Long-Term Surgical Outcomes in Double Outlet Right Ventricle Based on Detailed Anatomical Sub-Typology.

OBJECTIVES: Long-term surgical outcomes for double outlet right ventricle (DORV) continue to pose challenges for most paediatric cardiac centres. This study aimed to investigate the mid- to long-term outcomes of a large cohort of DORV patients. METHODS: DORV patients who underwent surgery guided by a refined anatomical sub-typology in our centre between August 2001 and December 2023 were retrospectively reviewed. The primary end-point was cardiac mortality and the secondary end-point was unplanned surgical reintervention. RESULTS: A total of 1135 patients were included in the study. Among them, 824 patients (72.6%) underwent biventricular repair, 258 (22.7%) received single-ventricle procedures, and 53 (4.7%) underwent palliative surgeries. The median age at surgery was 25.0 months [IQR: 9.0-72.0]. In-hospital deaths occurred in 14 cases (1.2%). Over a mean follow-up period of 8.9 years, the estimated overall survival rates at 5, 10, and 15 years were 96.3% [95% CI, 95.1-97.5], 93.0% [95% CI, 91.6-95.2], and 92.7% [95% CI, 87.7-96.9], respectively. Second end-point-free survival rates at the same time points were 95.2% [95% CI, 94.8-95.7], 89.5% [95% CI, 88.7-90.3], and 82.1% [95% CI, 80.7-83.5], respectively. Transposition of the great arteries-type (TGA-type) anatomy and palliative procedures were independently associated with increased mortality. CONCLUSIONS: Optimal outcomes in DORV can be achieved through precise anatomical evaluation and tailored surgery. TGA-type anatomy and palliative procedure were key risk predictors.