Predictors of outcome in adults with double outlet right ventricle
File(s)
Accepted version
Author(s)
Type
Journal Article
Abstract
Background
Double outlet right ventricle (DORV) encompasses a range of congenital heart defects. Although survival to adulthood has improved, late risks, particularly life-threatening arrhythmias and sudden cardiac death (SCD), persist.
Objectives
We evaluated long-term outcomes in an adult DORV cohort, including SCD risk and device therapy.
Methods
This multicentre, retrospective cohort study enrolled adults with DORV under follow-up at two UK tertiary centres from 2000 to 2023. Patients were classified by anatomic subtype and surgical strategy. The primary composite outcome was all-cause mortality, heart or heart-lung transplantation, aborted SCD, or appropriate implantable cardioverter defibrillator (ICD) shock.
Results
Among 254 patients (median age 20[IQR 17.6-29.2] years; 52.8% male), 61.4% had biventricular repair. Complex (n=90, 35.4%) and Fallot-type (n=79, 31.1%) DORV predominated. Over a median 12.4[7.4-17.3] years, 47(18.5%) patients died: heart failure (40.4%) and SCD (25.5%) were leading causes. Aborted SCD occurred in 4(1.6%) cases, 4(1.6%) underwent heart transplantation, and 10(4.0%) received appropriate ICD shocks. One in eight patients experienced ventricular tachycardia and one in three developed supraventricular tachycardia (SVT). In exploratory multivariable analysis restricted to patients with biventricular repair, older age, pulmonary hypertension and baseline QRS >180 ms were associated with the composite outcome.
Conclusions
Amongst adult survivors of DORV, late mortality due to heart failure and SCD was considerable, whereas transplantation was uncommon. In the biventricular-repair subgroup, older age, baseline QRS >180 ms and pulmonary hypertension were associated with adverse outcome in exploratory analysis; these findings require validation.
Double outlet right ventricle (DORV) encompasses a range of congenital heart defects. Although survival to adulthood has improved, late risks, particularly life-threatening arrhythmias and sudden cardiac death (SCD), persist.
Objectives
We evaluated long-term outcomes in an adult DORV cohort, including SCD risk and device therapy.
Methods
This multicentre, retrospective cohort study enrolled adults with DORV under follow-up at two UK tertiary centres from 2000 to 2023. Patients were classified by anatomic subtype and surgical strategy. The primary composite outcome was all-cause mortality, heart or heart-lung transplantation, aborted SCD, or appropriate implantable cardioverter defibrillator (ICD) shock.
Results
Among 254 patients (median age 20[IQR 17.6-29.2] years; 52.8% male), 61.4% had biventricular repair. Complex (n=90, 35.4%) and Fallot-type (n=79, 31.1%) DORV predominated. Over a median 12.4[7.4-17.3] years, 47(18.5%) patients died: heart failure (40.4%) and SCD (25.5%) were leading causes. Aborted SCD occurred in 4(1.6%) cases, 4(1.6%) underwent heart transplantation, and 10(4.0%) received appropriate ICD shocks. One in eight patients experienced ventricular tachycardia and one in three developed supraventricular tachycardia (SVT). In exploratory multivariable analysis restricted to patients with biventricular repair, older age, pulmonary hypertension and baseline QRS >180 ms were associated with the composite outcome.
Conclusions
Amongst adult survivors of DORV, late mortality due to heart failure and SCD was considerable, whereas transplantation was uncommon. In the biventricular-repair subgroup, older age, baseline QRS >180 ms and pulmonary hypertension were associated with adverse outcome in exploratory analysis; these findings require validation.
Date Acceptance
2026-09-21
Citation
Open Heart
ISSN
2053-3624
Publisher
BMJ Publishing Group
Journal / Book Title
Open Heart
Copyright Statement
Subject to copyright. This paper is embargoed until publication. Once published the Version of Record (VoR) will be available on immediate open access.
Publication Status
Accepted