Heart Vessels (2002) 17:8385

Springer-Verlag 2002

CASE REPORT

Tadahiko Ito Tadashi Okubo

A neonate with mitral stenosis due to accessory mitral valve, ventricular septal defect, and patent ductus arteriosus: changes in echocardiographical ndings during the neonatal period

Received: May 30, 2002 / Accepted: September 6, 2002

Abstract A female neonate with mitral stenosis due to accessory mitral valve with ventricular septal defect and patent ductus arteriosus is described. She was referred to our hospital because of neonatal asphyxia. Asphyxia was improved by ventilator support, but rapid deterioration of respiration with pulmonary congestion and hemorrhage appeared 8 days after birth. Echocardiography revealed an accessory mitral valve attached to the anterior mitral leaet with a perimembranous ventricular septal defect and patent ductus arteriosus. Although there were no echocardiographical ndings indicating mitral stenosis on admission, the mitral stenosis blood ow patterns were detected by color and pulsed Doppler examination performed on the eighth day after admission. Transaortic resection of accessory mitral valve tissue was performed with patch closure of the ventricular septal defect and ligation of the ductus arteriosus 35 days after birth. After operation, pulmonary congestion and hemorrhage were improved. Postoperative echocardiography showed complete resection of the accessory mitral valve and no mitral insufciency. We concluded that the combination of the accessory mitral valve and left-to-right shunt due to ventricular septal defect or patent ducturs arteriosus might have led to a critical hemodynamic condition due to relative mitral stenosis in the neonatal period with the decrease in pulmonary vascular resistance. Key words Accessory mitral valve Mitral stenosis Neonate Echocardiography

Introduction
Accessory mitral valve is an abnormal tissue attached to the mitral valve apparatus including leaets, orice, chordae, and papillary muscles. Most cases of accessory mitral valve are associated with other congenital heart anomalies such as transposition of the great arteries.1 Although it has been reported that accessory mitral valve caused left ventricular outow tract obstruction or stenosis,25 no case has been described in which accessory mitral valve caused mitral stenosis. We report on a neonate with critical and relative mitral stenosis due to accessory mitral valve associated with patient ductus arteriosus and ventricular septal defect.

Case report
A female neonate, weighing 3386 g, was delivered at 36 weeks of gestation. She was referred to our hospital because of neonatal asphyxia. On admission, echocardiography revealed a perimembranous ventricular septal defect, patent ductus arteriosus, and an accessory mitral valve attached to the anterior mitral leaet. The diameter of the ventricular septal defect was 6 mm and that of the ductus arteriosus was 4 mm. The shunt ow through the ventricular septal defect and ductus arteriosus was bidirectional because of the pulmonary hypertension. Although the accessory mitral valve caused mild left ventricular outow stenosis, mitral stenosis was not detected. Color Doppler examination did not show a mosaic signal in the mitral blood ow and pulsed Dopper examination did not show a mitral stenosis pattern of left ventricular blood inow (Fig. 1). Asphyxia was improved by ventilator support and she was extubated. However, rapid deterioration of respiration with pulmonary hemorrhage appeared 8 days after birth, so that ventilator support was started again. Chest radiography revealed marked pulmonary congestion. Two-dimensional echocardiography performed 8 days after admission revealed marked dilation of the left atrium and color Doppler examination showed mosaic ow signals in left ventricular inow and outow.

T. Ito (*) Department of Pediatrics, Nakadori General Hospital, 3-15 Misonocho, Minami-dori, Akita 010-8577, Japan Tel. 81-188-33-1122; Fax 81-188-31-9418 e-mail: syomu@meiwakai.or.jp T. Okubo Department of Cardiovascular Surgery, Nakadori General Hospital, Akita, Japan

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The peak blood ow velocity of left ventricular inow obtained by continuous wave Doppler examination was accelerated to 2.2 m/s (Fig. 2). The peak blood ow velocity of the left ventricular outow was 2.0 m/s (Fig. 2). The accessory mitral valve disturbed complete opening of the anterior mitral leaet by touching the interventricular septum during diastole and caused mitral stenosis (Fig. 3). From these echocardiographical ndings, we thought that mitral stenosis due to the accessory mitral valve and left-to-right shunt due to the ventricular septal defect and patent ductus arteriosus led to the marked pulmonary congestion and hemorrhage. Transaortic resection of accessory mitral valve tissue was performed with patch closure of the ventricular septal defect and ligation of ductus arteriosus 35 days after birth. After operation, pulmonary congestion and hemorrhage were improved. Postoperative echocardiography showed complete resection of the accessory mitral valve

and no mitral insufciency (Fig. 4). Both of the mosaic ow signals in the left ventricular outow and inow were diminished in the color Doppler examination and the peak blood ow velocity of transmitral ow decreased to 0.8 m/s (Fig. 4).

