BIVENTRICULAR REPAIR FOR DOUBLE-OUTLET RIGHT VENTRICLE - RESULTS AND LONG-TERM FOLLOW-UP

Background-The purpose of the present study was to define the optimal management and to identify the risk factors for death and repeat opera tion in patients with double-outlet right ventricle. Methods and Resul ts-From 1985 through 1996, 154 consecutive patients underwent biventri cular repair for double-outlet right ventricle. The presence of bilate ral infundibular structures was the major inclusion criteria (142 pati ents). According to the relationship of the ventricular septal defect (VSD) to the great arteries, there were 86 patients with a subaortic V SD (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 proce dures. At repair, the median age was 10 months, and the median weight was 6.5 kg. Two main types of repair were used: intraventricular baffl e repair (n=115) and arterial switch operation with VSD-to-pulmonauy a rtery baffle (n=39). There were 14 hospital deaths (9%; 70% confidence limit [CL], 7% to 12%). The only significant risk factor for early de ath was the presence of congenital mitral valve anomalies (P=0.02). Tw enty-eight patients (18%) required 39 repeat operations. The repeat op eration 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 cons tituted a low-risk group for global death (P=0.008). The median follow -up was 52 months. Ten-year actuarial survival and survival with freed om from repeat operation rates were 86% and 62% (70% CL, 83% to 89% an d 54% to 70%), respectively. Conclusions-Long-term survival with good quality of life can be achieved after either 1- or 2-stage repair of t his complex anomaly.