ACP/AATS/PCNA1SCAl/STS Guideline for the K EV P O I N T
diagnosis and management of patients with stable ischemic heart dis
ease: a report of the American College of Cardiology Foundation/ • Exercise electrocardiographic testing is recommended
American Heart Association Task Force on Practice Guidelines, and the as the initial test of choice in patients with a normal
American College of Physicians. American Association lor Thoracic
Surgery, Preventive Cardiovascular Nurses Association. Society for baseline electrocardiogram and an intermediate pre
Cardiovascular Angiography and Interventions, and Society of Thoracic test probability of coronary artery disease based on
Surgeons. J Am Coll Cardiol. 2012 Dec 18:60(24):e44-el64. lPMID:
23182125] age, sex, and symptoms.
Bibliography
Qaseem A. Fihn SD, Williams S. Dallas P. Owens DK, Shekelle P; Clinical
Item 8 7 Answer: B Guidelines Committee of the American College of Physicians. Diagnosis
of stable ischemic heart disease: summary ofa clinical practice guideline
Educational Objective: Evaluate a woman with atypical
from the American College of Physicians/American College of Cardiology
chest pain with exercise electrocardiography. Foundation/ American Heart Association/American Association for
Thoracic Surgery/Preventive Cardiovascular Nurses Association/Society
This patient should undergo exercise electrocardiography of Thoracic Surgeons. Ann Intern Med. 2012 Nov 20; 157(10):729-34.
[PMID: 23165664]
(ECG). Although she has several risk factors for coronary
artery disease (CAD) , including hyperlipidemia and a family
history of premature CAD, her symptoms are not typical for Item 88 Answer: E
angina, which requires the presence of pain precipitated by Educational Objective: Manage aspirin use for primary
exercise or emotion, a substernal location of the pain, and prevention in a patient with diabetes mellitus and a low
relief with rest or nitroglycerin. Because she has only two cardiovascular risk.
of the three diagnostic criteria for angina, she is classified as
having atypical angina. Women in her age group with atyp No further testing or therapy would be most appropriate
ical angina have an intermediate pretest probability of CAD in this patient. Although she has diabetes mellitus, she has
(approximately 22%) . For patients with an intermediate pre no other major cardiovascular risk factors. Risk assessment
test probability of disease and a normal resting ECG, exercise for atherosclerotic cardiovascular disease (ASCVD) has tradi
ECG testing is recommended as the initial test of choice. tionally been with the Framingham risk score, although the
Conventional coronary angiography identifies the loca American College of CardiologyIAmerican Heart Association
tion and severity of blockages and allows vascular access for Pooled Cohort Equations, a new method for assessment that
percutaneous intervention. Because of the invasive nature includes additional variables for risk stratification, is increas
of coronary angiography and the inherent risks of vascular ingly being used. With this method, a 10-year risk of ASCVD
complications, it should be reserved for patients with acute of less than 5% is considered low risk, 5% to below 7.5% is
coronary syndrome requiring immediate intervention, life considered intermediate risk, and 7.5% and above is desig
style-limiting angina despite medical therapy, or high-risk nated as high risk. This patient has a calculated 10-year risk of
criteria on noninvasive stress testing. This patient's pretest 2.7%, making her low risk for ASCVD. Therefore, no additional
probability of CAD is intermediate, which is not high enough testing is indicated at present.
1 99
Answers and Critiques
It is reasonable to give low-dose aspirin to adults with card i a c o u t p u t is a very h igh systemic vasc u l a r resista nce.
diabetes and no previous history of vascular disease who as t he syste m i c c i rc u l a t i o n in creases a f'terload to m a i n t a i n
are at increased cardiovascular risk and without increased bl ood pressure i n t he sett i ng o f low stroke vo l u m e. W i t h cor
risk for bleeding. However, aspirin should not routinely be rection of t h e vo l u me overload s t a te. t h e next step in l herapy
given to patients with diabetes who are at low risk (men is to red uce a fterloacl w i t h n i t ropruss icle.
younger than SO years and women younger than 60 years N i l ro p ru s s i d e is an i n t ravenously a d m i n istered vaso
without other major risk factors such as hypertension or c l i l a t o r t h a t lowers sys t e m i c vascu l a r res i s ta n ce a n d . t h e re
tobacco use) . The risks of gastrointestinal bleeding or hem fore . i n creases c a rd i ac o u t p u t . ll1 is t h e ra py should be used
orrhagic stroke outweigh the benefits of aspirin in this low o n ly i n the s e t t i ng o f i nvasive mo n i to r i n g. i n c l u d i ng a righ t
risk patient. h e a rt ca t h e t e r a n d pos s i b ly an a r t e ri a l l i n e lo c l osely mea
Coronary artery calcium scoring is reasonable to fur s u re syst e m i c p ressu re . C o u n t e ri n t u i t ive lo w h a t wou ld
ther define cardiovascular risk in patients with intermediate be expected. t h e b l ood pressu re u s u a l ly rises w i t h n i t ro
risk as determined by the Pooled Cohort Equations (S"lo to prusside because or t he i m p roved c a r d i a c pe rfo r ma n ce.
<7.S%). However, this patient's risk of ASCVD is considered N i t roprussi de is assoc i a ted w i t h pos s i b l e rebo u n d vaso
low; therefore, good adherence to lifestyle factors and mon con s l r i c t i o n fol lowi ng d i scon t i n ua t i o n a n d poten t i a l tox
itoring of cardiovascular risk factors are most appropriate in i c i ty cl ue lo i t s m e t abol i s m to cya n i d e w i t h l onger term
this patient. use: t he re fore. t h e rapy i s genera l ly l i m i ted to no more t h a n
There is no role for routine exercise testing in an asymp 24 l o 48 hours i n most p a t i e n t s . Pat i e n t w i t h card i oge n i c
tomatic patient. In patients with low coronary artery disease s h o c k may a l so be t reated w i t h an i no t ro p i c agent s u c h as
pretest probability, false-positive results will be more com d o b u l a 111 i ne.
mon than true-positive results and may lead to unnecessary C h a n g i n g lo cont i n uous i n t ravenous furose m i d e is not
downstream testing and treatment. i n d icated because t he patient h a s normal fi l l i ng p ressures
Although elevated homocysteine levels are associated m a n i fested by t h e p u l m o n a ry ca p i l l a ry wedge pressure
with cardiovascular risk, no data support the use o f folic acid or l 6 m m Hg and right a t r i a l pressure of 4 mm Hg. More
supplementation. which can lower homocysteine levels, to aggressive d i uresis will not i m pact the p r i n c i p a l p ro b l e m .
reduce the risk. w h i c h is low card i ac o u t p u t a n d a h i g h sys t e m i c vasc u l a r
res ista nce. S t u d ies have eva l ua ted t h e e f fi c acy of co n t i n u
KEY POINT
ous versus i n te r m i l le n t b o l u ses o f i n t ravenous d i ur e t i c s i n
• Aspirin should not routinely be given to patients with pa t i e n t s hosp i t a l ized w i t h <lcute heart fa i l u re. ll1ere W<lS
diabetes mellitus who are at low cardiovascular risk no d i f ference d e m o n s t ra ted in p a t i e n t s· sy m p toms . k i d ney
(men younger than SO years and women younger !'u nct ion . or l e n g t h of s t ay between t he two s t ra t egies .
than 60 years without other major risk factors such as H igh - versus l ow-d ose d i u re t ics a l so have been eva l ua ted .
hypertension or tobacco use). P<J t i ents t a k i ng h i g h dosages e x h i b i ted a t rend toward m o re
d i u resis a n d s l i g h t \.vo rs eni n g of k i d ney fu nct io n. D i u resis
Bibliography s h o u l d be performed using w h a tever s t ra t egy i s necessa ry
Pignone M. Alberts MJ. Colwell JA, et al. Aspirin for primary prevention of to remove t he f l u i d .
cardiovascular events in people with diabetes: a position statement of the
Dopa m i ne was rece n t ly compared vvi l h nes i r i t i d e a nd
American D iabetes Association. a scientific statement of the American
Heart Association, and an expert consensus document of the American placebo in pat ients w i t h acute heart f a i l u re a n d m i ld k i d
College of Cardiology Foundation. Circulation. 2010 Jun 22;121 (24):2694 - ney dysfu nc t ion . No bene f i t was demonstrated w i t h e i t her
701 . IPMID: 20508178]
dopa m i n e o r nes i r i t icle compared with p l acebo fbr either
u ri ne output or protec t i o n o f k i d ney func t ion . I n ge neral .
