The results of a surgical program for interrupted aortic arch.

Seventy-one patients with interrupted arch entered the Boston Children's Hospital between Jan. 1, 1974, and Jan. 1, 1987, of whom 63 underwent an operation. Type B was the most prevalent form of interrupted arch, and ventricular septal defect alone was the commonest coexisting cardiac anomaly. Among the 63, the 30-day and the 1-, 5-, and 10-year survival rates were 61%, 52%, 48%, and 47%, respectively. The mortality rate declined strikingly during the experience, and by multivariate analysis in patients with coexisting ventricular septal defect operated on in 1986, the probability of death within 2 weeks of repair was only 7%. Also, preoperative therapy became progressively more intense and more prolonged. The complication of left ventricular outflow tract obstruction developed in eight of the 33 patients undergoing repair of interrupted arch and of isolated ventricular septal defect. The time-related freedom from this complication was 97%, 78%, and 58% at 1 month, 1 year, and 3 years, respectively. Seven of the eight patients underwent a surgical procedure directed against the left ventricular outflow tract obstruction, and all have survived. Recurrent or persistent aortic arch obstruction became evident after repair in 15 patients and appeared more frequently and earlier after direct anastomosis than after tube graft repair. All patients had either reoperation or balloon dilation, but all were alive at follow-up. Most surviving patients are active and without symptoms. Inferences: An aggressive surgical program can result in survival and a good clinical state for at least 10 years after birth of over 40% of patients born with interrupted arch. Multiple anatomic bases account for the development of left ventricular outflow tract obstruction in about 50% of the patients undergoing repair of interrupted arch with coexisting ventricular septal defect. Repair by direct anastomosis combined with repair of the coexisting defect whenever possible is optimal therapy.