Total correction for tetralogy of Fallot with anomalous coronary artery.

Abstract

From 1955 through 1972, 27 patients with anomalous coronary artery crossing the right ventricular outflow tract in association with tetralogy of Fallot underwent total correction. Eighty percent of these were males, and 17 were less than ten years of age. All anomalies were first discovered at operation, and definitive repair was performed in all but two patients. The most common anomaly was an anterior descending branch arising from the right coronary artery (23 patients). For repair, a single right ventriculotomy was employed in 70 percent. Other procedures used successfully in selected patients were double ventriculotomy, right ventricular outflow conduit, and saphenous vein bypass graft. A palliative pulmonary valvotomy was used in one patient. Eight of the 27 patients died (30 percent); the mortality rate was 100 percent prior to 1961 and 17 percent thereafter. Most deaths resulted from myocardial infarction induced by injury to the anomalous coronary artery. Our experience suggests that in the management of this condition injury to the anomalous artery should be avoided and relief of pulmonary stenosis should be attempted through a ventriculotomy caudal to the anomalous artery or, if necessary, caudal and cephalad to the artery (and closure of the ventricular septal defect). If the stenosis cannot be relieved or if the ratio of right ventricular-to-left ventricular peak pressure is greater than 0.9 after bypass has been discontinued, the operation should be completed by placing a conduit from the caudal ventriculotomy to the main pulmonary artery.