Background: Our institution has adopted a protocol of primary repair for all patients with double-outlet right ventricle.
Methods: Since May 1989, 24 consecutive neonates and infants with double-outlet right ventricle and atrioventricular concordance (median age, 4 months) underwent anatomic biventricular repair. One patient (4%) received prior pulmonary artery banding but was still repaired as a neonate at 22 days of age. Twelve patients had a subaortic ventricular septal defect (VSD), 5 patients a subpulmonary VSD, 3 patients doubly committed VSD, and 4 patients a noncommitted VSD. Sixty-nine of 72 associated lesions were repaired simultaneously. Four types of repairs were used: intraventricular rerouting in 16 patients, arterial switch operation with VSD closure into the pulmonary artery in 4 patients, Rastelli-type repair with extracardiac conduit in 3 patients, and the Damus-Kaye-Stansel repair with concomitant repair of aortic arch obstruction in 1 patient. Ventricular septal defect enlargement was necessary in 15 patients. Repair of subpulmonary stenosis and of subaortic stenosis was carried out in 13 and 4 patients, respectively. Three patients underwent simultaneous repair of aortic arch obstruction with no mortality. Two of the patients with noncommitted VSD had simultaneous repair of complete atrioventricular canal and repair of severe pulmonary venous obstruction.
Results: The perioperative mortality was 8% (2 patients, and there was one late death (4%). Two patients (9%) underwent early successful reoperations (5 and 8 weeks postoperatively). The two reoperations were for residual VSD (1 patient) and severe mitral regurgitation (1 patient). All 21 survivors are alive at a mean follow-up of 40 months (range, 7 months to 6 years). The estimated 5-year actuarial survival is 88%, with no deaths after 2 months postoperatively. Ninety-five percent of long-term survivors have no restriction of physical activities because of cardiac status and are receiving no cardiac medications.
Conclusions: An institutional protocol of early anatomic biventricular repair of double-outlet right ventricle in infants and neonates achieves excellent survival, making palliative operations unnecessary. Associated lesions should be repaired simultaneously. The complexity of these malformations requires a highly individualized and flexible surgical approach.