Cl Ed ucational Objective:
Item 89 Answer: D for the t reat me n t of patients hos p i t a l ized w i t h acute heart
Treat low cardiac output in fa i l u re have been negative. For t h e rou t i ne care of p a t i e n t s
heart failure by reducing afterload. hosp i t a l ized w i t h h e a r t f a i l u re . dopam i ne. clob u l a m i ne . a n d
m i l ri none h ave not been shown lo be h e l p fu l a n d m a y b e
Th e most a p p ropriate add i t io n a l t re a t m e n t fbr t h i s p a t i e n t
associated w i t h adverse outcomes.
i s n i t ropru s s i de. A rter several days o r d i u resis . t h i s p a t i e n t
Esmolol i s <111 i n t rnvenous ()-b locker. Li ke a l l (}-blockers.
h<lS <l n o r m a l r i g h t a t ri a l pressure ( 0 -5 111111 Hg) a n d p u l
il has some negat ive i n o t ropic activi ty. and use of t h is
m o n a ry cap i l l a ry wedge pressure above normal but w i t h i n
d rug m ig h t worsen t he p a t i e n t ' s hemody n a m i c status. not
t h e accept a b l e range f b r p a t i e n t s w i t h heart fa i l u re (<1 8 111111
i m p rove i t .
Hg) lo provide opt i m a l ven t ri c u l a r fi l l i ng. These hemocly
n a m i c parameters suggest t ha t t h e card iac outpul is very low K EY P O I N T
a n d is t h e major explanat ion fbr t h e p a t i e n t ' s heart f�1 i l u re • In patients with low-output heart failure, nitroprus
sym p to ms . Acu t e heart ta i l u re is typica l ly m a rked by a com
side can reduce afterload and increase cardiac output;
b i n a t i o n of vo l u m e overload ( m a n i fested by an i n c reased
nitroprusside should be used only in the setting of
p u l m o n a ry capi l l a ry wedge pressure . usual ly 2'.1 8 111 111 Hg)
invasive cardiac monitoring.
a n d reduced card i a c o u t p u t . Part o f t he reason for reduced
200
Answers a n d Critiques
Bibliography Bibliography
Khand aker MH, Espinosa RE, Nishimura RA. et al. Pericardia! disease: diag Fihn SD. Gardin JM. Abrams J, et al: American College of Cardiology
_ and management. Mayo Clin Proc. 2010 Jun;85(6) :572-93. [PMID:
nosis Foundation: American Heart Association Task Force on Practice
20511488] Guidelines: American College of Physicians; American Association for
201
Answers a n d Critiq ues
Thoracic Surgery: Preventive Cardiovascular Nurses Association; Society PCl -c<lp<lble hospital c a n be achieved ( f i rst medical contact
for Cardiovascular Angiography and Interventions: Society of Thoracic
to- device time of 120 minutes or less) . I n studies of patients
Surgeons. 2012 CCF/AHA/ACP/AATS/PCNA/SCAl/STS Guideline for the
diagnosis and management of patients with stable ischemic heart dis t rans ferred from <J non - PCI faci l i ty fo r pri m<J1y PCI, more
ease: a report of the American College of Cardiology Foundation /
than half of patients w i t h STE M ! did not undergo perfusion
American Heart Association Task Force on Practice Guidelines, and the
American College of Physicians. American Association for Thoracic i n 1 20 m i n utes or Jess . In t h i s case, t ra nsfer l i me would be
Surgery. Preventive Cardiovascular Nurses Association. Society for prolonged (>120 m i n u tes) : t here fore. t h rombolytic t herapy is
Cardiovascular Angiography and Interventions, and Society of Thoracic
Surgeons. J Arn Coll Cardiol. 2012 Dec 18:60 (24):e44 -el64. [PMID: the best reperfusion strategy.
23182125]
KEY POINT
• Patients with an ST-elevation myocardial infarction
Cl Educational Objective:
Item 92 Answer: A
Manage a patient with an acute
presenting within 12 hours of symptom onset to non
percutaneous coronary intervention (PCI)-capable
coronary syndrome with thrombolytic therapy. hospitals should receive either primary PC! (if availa
·n1is pat ient should receive a t h rombolytic agent such as ble in <120 minutes) or thrombolytic therapy (if pri
tenecteplase and be transferred to a center capable of' per mary PC! is not available within 120 minutes).
form i ng percutaneous coronary intervention (PCI) . He has
electrocardiographic changes consistent w i t h an acute i n fe Bibliography
rior ST-e leva t ion myoca rdial i n farction (STEM ! ) . Pa t ie n ts O'Gara p·i: Kushner FG. Ascheirn DD. et al: American College or Cardiology
Foundation/American Heart Association Task Force on Practice
w i t h a STE M ! p resen t i ng w i t h i n 12 hours of symptom onset Guidelines. 2013 ACCF/AHA guideline tor the management or
should receive reperf'u sion t herapy w i t h eit her primary PC! ST-elevation myocardial infarction: a reporl of the American College of
Cardiology Foundation/American Hearl Association Task Force on
or t h ro mbolys i s . with PCI being the preferred intervention
Practice Guidelines. Circulation. 2013 Jan 29:127(4):e362-425. Erratum
owing to increased eITT cacy. When transfer t i mes for primary in: Circulation. 2013 Dec 24:128(25):e48l. [PMID: 23247304]
PC! exceed 120 mi nutes from presen tat ion . admin istration of'
t h rombolytic t h e rapy is recom mended . such as i n this pat ient
presenting to a faci l i ty wit hout PC! capabi l i ty and a n inabi l i ty Item 93 Answer: A
to transport h i m for treat ment wit h i n t hat t i me frame.
Educational Objective: Diagnose severe aortic
' l h i s pat i e n t has no absol ute con t ra i nd i cations to
regurgitation.
t h romboly t i c therapy, which i nclude previous i n t racerebral
hemorrhage. a known cerebrovascu lar lesion (such as a n T h is patient has aortic regurgitation. The murmur of aortic
a rteriove nous mal forma t i o n ) . suspected aort ic d issection . regurgitation, described as a diastolic decrescendo murmur, is
active bleedi ng or bleed i ng diat hesis (excluding menses) . heard best at the third left intercostal space and may be better
sign i f i ca n t closed head or facia l t ra u ma w i t h i n 3 months. heard when the patient is at end-expiration, leaning forward.
a nd ischemic st roke w i t h i n t he past 3 months. A relat ive Chronic aortic regurgitation has many <Jssociated findings,
con t ra i ndication for t h rombolysis is severe hypertension including widened pulse pressure, bounding carotid and
(defi ned as a systolic blood pressure > 1 80 mm Hg) : however. peripheral pulses, and a diffuse and laterally displaced point
t h i s pat ient's systolic blood pressure does not meet t h is of maximal impulse. A low-pitched rumbling diastolic mur
t h reshold. Even when t h rombolytic t herapy is ad m i n istered, mur ("Austin Flint murmur") can accompany aortic regurgi
t reatment guide l i nes recom mend t hat pa t ients be t rans tation and is caused by premature closure of the mitral leaflets
ferred to a PCl -capable fac i l i ty because of t he poten tial for clue to the regurgitant aortic flow.
