Year: 2026
Type: article
Study design: Observational Study
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 anatomical 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 to 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 to 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. 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.
Source: Open Heart
Authors: Omar Mubarak, Andrew Constantine, Saadiq Moledina, Aleksander Kempny, Paul F Clift, Lucy Hudsmith, Sarah Bowater, Nada Al-Sakini, Sayqa Arif, Phil Botha, Natasha Khan, Timothy J Jones, Christopher Bond, Andreas Hoschtitzky, Seshika D Ratwatte, Madiha Iqbal, Sara Thorne, Sarah Ghonim, Ee Ling Heng, Sonya Babu-Narayan, Michael Gatzoulis, Wei Li, Konstantinos Dimopoulos
Language: English
Subfield: Cardiology and Cardiovascular Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $2,391
OpenAlex ID: W7221230821