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

TL;DR

This study examines long-term surgical outcomes for patients with double outlet right ventricle (DORV) and finds that precise anatomical evaluation and tailored surgery improve survival rates.

Contribution

The study introduces a refined anatomical sub-typology for DORV surgery, showing improved long-term outcomes and identifying key risk factors.

Findings

Overall survival rates at 5, 10, and 15 years were 96.3%, 93.0%, and 92.7%, respectively.

TGA-type anatomy and palliative procedures were independently associated with increased mortality.

Abstract

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.

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.

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.

Optimal outcomes in DORV can be achieved through precise anatomical evaluation and tailored surgery. TGA-type anatomy and palliative procedure were key risk predictors.

Double outlet right ventricle (DORV) is a complex heart defect characterized by both the pulmonary artery and aorta originating predominantly or entirely from the right ventricle.

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