t h rombolytic fai l ure. O p t i ma l management o f patients w i t h The auscultato1y findings for mitral stenosis include
STE M ! relies heav i ly upon physician recogni t ion and rapid an opening snap with a low-pitched mid-diastolic murmur
i n i t iation o f reperf'usion t herapy, w i t h e i t her t h romboly t ic (often described <ls a rumble) that accentuates presystole and
t he ra py or PC! . Pat ients w i t h an acute coronary syndrome is heard best at the apex with the patient in the left lateral
a nd a n elect rocardiogram compatible w i t h STE M ! should be decubitus position. It most often occurs in patients with
t reated w i t h reperf'usion t herapy wit hout biomarker con f i r rheumatic valve disease and is frequently associated with
mation. as early bioma rker resu l ts may be normal in pat ients atrial fibrillation.
with ST E M ! . Therefore. wa i t i ng for the resu lts of' card iac bio A small patent ductus arteriosus in the adult produces
rna rker l eve ls wou l d delay appropriate t reatmen t . an arteriovenous fistula with a continuous murmur that
'l h e u s e o f a glycoprotein l i b / I l l a i n h i b i tor. such as envelops the S2 and is characteristically heard beneath the
abcix imab. has not been shown to improve outcomes o f left clavicle. Patients with a moderate-sized patent duc
pat ients w i t h S T E M I p r i o r to t h e primary PC! procedure a n d tus arteriosus may present with a continuous "machinery
s h o u l d be reserved f o r a d m i n istra t ion in t he catheteriza t ion type" murmur best heard at the left infraclavicular area and
labora to1y during primary PC! . bounding pulses with a wide pulse pressure.
Tra nsfer fo r primary PC! is a reasonable a l ternat ive The sinuses of Y<llsalva are three aortic dilatations just
lo l h rombolytic t herapy in t h e set t i ng of' absolute con t ra above the aortic valve cusps. Two of the three sinuses are the
i nd ications to t hrombolylic therapy or h igh-risk c l i n ical origins of the coronary arteries. Regurgitant blood flow into
feat ures <J nd i f' a n accepl<1 b le l i me to t ra nsfer the patient to a the sinus structures fills the coronary arteries and assists
202
Answers a n d Critiques
in the closure of the aortic valve cusps. Sinus of Valsalva An ABI greater than 1 .40 is associated with calcification
aneurysm is a type of aortic root aneurysm. Rupture of the of the arterial wall and may occur in patients with medial
aneurysm will allow flow between the sinus o f Valsalva and calcinosis, diabetes mellitus, or end-stage kidney disease.
either the right atrium or right ventricle, producing a con This finding is uninterpretable and is associated with worse
tinuous systolic and diastolic murmur heard loudest at the cardiovascular outcomes than a normal ABI; t herefore, an
second left intercostal space. Clinical presentation can vary, appropriate next step after this finding is to either measure
ranging from asymptomatic to decompensated heart fail great toe pressure or calculate a toe-brachia! index (systolic
ure. Ruptured sinus of Valsalva aneurysm more frequently great toe pressure divided by systolic brachial pressure) , a
involves the left or right coronary cusps and less frequently test that is typically performed in a vascular laboratory. This
the noncoronary cusp. patient's ABI is normal, so measurement of the toe-brachia!
index is not necessary.
K EY P O I N T
• The murmur of aortic regurgitation is a diastolic KEY P O I NT
decrescendo murmur heard best at the left third • Patients with pseudoclaudication (lumbar spinal ste
intercostal space; associated findings include widened nosis) may report bilateral leg weakness associated
pulse pressure, bounding carotid and peripheral with walking or with prolonged standing; symptoms
pulses, and a diffuse and laterally displaced point are aggravated by prolonged standing and are relieved
of maximal impulse. with bending at the waist.
Bibliography Bibliography
Choudhry NK, Etchells EE. The rational clinical examination. Does this Chou R. Qaseem A, Owens DK, Shekelle P; Clinical Guidelines Committee
patient have aortic regurgitation? JAMA. 1999;281(23):2231-8. lPMID: of the American College of Physicians. Diagnostic imaging !Or low back
103765771 pain: advice for high-value health care from the American College of
Physicians. Ann Intern Med. 2011 Feb 1 ; 154(3) :181-9. Erratum in: Ann
Intern Med. 2012 Jan 3; 156(1 Pt 1 ) :71. [PMID: 21282698]
Item 94 Answer: B
Educational Objective: Distinguish lumbar stenosis Item 95 Answer: A
from peripheral arterial disease. Educational Objective: Manage acute decompensated
systolic heart failure with diuretics.
An MRI of the lumbar spine is most likely to confirm the
diagnosis in this patient. This patient's normal ankle-brachial I n this patient with recently diagnosed heart failure, the
index (AB!) bilaterally, normal distal pulses, lack of a bruit, dosage of furosemide should be increased. She has signs of
normal skin findings, and clinical history all suggest a diagno volume overload (elevated central venous pressure, an S3 ,
sis other than peripheral arterial disease (PAD). Patients with peripheral edema, weight gain) .
pseudoclaudication (lumbar spinal stenosis) may report bilat Given the patient's relative hypotension and volume
eral leg weakness associated with walking or with prolonged overload, increasing her diuretic dose would be more appro
standing; symptoms are aggravated by prolonged standing priate than increasing the dose of her ACE inhibitor, which
and are relieved with bending at the waist. Nearly half of might lead to low blood pressure and would not improve her
patients have absent deep tendon reflexes at the ankles, but volume overload.
reflexes at the knees and muscle strength are usually pre Although there is a mortality benefit to the use of
served. lhe American College of Physicians recommends that p-blockers in patients with systolic heart failure, these
advanced imaging with MRI or CT should be reserved for agents have negative inotropic activity, and initiation of
patients with a suspected serious underlying condition or P-blocker therapy is relatively contraindicated i n patients
neurologic deficits, or who are candidates for invasive inter with evidence of decompensated heart failure. Once the
ventions. In the absence of these indications, back imaging is patient has been appropriately diuresed, a p-blocker can
not indicated. be added. Even patients with a low systolic blood pres
Measuring the exercise ABI can be useful in diagnosing sure, once euvolemic, can often tolerate low doses of a
PAD when the resting AB! is normal and the index of suspi P-blocker.
cion is high for PAD. This patient's history and examination Spironolactone is an appropriate agent to add for treat
findings point to a diagnosis other than PAD, so measuring ment of stable patients with New York Heart Association
the exercise AB! will not add helpful information at this (NYHA) functional class II to IV heart failure. This patient,
time. however, has acute volume overload, which should be treated
Segmental limb plethysmography is useful in patients before initiation of this therapy. Although spironolactone
with an established diagnosis of PAD to help localize the site has some diuretic activity, at the usual doses prescribed for
of stenosis. In this test, blood pressures are recorded using patients with heart failure (12.5-25 mg/d) , it would not have
plethysmographic cuffs placed at the upper thigh, lower sufficient diuretic effect in this patient.
thigh, calf, and ankle. A drop in systolic pressure of 20 mm This patient's presentation demonstrates the i mpor
Hg identifies a zone of significant disease. tance of an early (within 7 days) post-hospital clinic visit for
203
Answers and Critiques
204
Answers a n d Critiques
Cl
Bibliography Item 99 Answer: D
Siu SC. Silversides CK. Bicuspid aortic valve disease. J Am Coll Cardiol. 2010
Jun 22;55(25):2789-800. [PMID: 20579534] Educational Objective: Treat native valve infective
endocarditis complicated by heart block.
