INSTITUTIONAL EXPERIENCE WITH A PROTOCOL OF EARLY PRIMARY REPAIR OF DOUBLE-OUTLET RIGHT VENTRICLE

Background. Our institution has adopted a protocol of primary repair f or all patients with double-outlet right ventricle. Methods. Since May 1989, 24 consecutive neonates and infants with double-outlet right ve ntricle and atrioventricular concordance (median age, 4 months) underw ent anatomic biventricular repair. One patient (4%) received prior pul monary artery banding but was still repaired as a neonate at 22 days o f 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 wer e repaired simultaneously. Four types of repairs were used: intraventr icular rerouting in 16 patients, arterial switch operation with VSD cl osure into the pulmonary artery in 4 patients, Rastelli-type repair wi th extracardiac conduit in 3 patients, and the Damus-Kaye-Stansel repa ir with concomitant repair of aortic arch obstruction in 1 patient. Ve ntricular septal defect enlargement was necessary in 15 patients. Repa ir of subpulmonary stenosis and of subaortic stenosis was carried out in 13 and 4 patients, respectively. Three patients underwent simultane ous repair of aortic arch obstruction with no mortality. Two of the pa tients with noncommitted VSD had simultaneous repair of complete atrio ventricular canal and repair of severe pulmonary venous obstruction. R esults. The perioperative mortality was 8% (2 patients, and there was one late death (4%). Two patients (9%) underwent early successful reop erations (5 and 8 weeks postoperatively). The two reoperations were fo r 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. Conclusio ns. An institutional protocol of early anatomic biventricular repair o f double-outlet right ventricle in infants and neonates achieves excel lent survival, making palliative operations unnecessary. Associated le sions should be repaired simultaneously. The complexity of these malfo rmations requires a highly individualized and flexible surgical approa ch.