Long-term outcome in double-chambered right ventricle: a retrospective single-center study

Double-chambered right ventricle (DCRV) is a rare congenital heart defect (0.5%-2.0%), characterized by hypertrophied muscular tissue causing obstruction within the right ventricle (RV). DCRV is often associated with other congenital heart defects, such as ventricular septal defects. Although surgery is commonly performed, the long-term prognosis, after both surgical and conservative treatment, remains poorly studied. We investigated the long-term outcome of patients who were observed at a tertiary care center.

In this retrospective study, 90 patients were included (follow-up between 1980 and 2023). Demographic, clinical, echocardiographic, and electrical parameters were analyzed. Descriptive statistics were applied.

Sixty-three patients (70%) underwent surgical intervention, mostly at a young age (median age 3 years, IQR: 0.8-12.3 years); 7.9% required reintervention at a later stage (median 12 years, IQR: 8.0-14.5 years). Conduction disorders were observed in 42% (mainly right bundle branch block), and 6.8% experienced arrhythmias. Most patients remained clinically stable (83% NYHA class I). Echocardiography showed persistent RV hypertrophy in 35.7% of patients; valve regurgitations were mostly mild. Four patients (4.4%) died in a maximum follow-up time of 7 decades.

Patients with DCRV generally experience positive long-term outcomes and respond well to both surgical and conservative treatments. Although echocardiographic and electrical abnormalities may be present, most patients remain asymptomatic and clinically stable over time.