205
Answers a n d Critiques
Cl Educational Objective:
Item 1 00 Answer: A from t h e American Col l ege o f ' Cardiology a nd t h e American
Heart Associat ion state t hat all patients with an acute coro
Treat an acute episode of supra
nary syndrome ( u nstable angi na, NSTE M I . or ST-eleva t ion
ventricular tachycardia.
myoca rdial i n farction) treated medically or w i t h a stent (bare
This pa t i e n t should be given adenosi ne. S he has hemo metal stent or drug-eluting stent) should be given P2Y " i n h i b
dynamically stable na rrow-co mpl ex tachycard ia consistent itor therapy ( fo r example. clopidogrel. prasugrel. or ticagrelor)
with suprave n t ricular tachycard ia . ll1 e rhyt h m is regular in add i tion to aspirin for at least 12 months.
a nd no obvious P waves are visible : t herefore . a t riovent ric Pat i ents who receive a stent in the absence or a n acute
u l a r nodal reciprocat i ng tachycardi a (AVN RT) is the most coronary syndrome ( t hat is, for stable a ngina pectoris) a lso
l i ke ly cause. AVN RT accoun ts for u p to two t h i rds o f cases requ i re dual a n t i p latelet t herapy w i t h aspirin and clopido
of' supravent ricular tachycardia. Pat ients often report neck grel u n t i l endot h e l i a lization o f the stent is completed and t he
pu lsations. which are caused by si m u l taneous con t ract ion risk for acute stent t h rombosis decreases. For a bare metal
o f t he atria and ven t ricl es. Because t h e patient n1 i led to stent placed u nder t hese c i rcu mstances, clopidogre l should
termi nate her tachycardia w i t h vagal maneuvers. adenosine be con t i nued for at least l mon t h : for a drug-e luting stent.
should be ad m i n istered . Adenosine is h ig h ly e f fective a t ter clopidogrel should be co n t i nued for at least l year. ll1ere
m i nation of nodal-dependent rhy t h ms and can help iden t i fy is no i ndication for dual a n t i p l atelet t herapy for less t h a n
t h e underlying et iology. For example. con t i n ued atrial act iv I month . N e i t h e r t icagre lor n o r prasugrel h a s been studied
ity ( P waves) d u ring at riove n t ricular block can help iden t i fy extensively in patients u n dergoing coronary stent i m pl a n
a t rial f l u tter and atrial tachyca rdia. Patients given adenosi n e tation for stable angina pectoris: t herefore. t hese patients
s h o u l d be on a cardiac monitor w i t h a running rhy t h m strip should be treated w i t h clop idogrel i n addition to aspi ri n .
on paper to document t h e resu l ts. Prior to giving adenosine.
KEY POINT
patients should be warned that t hey may experience nausea.
f l us h i ng. chest pain. or a sense o f' d read . Pa t ients w i t h bron • Patients with an acute coronary syndrome should be
chospastic lung disease should not receive adenosine. treated with dual antiplatelet therapy (aspirin and a
Alt hough a m iodarone would be e f lect ive for term i na t P2Y 12 inhibitor) for 1 year regardless of initial treat
i ng t h is pat ient's <l rrhy t h m i a . it has many l ong- term risks, ment approach.
i ncluding t hyroid . l iver, pu l monary, and neurologic toxici ty.
I n t h is young pat ient, a m iodarone would not be an appro Bibliography
priate opt i on . 2012 Writing Committee Members. Jneid H, Anderson JL, Wright RS, et al;
Ca rcl ioversion is not i n d icated because t he patient is American College of Cardiology Foundation; American Heart
Association Task Force on Practice Guidelines. 2012 ACCF/AHA focused
hemodynam ical ly stable. a n d pharmacologic a t tempts at update or the guideline for the management of patients with unstable
carcl ioversion. such as adenosine. have not been at tempted . angina/ Non-ST-elevation myocardial infarction (updating the 2007
guideline and replacing the 2011 focused update): a report or the
l bu t i l icle is an intravenous Vaughan-Will iams class I l l American College or Cardiology Foundat ion/ American Heart
an t i a rrhy t h m ic d rug FDA approved for pharmacologic Association Task Force on practice guidelines. Circulation. 2012 Aug
14;126(7):875-910. [PMlD: 22800849]
cardioversion of' a t rial fibri l lati on. ll1e patient has regu lar
supravent ricular tachycardia, not atrial flbril la t ion .
Cl
KEY POINT Item 1 02 Answer: A
• Patients with hemodynamically tolerated supraven Educational O bjective: Recognize late complications in
tricular tachycardia refractory to vagal maneuvers a cardiac transplant patient.
should be given adenosine.
This pat ient should undergo coronary angi ogra p hy. H e
underwent heart transplantation 1 0 years ago a n d presents
Bibliography with exertional dyspnea. ll1e two most co m mon causes of
Link MS. Clinical practice. Evaluation and initial treatment or supraven
dyspnea in post-ca rdiac t ransplant patients are rejection
tricular tachycardia. N Engl J Med. 2012 Oct 11 :367(15):1438-48. [PMID:
23050527] a nd cardiac a l l ograft vascu lopathy. The p revalence of' car
d iac a l l ograft vascu lopa t hy is approximately sou;., by year s
post - t ra nsp la n t a nd is t h e most common cause of mortal i ty
i n patients a fter t he first year post- t ra nsplan t . Because t he
Cl Educational O bjective:
Item 1 0 1 Answer: c
transplanted heart is denervated at t h e t i m e or tra nsplant .
Manage dual antiplatelet ther
vascu lopa t hy and subsequent ischemia may occur without
apy in a patient who had a non-ST-elevation myocardial
t h e classic symptoms o f angina . ll1eretore. t h is diagnosis
infarction treated with a bare metal stent.
must be suspected in long-term transplant pat ients present
A f'u l l year of' clopidogrel t herapy is indicated in t h i s pat ient i ng w i t h symptoms compat ible with ischemia w i thout chest
who has sustained a non - ST-elevation myoca rdial i n farction pain . In t h is patient with exert ional dyspnea 10 years a fter
(NSTEM I ) . Clopidogrel added to aspirin i mproves outcomes transpl a n tation, t he most l i kely cause is cardiac a l l ograft
after hospital ization in pa t ients w i t h NSTE M I rega rdless of t h e vasculopat hy. a n d t here fore proceeding to coronary angiog
i n- hosp ital t reatment approach . Current recommendations raphy to con firm the diagnosis is t he appropriate next step.
206
Answers a n d Criti q u es
207
Answers a n d C ritiq u es
tion. The electrocardiogram may be normal or demonstrate com mo n ly presen t w i t h pericardia! tamponacle (due to
first-degree atrioventricular block and incomplete right bun hemopericardium ) . pulseless electrical activity, and deat h.
dle branch block. Left axis deviation on the electrocardio Emergen t pericarcl iocentesis and subsequent surgical recon
gram is not found in patients with an ostium secundum ASD. st ruc t i o n cc111 i m p rove surviva l . T h e echoca rd iographic r i nd
Patients with patent foramen ovale have a normal echo i ngs in this patient did not reveal pericardia! e ffusion or
cardiogram and physical examination. These patients are tamponade. and h is presen tation is consistent with VSD.
also generally asymptomatic. ll1 is patient has an occluded left a nterior descend i ng
Patients with sinus venosus ASD have features of right artery and persistent ST- segmen t elevation : however. the
heart volume overload but do not have mitral valve disease presence o f' hemodynamic comprom ise and echocard io
and thus will not have a murmur of mitral regurgitation. graphic evidence of VSD requi res emergency cardiac sur
The electrocardiogram may be normal or demonstrate first gery. A l t hough coronary artery bypass gra fl i ng is usual ly
degree atrioventricular block and incomplete right bundle performed during an attempted repa i r of the septa I defect .
branch block. percutaneous coronary i nterve n t ion o f' t he left a n terior
208
Answers a n d Critiques
CJ d escen di ng artery is not ind icated once th e \/SD has b ee n not have evidence of dyssynchrony or an ejection fraction of
d i agnosed. 35% or less, she is not a candidate for treatment.
CONT.
The performance of right heart catheterization may aid
KEY POINT
i n the d iagnosis of \/SD. and the use ofa vasopressor such as
dopam in e may help i n i t i a l ly stab i l i ze the patien t . However, • Hydralazine and isosorbide dinitrate improve symp
t h js patient has a severe hemodynamic impa i rment and toms and reduce mortality in patients with New York
requires emergency cardiac surgery. Heart Association class I I I or IV heart failure symp
toms who are black and are already on maximal
K EY P O I N T
therapy.
