Pulmonary atresia with intact ventricular septum and ventriculocoronary communications: surgical significance.

The first stage of a repair of pulmonary atresia with intact ventricular septum (type I) was attempted in a 2-day-old infant. At surgery, decompression of the hypertensive small right ventricle was followed by a sudden loss of myocardial contractility and death. Postmortem examination revealed a fistula with a large orifice in the right ventricular infundibulum that communicated directly with the left main coronary artery. Severe hypertensive changes indicative of abnormally high perfusion pressure were noted in the distal left coronary artery branches. The clinical course suggests that the effect of relieving right ventricular outflow obstruction was a reduction of left main coronary artery blood flow, resulting in fatal intraoperative myocardial ischemia. This unusual case draws attention to the anomalous ventriculocoronary communications often present in pulmonary atresia and their potential for limiting a successful surgical repair.