Surgical management of double outlet right ventricle with outflow obstruction: a systematic review of biventricular strategies.

BACKGROUND

Double outlet right ventricle is a heterogeneous congenital defect in which both great arteries arise predominantly from the right ventricle. Several operative strategies exist, but contemporary paediatric outcome data have not been systematically synthesised.

MATERIAL AND

METHODS

A systematic search of PubMed, Embase, Scopus, the Cochrane Library, and medRxiv (2000-2025) identified cohort studies and case series including ≥10 children undergoing biventricular repair strategies reported in double outlet right ventricle cohorts, including intraventricular rerouting procedures, root-based realignment techniques, and neonatal pathway operations such as the Yasui procedure. Two reviewers independently screened studies, extracted anatomical and operative data, and assessed risk of bias. Early mortality and 10-year survival were pooled using random-effects models with logit transformation.

RESULTS

Thirteen studies (413 children; median follow-up ∼7 years) met inclusion criteria. Early mortality ranged from 0% to 6.8%. Pooled early mortality was 2.7% for Rastelli, 3.6% for REV, and 5.8% for Nikaidoh/Bex operations. Ten-year survival exceeded 90% across all major strategies. Conduit replacement was frequent after Rastelli and Nikaidoh/Bex repair. REV avoided a conduit but required right ventricular outflow tract reinterventions in about one-quarter of patients. Nikaidoh/Bex procedures provided durable left ventricular outflow but showed 19-35% conduit failure. Yasui repairs achieved excellent neonatal survival but required frequent conduit replacement.

CONCLUSIONS

Contemporary double outlet right ventricle repair provides excellent long-term survival with early mortality <6%. Procedure selection should consider ventricular septal defect position, risk of postoperative left ventricular outflow tract obstruction, coronary anatomy, and patient size.