• Urgent cardiac surgery offers the only chance o f sur
vival for patients with large postinfarction ventricular Bibliography
septal defects, especially in the presence of cardio Flack JM, Sica DA, Bakris G, et al; International Society on Hypertension in
genic shock Blacks. Management of high blood pressure in Blacks: an update of the
International Society on Hypertension in Blacks consensus statement.
Hypertension. 2010 Nov;S6(5):780-800. [PMID: 20921433]
Bibliography
Van de Werf F, Bax J, Betriu, A, et al; ESC Committee for Practice Guidelines
(CPG). Management of acute myocardial infarction in patients presenting
CJ
with persistent ST-segment elevation: the Task Force on the Management Item 1 07 Answer: D
of ST-Segment Elevation Acute Myocardial Infarction of the European
Society of Cardiology. Eur Heart J. 2008 Dec;29(23):2909-45. [PMID: Educational Objective: Evaluate for suspected perival
19004841] vular abscess in a patient with infective endocarditis.
209
Answers a n d Critiques
210
Answe rs a n d Critiq u e s
bundle branch block, atrial fibrillation with pauses greater Association guidelines d o not currently provide recommen
than 5 seconds, and complete heart block are all indications dations for use of fish oil after MI. Fish oil is effective in
for permanent pacing. This patient has none of these condi reducing triglyceride levels; however, this patient' s triglycer
tions, and neither dual-chamber nor single-chamber pacing ide levels are normal.
is indicated. Recently released guidelines recommend treatment
of patients with established atherosclerotic disease with a
K EY P O I NT
high-intensity statin with a goal of lowering the LDL choles
• Asymptomatic first-degree atrioventricular block with terol level to less than 50% of the baseline level but without
bifascicular block does not require pacemaker treatment to a specific LDL cholesterol level. As this patient
implantation. has had the expected decrease in LDL cholesterol level on her
present regimen, the addition of another agent for managing
Bibliography dyslipidemia, such as niacin, would not be appropriate.
Epstein AE. DiMarco J P. Ellenbogen KA. et al; American College of Although moderate alcohol consumption (approximately
Cardiology/American Heart Association Task Force on Practice
Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 one to three drinks daily) is associated with a lower risk of
Guideline Update for Implantation of Cardiac Pacemakers and coronary heart disease, excessive alcohol intake accounts for
Antiarrhythmia Devices): American Association for Thoracic Surgery:
approximately 4% of cases of dilated cardiomyopathy. How
Society of Thoracic Surgeons. ACC/AHA/HRS 2008 Guidelines for
Device-Based Therapy of Cardiac Rhythm Abnormalities: a report of the ever, reducing this patient's current level of alcohol consump
American College of Cardiology/American Heart Association Task Force tion will not reduce her risk of a future cardiovascular event.
on Practice Guidelines (Writing Committee to Revise the ACC/AHA/
NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers
KEY POINT
and Antiarrhythmia Devices): developed in collaboration with rhe
American Association for Thoracic Surgery and Society of Thoracic • Patients with prior myocardial infarction should
Surgeons. Circulation. 2008 May 27:117(2 l ) :e350 -408. Erratum in:
Circulation. 2009 Aug 4; 120(5):e34-5. [PMID: 1 84832071 receive an ACE inhibitor for secondary cardiovascular
prevention.
Bibliography
Item 1 1 0 Answer: D
Qaseem A. Fihn SD. Dallas P, Williams S, Owens DK. Shekelle P: Clinical
Educational Objective: Manage secondary risk reduc Guidelines Committee of the American College of Physicians.
Management of stable ischemic heart disease: summary of a clinical
tion in a patient with established coronary artery disease.
practice guideline from the American College of Physicians/American
College of Cardiology Foundation/American Heart Association/American
The intervention that offers the greatest cardiovascular Association for Thoracic Surgery/Preventive Cardiovascular Nurses
risk reduction for this patient is to start an ACE inhibitor Association/Society of Thoracic Surgeons. Ann Intern Med. 2012 Nov
20:157(10):735-43. [PMID: 231656651
for secondary prevention after myocardial infarction (Ml)
and to reduce systolic blood pressure. ACE inhibitors have
Cl
been shown to decrease both cardiovascular and all-cause Item 1 1 1 Answer: A
mortality in patients with chronic ischemic heart disease,
Educational Objective: Manage revascularization in a
especially in those patients with prior MI, left ventricular
patient with an acute coronary syndrome with a high TIMI
systolic dysfu nction, or heart failure. Guidelines from the
risk score and muitivessel disease.
American College of Physicians/American College of Cardi
ology Foundation/ American Heart Association recommend 1l1is patient should undergo coronary artery bypass graft
a treatment goal of 140/90 mm Hg or below in patients (CABG) surgery. In patients with a non-ST-elevation acute
with stable ischemic coronary heart disease. Although pre coronary syndrome (unstable angina or non-ST-elevation
vious guidelines recommended treatment to reduce blood myoca rdial infarction) . the TIMI risk score is used to deter
pressure to below 130/80 mm Hg, there is limited evidence mine whether a conservative strategy or an early invasive
to show a benefit of doing so, with the potential for adverse strategy is warranted. This patient has several TIMI risk fac
consequences owing to overtreatment of blood pressure in tors. including aspirin use in the past week, ST-segment
these patients. The 2014 Eighth Joint National Commission deviation. elevated biomarkers. more than three traditional
(JNC-8) report does not provide specific recommendations coronary artery disease (CAD) risk factors, and documented
for treatment of patients with established atherosclerotic CAD with greater than or equal to 50% diameter stenosis;
disease, although the recommended threshold for treat therefore. an early invasive strategy is warranted. In this
ment for all patients younger than 60 years is also 140/90 patient, an oral P2Y 11 inhibitor (clopidogrel. prasugrel, tica
mm Hg. Therefore, this patient would be expected to ben grelor) was not administered, but if this had been given, the
efit from therapy to decrease her blood pressure to at least surgery should be del ayed S days to allow discontinuation
140/90 mm Hg or below. and excretion of the antiplatelet medication.
While there is observational evidence from the GISSI Intra-aortic balloon pump placement may be considered
(Gruppo Italiano per lo Studio della Soprawivenza nell'In for patients with recurrent cardiac ischemia and poor left ven
farto M iocardico) Prevention study that patients with prior tricular function. However. although this patient had recur
MI who take fish oil have a 20% reduction in mortality rate, rent chest pain during hospitalization, his symptoms improved
the current American College of Cardiology/American Heart with intravenous nitroglycerin and medical therapy, and he
21 1
An swers a n d Criti q u es
Bibliography
Cl Educational Objective:
Item 1 1 2 Answer: c Lindman BR, Bonow RO, Otto CM. Current management of calcific aortic
stenosis. Circ Res. 2013 Jul S;ll3(2):223-37. [PM ID: 23833296]
Treat a patient with severe cal
cific aortic stenosis and aortic regurgitation with surgical
valve replacement. Item 1 1 3 Answer: D
Educational Objective: Diagnose a left atrial myxoma.
Tl1 is pa t i e n t s hould u n dergo surgical aortic valve replace
men t. Surgica l aortic valve rep lacement is t he o n ly t reat This patient most likely has a myxoma that is causing her
ment of aortic stenos i s associ ated w i t h a survival beneft t symptoms and clinical findings. Left atrial myxomas are the
a n d d u rable sym p tom rel i e f'. Surgical aortic valve rep lace most common benign tumors of the heart. These lesions can
ment is the t reatm e n t of c ho ice for most pat ien t s w i t h cause constitutional symptoms, such as fatigue, dyspnea,
sym ptomal ic severe aortic stenosis a nd i s associated vvi l h fever, and weight loss, related to tumor cytokine produc
low mort a l i ty rates for p a t i e n ts younger than 70 years tion; systemic embolization from either tumor fragments
( l'Y.,- 3 %) or associated thrombi may cause neurologic symptoms or
Aort ic valve rep a i r i s a n option i n a l i mi ted nu mber other systemic sequelae. Left atrial myxomas most com
of adu l t patients w i t h aortic valve disease. In genera l . it is monly appear as a mass arising from the atrial septum; the
restricted lo pa tients w i t h aortic regurgitation a nd :rna mass can involve the mitral valve intermittently to cause
tomica l ly favorable aortic valve and root a natomy and can a "tumor plop" that may clinically mimic mitral stenosis.
range from si mple cusp plication to complex valve-sparing Myxomas may occur as part of the Carney complex, which
aortic root repl aceme n t . Tl1is pat ient has severe cal c i A c is an autosomal dominant disorder associated with pigmen
aort i c stenosis a n d a va lve t h a t is u n l i ke ly to b e amenable tation abnormalities (such as blue nevi) , schwannomas, and
to repair. endocrine tumors.
