Between January 1973 and February 1986, operations were performed on 120 consecutive patients having usual atrial arrangement (atrial situs solitus), concordant atrioventricular connection, and double-outlet right ventricle. The ages at operation ranged from 1 day to 44 years and the weights from 2.6 to 84 kg. Sixty-three patients had one or more palliative procedures. For those, the hospital mortality rate was 9.5%. Palliation was considered a definitive procedure in 13 patients. Ninety-three patients had a reparative operation, with a 26.9% early mortality rate. In the group who had complete correction, taken as a whole, the surgical outcome was significantly affected by the position of the ventricular septal defect and by the year of operation. The year of operation was the main factor that, by multivariate analysis, correlated significantly with the hospital mortality in those patients having a subaortic defect and spiraling great arteries (p < 0.05). No difference was found among this group for those patients having the morphologic characteristics of tetralogy of Fallot. The change-over point from the Mustard to the arterial switch procedures was the event with the greatest effect on hospital mortality in patients with a subpulmonary ventricular septal defect (p < 0.025). Two late deaths have occurred among the 21 patients who had palliative intervention only. Sixty of the 68 survivors with intracardiac repair have been followed up for a period of 2 to 184 months (median 44 months). There were five late deaths (8.3%). Eight patients underwent successful reoperation. All except three of the long-term survivors were in functional class I. Good early and long-term results can be anticipated for the intracardiac repair of double-outlet right ventricle when the ventricular septal defect is subaortic or doubly committed. The arterial switch operation has been demonstrated to be the optimal approach for double-outlet right ventricle with subpulmonary ventricular septal defect. Results in patients with noncommitted ventricular septal defect have remained poor.