Multicentre study on late outcomes of biventricular repair of double outlet right ventricle

Abstract

Summary OBJECTIVES This retrospective multicentre study aims to present late surgical outcomes of Double Outlet Right Ventricle (DORV) coming from emerging countries. METHODS Mécénat Chirurgie Cardiaque brings to France for surgery selected children with simple and complex congenital diseases, including DORV. The patients are operated in nine hospitals, specialized in paediatric cardiac surgery. heart Data are collected from the MCC comprehensive database, with a strict post-operative follow-up. Patients included only biventricular repair of DORV with 2 viable ventricles. According to the classification of Eleventh Revision of the International Classification of Diseases (ICD11), DORV was defined as a congenital cardiovascular malformation in which both great arteries arise entirely or predominantly from the morphologically right ventricle. RESULTS From Jan. 1996 to Jan. 2022, 81 consecutive DORV biventricular repair (BVR) were performed. There were 6 subtypes of DORV divided in two groups:—DORV-committed VSD: DORV-VSD (n:25), DORV-Fallot (n:34), DORV-TGA (n:5);—and DORV-ncVSD: DORV-nc-VSD-no PS (n: 7), DORV-nc-VSD-PS (n: 5), and DORV-AVSD-PS (n: 5). Four Fontan patients were excluded. Three patients were lost to follow-up, (3.4%). The overall peri-operative mortality was 7.4% ± 2.6%, 6/81, [95% CI: 2.8% - 15.4%] ranging from 0% in DORV-AVSD-PS to 14% for DORV-ncVSD-no PS. The overall 10Y survival was 86%. The early mortality of DORV-non committed VSD at 5.9% ± 2.4%, (1/17) was similar to DORV-committed VSD at 7.8% ± 2.7%, (5/64), p: 0.79 There was a trend for optimal outcome for arterial switch operation and DORV-AVSD-PS repair. VSD enlargement was significantly more frequent in DORV-non committed VSD, 42% (5/12), p: 0.001. There were low numbers in complex groups. The number of Fontan is noticeably low. The fundamental anomaly and surgical challenge in DORV is the aorta standing entirely on the right ventricle. CONCLUSIONS Overall survival at 10Y was 86%. This study shows a trend towards satisfactory early and late outcomes in biventricular repair of simple DORV with committed VSD, compared to complex DORV with non-committed VSD.