Balloon valvu loplasty. a l though import a n t i n the t reat Metastatic adenocarcinoma with cardiac involvement
ment or ped iat ric patients w i t h severe aort ic stenosis. has a can manifest from direct invasion or hematogenous spread,
more l i m i ted role in adults. e i t her as a bridge to deAn i t ive with symptoms and signs dependent on the site of involve
t rea l m c n l . to d i lTcrc n l i a l c clyspnea symptoms i n h igh - ris k ment. Metastatic adenocarcinoma with cardiac involvement,
patients with comorbid conditions such as COPD, or to treat although common in patients with this tumor type (15% of
patients w i t h calciAc aortic stenosis w i t h hemodyna m ic patient at autopsy) , would be less likely in this patient given
i nstabi l i ty or decompensation . Whi l e bal loon valvuloplasty the isolated anatomic location and recent negative malig
is a potential considera t ion for t h i s patient. the presence nancy screening results.
of sign i Aca n t aortic regurgitation is a con t rai nd ication . Angiosarcomas are malignant tumors that can occur in
I mprovement i n aortic valve area from this procedure is the atria but are less common than myxomas and typically
modest. and many patients have residual severe aort ic stc inAltrate the myocardium, which is normal in this patient.
nosis i m mediately after valvu loplasty. Bal l oon valvuloplasty Lipomas typically are located in the subendocardium,
would not be the best option for t h is patient. not the atrium, and rarely cause symptoms.
21 2
Answers a n d Critiques
Bibliography
Shapiro LM. Cardiac tumours: diagnosis and management. Heart. 2001
Feb;85 (2):218-22. [PMID: 11156679]
Item 1 1 5 Answer: C
Educational Objective: Manage a patient with
Wolff-Parkinson-White syndrome with syncope.
Item 1 1 4 Answer: B
This patient should undergo an electrophysiology study. He
Educational Objective: Manage acute pericarditis on an
has evidence of pre-excitation on his electrocardiogram with
outpatient basis.
a history of palpitations and syncope. The slurring of the
This patient should receive clinical follow-up without hos QRS complex (delta wave) represents early ventricular depo
pital admission or further diagnostic testing to monitor her larization owing to conduction over the accessory pathway
response to therapy, evaluate for possible complications, and (bypass tract) . The presence of a delta wave and symptoms
assess the timing for tapering her medications. Slow tapering of tachycardia are consistent with Wolff-Parkinson-White
over 2 to 4 weeks after initial presentation with improvement syndrome. The episodes could be caused by supraventric
in symptoms is usually performed to reduce the risk of recur ular tachycardia (orthodromic or antidromic reciprocating
rent inflammation. tachycardia) or pre-excited atrial fibrillation. The presence
The vast majority of patients with acute pericarditis, of syncope suggests that these episodes are hemodynami
including the patient presented, can be managed medically cally significant. Identification of syncope in a patient with
on an outpatient basis. For a subset of patients, high-risk fea Wolff-Parkinson-White syndrome should prompt referral to
tures of acute pericarditis may be present and warrant hos a cardiologist or electrophysiologist.
pitalization for treatment and monitoring for possible com An electrophysiology study would allow diagnosis of the
plications; these include fever, leukocytosis, acute trauma, cause of this patient's palpitations and allow risk stratifica
abnormal cardiac biomarkers, an immunocompromised tion for risk of sudden cardiac death. The electrophysiology
host, oral anticoagulant use, large pericardia! effusions, or procedure also affords the opportunity to ablate the acces
evidence of cardiac tamponade. sory pathway and potentially cure his arrhythmia. Stress
CT can be used to show pericardia! thickening in testing can be an appropriate method for risk stratification
patients with acute pericarditis. However, this finding would in patients with asymptomatic pre-excitation; however, this
not change the diagnosis or appropriate management strat patient clearly has symptoms and therefore should undergo
egy in this patient. invasive testing and ablation.
Medical therapy with anti-inflammatory agents is Antiarrhythmic drug therapy is not indicated in this
appropriate for acute pericarditis. However, glucocorticoids patient because the type and mechanism of the arrhyth
are reserved for patients who do not respond to NSA!Ds, such mia are not known. Catheter ablation is preferred in young
as ibuprofen, aspirin, and indomethacin, none of which has persons with Wolff-Parkinson-White syndrome in order to
been tried yet in this patient. Glucocorticoid therapy may avoid lifelong use of potentially toxic medications. Antiar
also increase the risk of recurrent pericarditis and should rhythmic agents are reserved for second-line therapy, par
only be considered in highly selected patients with refrac ticularly in patients with accessory pathways located close
tory pericarditis. to the atrioventricular (AV) node.
Pericardiocentesis is indicated only for patients with Metoprolol and diltiazem are AV nodal blockers and
tamponade or for those in whom the analysis of pericardia! may be unsafe if the patient has anterograde conduction
fluid can be of assistance in diagnosis and management. down the accessory pathway during atrial fibrillation. These
Signs of tamponade are not present in this patient whose drugs can block the AV node and promote rapid 1:1 conduc
inferior vena cava is normal in size on echocardiography, tion from the atrium to the ventricle during atrial fibrillation
whose Doppler ultrasound shows minimal change in mitral and thus induce ventricular fibrillation. AV nodal blockers
inflow with respiration, and whose bedside maneuvers are contraindicated in patients with pre-excited atrial fibril
reveal no pulsus paradoxus. lation, such as this patient.
21 3
Answers and Critiques
KEV P O I NT Bibliography
Fermann GJ. Collins S P. Initial management of patients with acute
• Identification of syncope in a patient with pre-excita heart failure. Heart Fa il Clin. 2013 J u l : 9 (3) : 2 9 1 - 3 0 1 . vi. [ P M ! D :
tion should prompt referral to a cardiologist or elec 23809416]
trophysiologist.
Item 1 1 7 Answer: B
Bibliography
Delacretaz E. Clinkal practice. Supraventricular tachycardia. N Engl J Med. Educational Objective: Appropriately perform surveil
2006 Mar 9;354(10):1039-51. [PM!D: 16525141] lance imaging in a patient with Marfan syndrome and aor
tic root dilation.
