The results of repair of double outlet right ventricle in 26 children are reported. Eighteen of these patients had subaortic ventricular septal defect (VSD), without pulmonary stenosis (PS) in 5 and with PS in 13. Another 8 patients had subpulmonic VSD, without PS in 6 and with PS in 2. All had concordant atrioventricular relationships. There were six early deaths and two late deaths of the 26 patients in our study. Of these, 5 patients had severe associated cardiac abnormalities, and 2 who died early had had an incision in the systemic (right) ventricle.