Left ventriculo-coronary communications in a double-outlet right ventricle with an intact ventricular septum

Double-outlet right ventricle with intact ventricular at 5 weeks of age (Fig. 1). Extensive communications septum is exceedingly rare [1,2]. We report on an between the left ventricle and the left coronary association of this rare congenital heart malformation arterial system were present (Figs. 1c and d). Cinewith left ventriculo-coronary communications that angiogram showed egress of the contrast from the left has not been described previously. ventricle during systole with filling of the left corA full term, 2.7 kg baby boy presented with central onary arterial branches. Both the left anterior decyanosis soon after birth. A single second heart sound scending and circumflex branches were dilated. In and a grade 3/6 ejection systolic murmur at the left contrast, the right coronary artery, filled during sternal border were the only significant cardiovascusystole by collaterals that communicate with the left lar findings. Chest roentgenogram revealed a carcoronary arterial system, has a normal calibre. Sigdiothoracic ratio of 0.6 with normal pulmonary nificant haemodynamic findings included a supravasculature. The electrocardiogram showed a frontal systemic left ventricular systolic pressure (108 QRS axis was 1908 and right ventricular hypermmHg as compared to a systemic systolic pressure of trophy, but with no evidence of myocardial is69 mmHg) and a mean pressure gradient of 10 chaemia. Echocardiographic findings were: situs mmHg between the left and right atrium. Balloon solitus, atrioventricular concordance, double-outlet atrioseptostomy enlarged the atrial septal defect from right ventricle with the aorta anterior and to the right 2.7 to 5.7 mm and reduced the inter-atrial mean of the pulmonary artery, valvar and subvalvar pulpressure gradient to 2 mmHg. The baby was dismonary stenosis. The atrial communication is restriccharged on the following day. tive, while the ventricular septum is intact. The mitral Ventricular septal defect constitutes an important valve, though hypoplastic, is competent. Coronary feature in double-outlet right ventricle malformations sinusoidal communications with the left ventricle was as it represents the outlet for the left ventricle. The suspected in view of a dilated left coronary artery and absence of a ventricular septal defect is a rarity. abnormal colour flow pattern on the left ventricular Proposed mechanisms included spontaneous closure epicardial surface. The echocardiographic findings of the ventricular septal defect and defective conal were confirmed by cardiac catheterization performed absorption [1]. With this unusual anatomy, the only outlet for the left ventricle is through an incompetent mitral valve and a non-restrictive atrial septal defect. *Corresponding author. Tel.: 1852-25-18-2629; fax: 1852-25-53In our patient, however, the mitral valve is fully 9491. E-mail address: xfcheung@hkucc.hku.hk (Y.F. Cheung). competent albeit hypoplastic and decompression of