Myocardial ischemia in patients with pulmonary atresia and intact ventricular septum.

Children who die after operation for pulmonary atresia and intact ventricular septum may have myocardial ischemia. The relation between histologic evidence of myocardial ischemic injury and the presence of a right ventricle to coronary artery fistula, coronary artery dysplasia and operation in 17 autopsy specimens was assessed. Age at death ranged from 1 day to 16 years (median, 11 days). Of the 17 hearts, 6 (35%) had right ventricle to coronary artery fistulas, 5 of which had coronary artery dysplasia. In three cases, there was segmental or complete absence of a coronary artery. Ischemia was present in four of these six hearts, two of which had right ventricular outflow reconstruction. Six of the 11 hearts without right ventricle to coronary artery fistulas also had myocardial ischemia. Of these six cases, four had right ventricular outflow reconstruction and two had shunt operations. Death occurred from 1 to 8 days (mean 3) after operation. Hearts with pulmonary atresia and intact ventricular septum may have myocardial ischemia with or without either right ventricle to coronary artery fistulas or coronary artery dysplasia. Myocardial ischemia may occur after right ventricular outflow reconstruction or shunt operations. Thus, myocardial ischemia occurs commonly in patients with pulmonary atresia and intact ventricular septum and is not always related to coronary abnormalities or operation.