Background: The purpose of the present study was to define the optimal management and to identify the risk factors for death and repeat operation in patients with double-outlet right ventricle.
Methods and results: From 1985 through 1996, 154 consecutive patients underwent biventricular repair for double-outlet right ventricle. The presence of bilateral infundibular structures was the major inclusion criteria (142 patients). According to the relationship of the ventricular septal defect (VSD) to the great arteries, there were 86 patients with a subaortic VSD (56%), 45 patients with a subpulmonary VSD (29%), 18 patients with a noncommitted VSD (12%), and 5 patients with a doubly committed VSD (3%). Sixty-five patients (42%) had undergone previous palliative procedures. At repair, the median age was 10 months, and the median weight was 6.5 kg. Two main types of repair were used: intraventricular baffle repair (n = 115) and arterial switch operation with VSD-to-pulmonary artery baffle (n = 39). There were 14 hospital deaths (9%; 70% confidence limit [CL], 7% to 12%). The only significant risk factor for early death was the presence of congenital mitral valve anomalies (P = 0.02). Twenty-eight patients (18%) required 39 repeat operations. The repeat operation rate was higher in patients with associated VSD enlargement at baffle construction (n = 29; 19%) (P = 0.01). There were 6 late deaths (4%; 70% CL, 2% to 7%). Patients presenting with pulmonary stenosis constituted a low-risk group for global death (P = 0.008). The median follow-up was 52 months. Ten-year actuarial survival and survival with freedom from repeat operation rates were 86% and 62% (70% CL, 83% to 89% and 54% to 70%), respectively.
Conclusions: Long-term survival with good quality of life can be achieved after either 1- or 2-stage repair of this complex anomaly.