Surgical management of double-outlet right ventricle.

From 1977 to 1983, 32 consecutive patients, ranging in age from 15 days to 24 years, underwent operations for double-outlet right ventricle. Twenty patients had a palliative operation either to increase (12 cases) or to reduce (eight cases) pulmonary blood flow: Ten of them have subsequently undergone total repair, and in another six correction was delayed because of possible incremental operative risk factors, such as multiple ventricular septal defects or the need for an extracardiac conduit. Four patients with multiple, complex associated intracardiac anomalies are currently considered to have uncorrectable defects. A total of 22 patients underwent correction either primarily (12) or after palliation (10). Intraventricular tunneling was performed in 16 patients with a subaortic ventricular septal defect and in one with a doubly committed ventricular septal defect. Seven of these had pulmonary stenosis and five had reconstruction of the right ventricular outflow by means of a patch (three) or a conduit (two); among this group, five also had enlargement of the ventricular septal defect. In three patients with a subpulmonary defect and in one with a remote ventricular septal defect, all of them without pulmonary stenosis, total repair was achieved by a Senning, a Mustard, or an arterial switch operation. Finally, the only patient with atrioventricular discordance and pulmonary stenosis had insertion of a left ventricle-pulmonary artery conduit. No operative deaths were observed after palliation, but one patient died of intrapulmonary hemorrhage after total repair (4.5%). Major postoperative complications included detachment of the ventricular septal defect patch in one patient and late progression of pulmonary vascular obstructive disease in another. No late deaths have been recorded. Surgical repair of double-outlet right ventricle can be accomplished with gratifying early and late results, the risk of operative death being below 5%. The outcome in patients with subaortic ventricular septal defect appears particularly favorable, despite the extensive intracardiac procedures required for total correction. An early intervention is recommended to prevent development of pulmonary vascular obstructive disease and to avoid massive cardiac hypertrophy and fibrosis, which may cause late rhythm disturbances and impede the intracardiac repair.