Current risks and protocols for operations for double-outlet right ventricle. Derivation from an 18 year experience.

Recent follow-up of 127 patients having repair of double-outlet right ventricle during an 18 year experience (1967 to July, 1984) indicated an overall actuarial survival rate at 12 years of 38%. However, multivariate analysis supported by contingency tables indicated that currently the early (2 week) survival rate after the intraventricular tunnel repair for double-outlet right ventricle with subaortic ventricular septal defect in 6-month-old infants is 99% and the 10 year survival rate 97%. Reoperation of the tunnel repair was rarely required (one of 56 patients), and the functional results were excellent. Results were similar in patients with doubly committed ventricular septal defect, except that two of 10 patients developed typical discrete localized subaortic stenosis late postoperatively. Early and late results in patients with double-outlet right ventricle and subpulmonary ventricular septal defect were poor when an atrial switch operation was part of the repair; when an intraventricular tunnel repair between the left ventricle and aorta was combined with a right ventricular-pulmonary arterial conduit, no early deaths occurred, but three patients died late postoperatively. Several techniques of repair of double-outlet right ventricle with noncommitted ventricular septal defect have provided only a 22% overall 10 year survival rate. These data are used to derive inferences as to optimal treatment protocols.