Discussion
Accessory mitral valve is a rare anomaly and is usually found as a complication with other congenital heart diseases.1 Accessory mitral valve was reported to induce critical subaortic stenosis necessitating operation during the neonatal period,24 but mitral stenosis due to this disease has not been reported. In the present patient, mitral stenosis was not detected by echocardiography on admission, but the transition from bidirectional shunt to left-to-right shunt in the blood ow through the ventricular septal defect and ductus arteriosus increased transmitral blood ow and led to relative mitral stenosis. We thought that the relative mitral stenosis due to the accessory mitral valve caused the rapid elevation of left atrial pressure and led to her critical condition including pulmonary congestion and hemorrhage. The exact ratio between pulmonary and systemic ows of blood (Qp/Qs) was unclear because cardiac catheterization was not done. Left ventricular end-diastolic diameter obtained by echocardiography was 2.0 cm, so the increase in the left ventricular volume owing to the left-to-right shunt was low despite the marked dilation of the left atrium. Therefore, we thought that Qp/Qs in the present patient was less than 2.0, and the deterioration of her hemodynamic state was attributable mainly to mitral stenosis due to the accessory mitral valve, rather than to the large left-to-right shunt. One of the mechanisms of the relative mitral stenosis in the present patient seemed to be the morphological characteristics of the accessory mitral valve. Because the accessory mitral valve was relatively large for the left ventricle and it was located between the anterior leaet and the interventricular septum, it disturbed the complete opening of the anterior leaet. This led to an increase in the transmitral blood ow due to the left-to-right shunt,

Fig. 1. Transmitral ow pattern obtained by pulsed Doppler echocardiography on admission. Pulsed Doppler examination showed no mitral stenosis ow pattern. E, transmitral ow during early diastolic lling; A, transmitral ow during atrial contraction

Fig. 2. Doppler echocardiography performed on the eighth day after admission. Continuouswave Doppler ow patterns of the left ventricular inow (left) and left ventricular outow (right). The peak blood ow velocities of left ventricular inow and outow accelerated to 2.2 and 2.0 m/s, respectively

85 Fig. 3. Two-dimensional echocardiographical ndings on the eighth day after admission. Accessory mitral valve attached to the anterior leaet caused left ventricular outow tract stenosis and mitral stenosis by disturbing the complete opening of the anterior leaet. AMV, accessory mitral valve; AO, aorta; LV, left ventricle; LA, left atrium; RV, right ventricle; RA, right atrium

Fig. 4. Postoperative echocardiogaphy. Complete resection of the accessory mitral valve without mitral insufciency are shown (upper left). Both of the mosaic ow signals in the left ventricular outow and inow were diminished in the color Doppler examination (upper right and lower right), and the peak blood ow velocity of transmitral ow decreased to 0.8 m/s (lower left)

by touching the interventricular septum at diastole. Transaortic resection of the accessory mitral valve with repair of the ventricular septal defect and patent ductus arteriosus seemed to be very effective in the present patient. We concluded that the combination of accessory mitral valve and left-to-right shunt due to ventricular septal defect or patent ductus arteriosus might lead to a critical hemodynamic condition due to relative mitral stenosis in the neonatal period with a decrease in the pulmonary vascular resistance. Especially when the accessory mitral valve is located between the anterior mitral leaet and the interventricular septum and is relatively large for the left ventricle, the possibility of relative mitral stenosis seems to be high. Serial observation of the blood ow patterns of left ventricular inow using Doppler echocardiography is important in these patients.

References
1. Garret HE Jr, Spray TL (1990) Accessory mitral valve tissue: an increasingly recognized cause of left ventricular out ow tract obstruction. J Cardiovasc Surg 31:225230 2. Calagro R, Santoro G, Pisacane C, Sarubbi B, Farina G, Pacileo G, Caianiello R (2000) Critical left ventricular outow tract obstruction due to accessory mitral valve tissue. Echocardiography 17:177180 3. Bilal MS, Oztunc F, Besikci R, Bilal S, Ozkara A, Olga R (1999) Accessory mitral valve tissue causing severe subaortic stenosis with dextrocardia in a premature newborn. J Thorac Cardiovasc Surg 47:252255 4. Meyer-Hetling K, Alexi-Meskishvili VV, Dahnert I (2000) Critical subaortic stenosis in a newborn caused by accessory mitral valve tissue. Ann Thorac Surg 69:19341937 5. Yasui H, Kado H, Tokunaga S, Kanegae Y, Fukae K, Masuda M, Tokunaga K (1993) Trans-ventricular septal defect approach for resection of accessory mitral valve tissue. Ann Thorac Surg 55:950 953

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