Item 1 1 6 Answer: c This patient with Marfan syndrome should undergo sur
Educational Objective: Consider reversible causes in veillance imaging annually. Dilation of the ascending aorta
the evaluation of heart failure. is a systemic feature of Marfan syndrome, and the most
life-threatening complication of Marfan syndrome is aortic
The most appropriate diagnostic test to perform in this young
aneurysm, which can lead to an acute aortic syndrome (aor
patient with new-onset heart failure is to obtain thyroid
tic dissection, rupture, or both) . Accordingly, examination
studies. This patient exhibits signs and symptoms consistent
of the ascending aorta and heart valves is mandatory i n
with a diagnosis of hyperthyroidism, including tachycardia,
patients with Marfan syndrome. The severity of aortic dis
a hyperdynamic precord ium . palpitations, weight loss. and
ease is in relation to the extent of aortic dilation, the length
loose stools. Hyperthyroidism is a we l l-described, reversible
of the dilated segment, and the location of aortic involve
cause of heart failure due to cardiac overstimulation by excess
ment. Most patients with Marfan syndrome present with
thyroid hormone that resembles sympathetic stimu lation .
enlargement of the ascending aorta; therefore, serial exam
Hyperthyroidism causes an increase in heatt rate and myocar
ination is focused mainly on assessing this portion of aorta.
dial contractility; systemic vascular resistance o ften decreases
American College of Cardiology Foundation/American Heart
and may result in a widened pulse pressure. Hypothyroidism
Association guidelines recommend follow-up imaging 6
is also a known cause of hea1t fai lure, alt hough it would be
months after diagnosis, with annual surveillance thereafter
less l i kely in this patient with symptoms more consistent with
if the aortic root is less than 4.5 cm in diameter and other
excess thyroid hormone. Because thyroid function abnormal
wise stable. This threshold is lower than for patients with an
ities are a potentially reversible cause of hearl failure, assess
aortic aneurysm due to other causes because of the tendency
ment of t hyroid function should be considered in patients
for complications in patients with Marfan syndrome with an
with new-onset heart fail ure and clinical fi ndi ngs suggestive
aortic root diameter above this level. If the aortic root diam
of thyroid dysfunction .
eter is 4.5 cm or greater or if the aortic root diameter shows
Evalu ation of unusual causes of heart fa i l u re s h o u l d
significant growth from baseline, more frequent imaging of
not b e performed rou tinely but shou l d be pursued when
the aorta should be considered.
there are suggestions o f specific diseases by h i story or phys
I maging of the aortic root in patients with M arfan
ical examin ation . TI1e pa tient has no signs or symptoms
syndrome is usually performed with transthoracic ultra
suggesting a rheumatologic d isorder, and rout ine screen
sound because i t is able to accurately evaluate this por
ing with an an tinuclear anti body level is not indicated .
tion of the aorta and is noninvasive. However, for aneu
S i m i l a rly. t h is patient does not have a history of fl u - l i ke
rysms above the aortic root, CT o r MRI i s p referred a s
symptoms suggesting a viral etiology, making the poten
t h e y more accurately measure t h e aort ic d imensions i n
tial yield of v i ral titers quite low. Furthermore. d i rected
t h a t region.
treatment options in the presence of posit ive viral t i ters a re
quite l i m i ted . KEV POINT
Endomyocard ial biopsy is rarely indicated in t he eval
• In patients with Marfan syndrome and aortic root
uation of acute heart failure a s i t is i nvasive a n d is unlikely
dilation, surveillance imaging should be performed
to be helpful in ident i fy i ng a reversible cause. It may be
6 months after diagnosis and annually thereafter i f
considered i n patients whose heart failure is unresponsive
t h e aortic size remains stable.
to medical t herapy or is associated with ven tricular a rrhyth
m ias or conduction b lock i n order to evaluate t o r g i a n t cell
myocard itis. Bibliography
Hiratzka LF: Bakris G�. Beckman JA. et al; American College of Cardiology
KEV POINT Foundat1on/Amencan Heart Association Task Force on Practice
Guidelines: American Association for Thoracic Surgery: American
• Evaluation of unusual causes of heart failure should
College of Radiology: American Stroke Association: Society of
not be performed routinely but should be performed Cardiovascular Anesthesiologists: Society for Cardiovascular
Angwgraphy and Interventions: Society of lnterventional Radiology:
when there are suggestions of specific diseases by his
Society of Thoracic Surgeons; Society for Vascular Medicine. 2010
tory or physical examination findings. A�CF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the
diagnosis and management of patients with Thoracic Aortic Disease: a
214
Answers and Criti ques
report of the American College of Cardiology Foundation/ American of t h i s patient's high-risk ECG stress test, he should
Heart Association Task Force on Practice Guidelines, American
Association for Thoracic Surgery, American College of Radiology,
undergo catheterization for a definitive diagnosis and
American Stroke Association, Society of Cardiovascular possible revascularization. There would be no benefit
Anesthesiologists, Society for Cardiovascular Angiography and
to a noninvasive imaging test prior to or instead of that
I nterventions, Society of l nterventional Radiology, Society of Thoracic
Surgeons, and Society for Vascular Medicine. Circulation. 2010 Apr intervention.
6 ; 1 2 1 ( 13):e266-369. Erratum in: Circulation. 2010 Jul 27;122(4):e410.
[PMID: 20233780] KEY POINT
• Cardiac catheterization is indicated in patients
with a positive electrocardiographic stress test and
Item 1 1 8 Answer: B
findings indicative of high-risk coronary artery
Educational Objective: Manage a patient with a high disease.
risk score on exercise treadmill testing with cardiac
catheterization.
Bibliography
This patient's exercise electrocardiographic (ECG) stress Mark DB, Hlatky MA, Harrell FE Jr, Lee KL, Califf RM, Pryor DB. Exercise
treadmill score for predicting prognosis in coronary artery disease. Ann
testing results indicate that he has coronary artery dis Intern Med. 1987 Jun;l06(6):793-800. [ PMID: 3579066]
ease (CAD) , and his Duke treadmill score ( - 11 . 5 ) indicates
the presence of high-risk disease. He should undergo
CJ
cardiac catheterization for diagnosis and possibly revas Item 1 1 9 Answer: c
cularization.
Educational Objective: Diagnose peripartum cardiomy
Exercise ECG stress testing can be used for the diagnosis
opathy.
of CAD (as in this patient) , to evaluate adequacy of medical
therapy in patients with known CAD, and to evaluate func TI1e most likely d i agnosis i n t h i s wom a n who gave b i rt h
tional status. When used to evaluate chest pain, the test is 2 weeks ago is peripartum card i o rnyopathy. Peripartum
considered diagnostic of obstructive CAD (>70% obstruction) cardiomyopathy is l e ft ven t ricu l a r systolic dysfu n c t io n
if there is greater than 1-mm ST-segment depression with i dent i fied toward t he e n d o f pregnancy o r i n the months
exercise in two contiguous leads. The findings in this patient fol l owing delivery i n the absence of another iden t i fiable
are consistent with occlusive coronary disease as the cause cause. This occurs with i ncreased frequency in women
of his exertional chest pain. w i t h a h istory of preeclampsia . Pat ients may be asymp
In addition to diagnosis, a positive treadmill study tomatic or presen t with feat u res o f heart fa i l u re. Prompt
can be used to further risk stratify obstructive CAD. The i n i tiation of medical t herapy is recom mended for women
Duke treadmill score is one method and is calculated with peripartum cardiomyopathy and i ncludes an ACE
as follows: Exercise time in minutes - (5 x ST-segment i n h ib i tor or an a ngiotensin receptor blocker (a fter del iv
depression) - (4 x angina score) . (Angina score: O = ery) , �-blockers, d igox i n , hyd ra l az i n e . n i t rates . and
asymptomatic; 1 = nonlimiting angina; 2 = exercise d iuretics.
limiting angina.) Scores below - 11 are high risk, and P u l mo n a ry embolism c a n occur postpar t u m , par
those above 5 are low risk. Patients with high-risk scores t i c u la rly if prolonged bed rest is req u i red i n t h e peri
are likely to have left main or proximal left anterior partum period . A l t h o ugh bot h p u l m o n a ry e m b o l i s m
descending (LAD) artery disease. Other markers of a a n d h e a r t fa i l u re fre q u e n t ly a re m a rked by dy sp n e a t h i s
,
high-risk exercise study that would be suggestive of pat i e n t ' s prese n t a t ion is more i n d i c a t ive o f heart fa i l u re.
p roximal LAD artery disease or multi-vessel disease w i t h p u l m o na ry congestion and elevated central ve nous
would include a drop in blood pressure with exercise or p ressure .
severe ST-segment depression. Based on his high-risk Mosl patients with ischem ic cardiomyopathy have
Duke treadmill score, this patient should be further eval symptomatic coronary artery d isease, abnormal electro
uated with coronary arteriography. cardiographic findings demonstrating previous myoc ard i a l
Although this patient should be treated with medi i n farction, or regional hypokinesis on echocardiography.
cal therapy including aspirin, a �-blocker, and a statin, he These fin d i n gs are absent in this patient.
should also undergo cardiac catheterization because of the Stress-induced cardiomyopathy ( ta kotsubo cardiomy
high likelihood of severe obstructive CAD. opathy) is characterized by transient cardiac dysfu nction
The use of imaging, such as stress echocardiography with ven tricular apical bal looning. usual ly triggered by
or myocardial perfusion imaging, can localize ischemia i n tense emotional or physical stress, although in several
to a vascular territory and can be helpfu l to determine publ ished cases. no trigger was identi fiable. The presenting
affected vascular territory prior to revascularization. clinical picture may mimic an acute coronary syndrome,
Stress testing with imaging can also be helpful in making with chest pa in, mil d ly elevated cardiac enzyme levels. and
the diagnosis of CAD in patients with equivocal exercise e lect rocardiographic changes consistent wit h ischemia. TI1e
stress tests or those in whom there is a higher likelihood patient's clin ical presenlation is not consistent with stress
of a false-positive exercise stress test. However, because cardiomyopat hy.
