KHONG LO VAN ONG MACH

PHOI
KEM THONG LIEN THAT
(Pulmonary atresia with ventricular septal
defect)
Con goi T chng
Fallot the khong lo
van MP (TOF with
pulmonary atresia
TOF/PA)
PGS. TS. Pham Nguyen Vinh
Vien Tim TP. HCM

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

Tan suat
2% BTBS
Nghien cu Baltimore Washington Infant Study
(BWIS) :
* 0,07 TOF/PA/1000 tre ra i con song
* 1,4% BTBS
* 20,3%/tat ca cac the TOF
Nam > n
TL :

Ferencz C et al. The Baltimore Washing Infant Study 1981 1989

Futura Publishing Co. 1993 : 33-62

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

Dch te
Yeu to moi trng :
* Me T : nguy c con x 10 lan TOF/PA
* Me T phu thuoc Insulin : nguy c con
TOF/PA x 20 lan
Yeu to gia nh : co
Yeu to di truyen : co
* Di George syndrome
* 22q11 deletion syndrome (DGS/VCFS hoac
CATCH 22)
* Vater syndrome, Alagille syndrome
3

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

Giai phau hoc : 4 kieu

KLVMP-TLT

TL : Castaneda AR et al. Cardiac Surgery for the Neonate and Infant . WB Saunders 1994, p 218

Giai phau hoc : Kieu thong

thng gia
ong mach bang he (APCAsAortopulmonary collateral arteries)
va MP

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

TL : Castaneda AR et al. Cardiac Surgery for the Neonate and Infant . WB Saunders 1994, p 219

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

Giai phau hoc : KLVMP-TLT vi

than MP (PT) va ong mach
phoi trai (LPA) teo nho
D : ductus
(ong ong
mach
Ao : MC
RAA : tieu nh
phai
LV : that trai
RV : that phai
TL : Emmanouilides GC et al. Heart Diseases in Infants, Children and Adolescent, William
& Wilkins 5th ed 1995, p. 988

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

Giai phau hoc : ong mach bang

he
xuat phat t MC xuong

TL :
Emmanouilides
GC et al. Heart
Diseases in
Infants, Children
and Adolescent,
William &
Wilkins 5th ed
1995, p. 988

LCCA : ong mach canh chung trai RCCA : ong mach canh chung
phai
RSA : ong mach di on phai

LSA : ong mach di

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

Giai phau hoc : ti mau phoi

do 3 nguon
Ong ong mach
M bang he chu phoi (APCAs)
Mang ong mach phe quan hoac ong
mach mang phoi

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

Giai phau hoc cac ton

thng khac
Thong lien that : quanh mang hoac vung pheu
MC ci nga
H van MC : do dan vong van hoac VNTMNT
Cung MC ben phai : 26-50% trng hp
Nh phai : dan va ph ai ; nh trai : bnh
thng
TLN2 hoac lo bau duc thong thng : 50%
trng hp
That phai : ph ai ; that trai : thng bnh
thng, oi khi ph ai
ng dan truyen : bnh thng

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

Giai phau hoc : ton thng

phoi hp
Ton tai TMC tren trai o vao xoang vanh
Noi lien bat thng TMP ban phan hay
toan phan
Hep hay khong lo van 3 la
Kenh nh that
Hoan v M
Hai lan bat tng hp (hoan v M co sa
cha)
Tim ben phai

10

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

Bieu hien lam sang

Trieu chng t luc mi sinh
Giam oxy mau va tm (cyanosis)
Trieu chng giam oxy mau tuy thuoc
OM ln nho hay tuan hoan bang he
Rat hiem : trieu chng suy tim do tang
tuan hoan phoi (OM ln hoac nhieu
mach bang he)

11

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

Trieu chng thc the

Tm nhe luc mi sinh, tang dan khi OM
ong
Mach ngoai vi va HA thng bnh thng
Mom tim thng v tr bnh thng ; tim
khong ln
T1bnh thng ; T2 n oc
Thng khong co ATTThu ; co the co AT
lien tuc do OM hay do mach bang he MCMP (nghe ro sau lng)

12

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

ECG
Giong T chng Fallot
That phai ph ai ; truc QRS lech phai
b/n co nhieu mach bang he : ph ai hai
that va ln nh trai
ECG/khong lo van MP kem VLT nguyen
ven : giam san that phai

13

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

Xquang ngc sau trc

Tim hnh guoc (coeur en sabot)
Cung MC ben phai (26-50% trng hp)
Mach mau phoi dang li (reticular
appearance) do tuan hoan bang he
KLVMP TLT kieu 1 : ong mach phoi trung
tam dan ln khi co OM ln

14

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

Xquang ngc sau trc

TL : Emmanouilides GC et al. Heart Diseases in Infants, Children and Adolescent, William

& Wilkins 5th ed 1995, p. 985

15

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

Sieu am tim
Giup gi y chan oan (MC ci nga -> 5
benh)
Khong giup mo ta ro MP hoac nguon
mau nuoi phoi; do o thng can thong
tim chan oan (diagnostic catheterization)
Mat cat tren hom c : giup thay MP,
OM va mach bang he, s hp lu MP

16

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

Mat cat canh c truc ngang, ngang van

MC:
van MC khong lo, than MC nho

TL : Atlas Sieu am tim 2D va Doppler mau. NXB Y hoc 2000, p.55

17

Mat cat tren hom c truc

doc :
ong mach bang he xuat
phat t MC

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

TL : Atlas Sieu am tim 2D va Doppler mau. NXB Y hoc 2000, p.56

18

Mat cat tren hom c truc

ngang :
khao sat Doppler xung dong chay

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

do phau thuat Blalock Taussig

TL : Atlas Sieu am tim 2D va Doppler mau. NXB Y hoc 2000, p.56

19

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

Thong tim va chup mach

Ch can khi khong ro MP
Phau thuat tam thi : co the khong can
thong tim khi thay ro MP
Phau thuat triet e : thong tim la ch
nh bat buoc

20

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

Chup chon loc ong mach

bang he
t MC xuong

TL : Emmanouilides GC et al. Heart Diseases in Infants, Children and Adolescent, William

& Wilkins 5th ed 1995, p. 992

21

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

Chan oan phan biet (1)

The tm sm, khong suy tim :
T chng Fallot the nang
Hoan v M
Khong lo van 3 la
TP 2 ng ra kem hep hay khong lo van
MP
Tam that oc nhat kem hep hay khong lo
van MP
NLBT/TMP toan phan kem nghen TMP

22

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

Chan oan phan biet (2)

The t tm kem suy tim :
TLT
Con OM ln
Kenh nh nhat
TP 2 ng ra hoac Tam that oc nhat
khong kem hep MP
Than chung ong mach
NLBT/TMP toan phan khong kem nghen TMP

23

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

Tien trien t nhien

Tuy thuoc vao ti mau phoi
OM bt -> tm nang (neu khong co mach
bang he)
-> t vong
Tuan hoan bang he +++ -> suy tim

24

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

ieu tr
KLVMP TLT kem hp lu MP va OM :
can phau thuat khan (nguy c bt OM)
Khi 1 hay ca 2 MP khong c cung cap
mau bi OM : can chup mach
Phau thuat tam thi : mau len phoi
Phau thuat nhieu giai oan (multi-stage
operation)
Hp nhat mach phoi (unifocalization)

25

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

KLVMP-TLT : chup MC
trc phau thuat va sau phau
thuat

TL : Emmanouilides GC et al. Heart

Diseases in Infants, Children and
Adolescent, William & Wilkins 5th ed
1995, p. 985

26