Tetralogy of Fallot with abnormal coronary artery: 1967 to 1977.

From 1967 to 1977, 25 of 250 children undergoing complete repair of tetralogy of Fallot had an abnormal coronary artery identified at operation. Eight (Group 1) had classical anomalous coronary artery, and 17 (Group 2) had a large aberrant artery in addition to a smaller, normally positioned artery. All 25 patients required surgical procedures different from those routinely employed in tetralogy of Fallot repair. These included a "tailored" ventriculotomy, double incision, and accessory right ventricle-to-pulmonary artery conduit. After identification of a parallel (smaller) coronary artery, the anomalous coronary artery was ligated in three. Two patients (Group 2) died following the operation, both with "tailored" incisions. These were two of the four with high residual right ventricular pressure. Survivors have done well clinically for at least 1 year. Postoperative catheterization has shown high residual right ventricular pressure in two patients and an insignificant residual ventricular septal defect in four patients none has required reoperation.