21 5
Answers and Criti q u es
216
I ndex
Note, Page numbers followed by f and t denote figures and tables. respectively. Test Angiotensin receptor blockers (ARB), 28t
questions are indicated by Q. in angina, 17
in heart failure, 34-3S, 34t, Q9
A in STEM!, 23
Abciximab, for acute coronary syndromes, 23 Ankle-brachia! index (ABI), 100, 101 - 102, 102t
Abdominal aortic aneurysm (AAA), 99-100, Q31 Anthracycline toxicity, 106- 108, 107t, Q77
open surgical repair vs. endovascular aneurysm repair, 100 Antiarrhythmic medications, SO-Sl, SOt
risk factors for, 99 Antibiotic therapy, for infective endocarditis, 83, 84, 84t
rupture, risk of, 100, lOOt Anticoagulants
screening and surveillance for, 99-100, Q80 for acute limb ischemia, 104
therapies for, 100 for atrial fibrillation, SS, Q27
Accelerated idioventricular rhythm (AIVR). 23 for non-ST-elevation acute coronary syndromes, 26, 27f
after coronary reperfusion, Q42 for patients with prosthetic valves, 86
ACE inhibitor-induced cough, 34, Q9 for peripartum cardiomyopathy, 110
ACE inhibitors, 28t during pregnancy, 112-113, 1 1 2t
in angina, 1 6 for pregnant women with mechanical valve prosthesis, Q60
i n cardiac disorders i n pregnancy, l ! O , l l l t for STE M ! , 23
i n heart failure, 34-3S for stroke prevention in atrial fibrillation, S6-S7, S7t, Q4, Q70
in peripheral arterial disease, 103 Antihypertensive therapy, in peripheral arterial disease, 103
in restrictive cardiomyopathy, 48 Antiplatelet therapy
for secondary cardiovascular prevention, QllO for angina, 1 6
in STEM!, 23 for non-ST-elevation acute coronary syndromes, 26
Acute coronary syndrome (ACS), 20 for patent foramen ovale, 86
care after, 30 for peripheral arterial disease, 103
diagnosis of, 20f for STEM!, 23
non-ST-elevation acute coronary syndromes (NSTE-ACSs), 20, 2S-30 Aortic atheroma, 99, 99f
not associated with obstructive coronary disease, 30 Aortic coarctation. 92-93, 92f, Q3S
pathophysiology of, 20 characteristic murmur of, 92
and percutaneous coronary intervention, medications after, QS3 clinical presentation of, 92
ST-elevation myocardial infarction (STEM! ) , 20-2S diagnostic evaluation of, 92
Acute limb ischemia, 104-IOS, Q66 follow-up after repair, 93
anticoagulation therapy in, 104 pathophysiology of, 92
categories and prognosis of, l OSt treatment of, 92-93
physical findings of, 104 Aortic disease, 95-100
treatment of, 104 abdominal aortic aneurysm, 99-100, IOOt
Adenosine acute aortic syndrome, 98-99, 98f
for arrhythmias, sot, Sl aortic atheroma, 99, 99f
for cardiac disorders in pregnancy, 110, l l l t imaging of thoracic aorta, 9S, 96t
for supraventricular tachycardia, S 3 , Q!OO thoracic aortic aneurysms, 96-98, 97t
Advisory Committee on Immunization Practices (ACIP), on influenza Aortic dissection, 9Sf, 98-99, 98f
vaccination, 18 type A , Q24
African Americans, cardiovascular disease in, 1 type B, Q71
Alcohol septa! ablation, for hypertrophic cardiomyopathy, 46 Aortic override, in tetra logy of Fallot, 93, 93f
Aldosterone antagonists, in heart failure, 34t, 3S-36 Aortic regurgitation, 71, 73t. 76t, 77t, 79-80, Q93
Alteplase, in STEM!, 22t clinical presentation and evaluation of, 79
Ambulatory ECG, for arrhythmias, 12. 14t, Sl, S4 management of, 79. QS9
American College of Cardiology/American Heart Association Aortic repair, in complicated type B aortic dissection, Q71
cardiovascular risk calculator, 3 Aortic root replacement, for aortic regurgitation, 79
American Heart Association's Heart Disease and Stroke Statistics. 1 Aortic stenosis, 71, 73t, 76t, 77-79, 77t, Q20, Q28, Q7S, Q112
Amiodarone causes of, 77
for arrhythmias, sot, Sl, 60 clinical presentation and evaluation of, 77-78
for cardiac disorders in pregnancy, l i l t management of, 78-79
Amitriptyline, and QT-interval prolongation, Ql4 paradoxic low-gradient, 78
Amlodipine pseudosevere aortic stenosis, 78
for coronary artery disease, 29t Aortic valve replacement
for heart failure, 36 in aortic stenosis, 78, Q28, Q 1 1 2
Aneurysm, aortic in bicuspid aortic valve, 8 0
abdominal, 99-100, Q31 Apixaban, for stroke prevention in atrial fibrillation,
thoracic, 96-98, 97t S7, S7t
Angina pectoris. See a lso Coronary artery disease (CAD) Appropriate use criteria (AUC), 18
stable, 14-19 Arrhythmias, S0- 64
testing in, lS, 16f antiarrhythmic medications, SO-Sl, sot
treatment of, 15-19, 17f, Qll, Q48 ARYC/D and, 6lt, 62
antianginal medications, 16, 18 atrial fibrillation, SS-S8
cardioprotective medications, 16-18 atrial flutter, S8-S9
coronary revascularization, 18-19 bradycardia, Sl -S3
dual antiplatelet therapy after revascularization, in Brugada syndrome, 6 1 , 6lt, 62f
19, 19t catecholaminergic polymorphic VT, 61-62, 61 t
Angiography, in acute limb ischemia, Q66 diagnostic testing for, 12. 14t
Angioplasty, in peripheral arterial disease, 104 early repolarization syndrome and, 61t, 62
Angiotensin-converting enzyme (ACE) inhibitors. See ACE inhibitors inherited syndromes, 61-62, 6lt
21 7
I n d ex
21 8
I ndex
219
I ndex
H
Heart failure, 32-42. Q47, Q54, Q56, Q69 , Q76, Q89, Qll6 Ibuprofen, in constrictive pericarditis, Q32
acute decompensated, 38-39, Q95 I CDs. See Implantable cardioverter-defibrillators (ICDs)
advanced refractory. 40-41 Implantable cardiac electronic devices. 52-53. 52t
assessment of. 37-38 and infection. 63. Q58
cardiomyopathies and. 41-42 Implantable cardioverter-defibrillators (ICDs), 52, 52t
chronic. 32. 37-38 in Brugada syndrome, Q68
clinical evaluation in. 32 heart failure and, 36-37, 37t, Q76
device t herapy in, 36 in hypertrophic cardiomyopathy, 44, Q79
cardiac resynchronization therapy. 3 7 . 37t preoperative device management. Ql03
implantable cardioverter-defibrillators. 36-37. 37t Implantable loop recorder, for arrhythmias, 14t
220
I n d ex
221
I nd ex
222
Index
223