Decades of single-center experience in managing 274 double-outlet right ventricle patients: Biventricular repair suitable for more complex forms?

This study sought to investigate the long-term outcomes of DORV, focusing on identifying specific subtypes capable of achieving successful BVR with favorable results. A retrospective review was conducted of 274 DORV patients followed at a single center from 1999–2023. After exclusions, 238 patients were analyzed: 97 underwent SVP and 141 underwent BVR. Outcomes were assessed using Kaplan-Meier survival and freedom from reintervention estimates, with competing risk analysis for survival, transplant, or death. Median follow-up was 11.9 years. Subaortic and subpulmonary VSDs were more common in the BVR group, while noncommitted VSDs and features suggestive of heterotaxy (e.g., persistent left SVC, complete AVSD) were more frequent in SVP. BVR tended towards improved transplant-free survival (8% vs. 16% transplant or death, p=0.06). At 40 years post-repair, estimated outcomes were 70% survival, 8% transplant, and 20% mortality for BVR, compared to 45%, 18%, and 37% 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=0.025). Freedom from reintervention was also comparable across subtypes. Our study showed BVR was associated with improved outcomes, including higher survival and lower rates of both mortality and transplant, compared to SVP. Decades of experience at our institution demonstrates the feasibility of BVR in complex DORV subtypes such as noncommitted and subpulmonary VSD.