Repair of double-outlet right ventricle: early and late results

Fifty-seven patients underwent reparative surgery between 1973 and 1981 for various forms of double-outlet right ventricle. Pulmonic stenosis was present in 35 (61%), 15 (43%) of whom required a valved conduit for repair. Subaortic stenosis was present in six patients (11%). Hospital mortality was 12% (7/57), with three of the deaths among five patients with associated defects of the atrioventricular valves. During a mean follow-up of 3.6 years (range 2 months to 9 years), there have been no late deaths. Repeat cardiac catheterization in 28 of 50 survivors (56%) showed a residual ventricular septal defect (Qp/Qs greater than 1.5) in six patients, residual pulmonic stenosis (gradient greater than 35 mm Hg) in six patients, and subaortic obstruction (gradient greater than 50 mm Hg) in two patients: The indications for reoperation in 12 of 50 survivors was a residual ventricular septal defect in five patients, atrioventricular valve regurgitation in three patients, subaortic or subpulmonic obstruction in three patients, and aortic valve regurgitation in one patient. None of the patients died after repeat surgery. Our results indicate that surgical risk is related to the anatomic complexity of the malformation. Although a good hemodynamic outcome was achieved at initial surgery in the majority of patients, a residual abnormality was present in 24% needing surgery. When present, significant defects can be repaired with low mortality and morbidity.