Surgical management of double-outlet right ventricle associated with atrioventricular discordance

Twenty corrective operations for this unusual syndrome have been performed since 1965. All but two patients also had pulmonary stenosis; one of the exceptions had had pulmonary arterial banding. Two patients had situs inversus of the atria and viscera. In only three of the 20 patients (15 percent) was the apex of the heart positioned normally. Six patients had an entirely intracardiac repair; two early and two late deaths occurred in this group. The other technique, performed in 14 patients, involved insertion of an extracadiac conduit between a ventriculotomy in the morphologically left ventricle and the distal end of the proximally oversewn pulmonary artery; one operative and two late deaths occurred in this group. Heart block occurred in six patients, including two among the eight who had technically satisfactory intraoperative mapping of the His bundle. The intraventricular course of the bundle was not consistently positioned, being anterior to the septal defect in four and posterior in four. No hospital deaths have occurred in the last 13 operations; all except one of the survivors are in New York Heart Association Class I or II. Thus correction currently provides a good early results but leaves the ventricles in an inverted relationship both functionally and anatomically.