Repair of double-outlet right ventricle. An analysis of 62 cases

Sixty-two patients with double-outlet right ventricle (DORV) underwent complete intracardiac repair between 1967 and July, 1978. Five patients (three deaths) with DORV and complete atrioventricular (AV) canal are the subject of a separate report and are not discussed further here. Twenty-eight patients had relatively uncomplicated DORV (subaortic ventricular septal defect [VSD], doubly committed VSD, or 1-malposition of the aorta with subaortic VSD) with or without pulmonary stenosis. Two (14%) of 14 died after a completely intraventricular repair, and one (12%) of eight after repair including use of a valved external conduit. Use of a transannular patch in this group was an incremental risk factor, five (83%) dying among six treated in this way. Anterior enlargement of a restrictive or unfavorably located VSD did not increase risk. A noncommitted VSD was an incremental risk factor, two (50%) of four dying after repair. No instances of complete heart block occurred in these groups. Two late deaths occurred in the uncomplicated group, from persisting servere pulmonary hypertension. The late results were good in the remainder and in the two surviving patients with noncommitted VSD. After repair of the Taussig-Bing type of DORV, eight deaths (32%) occurred among 25 patients. Complete heart block developed in two patients. In the Taussig-Bing type of DORV, the 6 year actuarial survival rate was only 38%. Most late deaths were related to improtant pulmonary vascular disease. The surgical technique that has evolved for the basic tunnel repair in the various types of DORV is described.