Decades of single-center experience in managing 274 double-outlet right ventricle patients: Biventricular repair...

To investigate the long-term outcomes of double-outlet right ventricle (DORV), focusing on identifying specific subtypes capable of achieving successful biventricular repair (BVR) with favorable results.

A retrospective review was conducted of 274 DORV patients followed at a single center from 1999 to 2023. After exclusions, 238 patients were analyzed, including 97 who underwent single ventricle palliation (SVP) and 141 who underwent BVR. Outcomes were assessed using Kaplan-Meier survival and freedom from reintervention estimates, with competing risk analysis for survival, transplantation, or death.

The median follow-up was 11.9 years. Subaortic and subpulmonary ventricular septal defects (VSDs) were more common in the BVR group, while noncommitted VSDs and features suggestive of heterotaxy (eg, persistent left superior vena cava, complete atrioventricular septal defect) were more frequent in SVP. BVR tended toward improved transplantation-free survival (8% vs 16% for transplantation or death; P = .06). At 40 years post-repair, estimated outcomes were 70% survival, 8% transplantation, and 20% mortality for BVR, compared to 45%, 18%, and 37%, respectively, for SVP. Subgroup analysis showed equivalent survival in noncommitted, subpulmonary, and doubly committed types, with superior survival in subaortic DORV after BVR (100% vs 75%; P = .025). Freedom from reintervention was also comparable across subtypes.

Our study shows that BVR was associated with improved outcomes compared to SVP, including higher survival rates and lower rates of both mortality and transplantation,. Decades of experience at our institution demonstrates the feasibility of BVR in complex DORV subtypes, such as noncommitted and subpulmonary VSD.