Long-term results after biventricular repair for double-outlet right ventricle

Purpose: The aim of this study was to review our surgical strategy and long-term follow-up results in patients with double-outlet right ventricle who underwent biventricular repair. Methods: From 1995 to 2003, a total of 15 patients (12 boys, 3 girls; mean age, 3.3 years) underwent biventricular repair for DORV. Among these patients, 8 patients received palliative procedures before definitive repair. According to the location of the VSD, these patients were divided into four groups: Five patients had subaortic VSD, 6 had subpulmonary VSD (Taussig-Bing type), 2 had doubly committed VSD, and 2 had remote VSD. In the subaortic, doubly committed, and non-committed VSD groups, the intraventricular baffle procedure to direct blood from the left ventricle to the aorta combined with right ventricular outflow tract (RVOT) reconstruction with or without extracardiac conduit implantation was performed. In the Taussig-Bing group, 4 patients underwent VSD closure and the Jatene operation, while 2 patients underwent VSD closure and the Mustard operation. Results: There was one early death, and no late death. One patient in the remote VSD group had complications of complete heart block and underwent pacemaker implantation. Patients were monitored for a mean period of 107.3 ± 31.0 months (range, 66-164 months). Two patients underwent re-operation during the follow-up period. All the survivors were in New York Heart Association (NYHA) class I or II. Conclusion: We suggest that most of DORV patients with adequate size of both ventricles can be successfully managed using biventricular repair. The initial survival was high, and long-term follow-up results were acceptable.