Long-term natural history and postoperative outcome of double-chambered right ventricle--experience from two tertiary adult congenital heart centres and review of the literature

Background Double-chambered right ventricle (DCRV) is a rare form of congenital heart disease. Little is known about the outcome during adult life. Here we report the combined experience of two tertiary Adult Congenital Heart Disease Centres and systematically review the published literature. Results 50 patients (32 female, median age 39years [IQR: 27;53]) with DCRV under follow-up could be identified. A retrospective review of medical records was performed. Almost all patients (96%) had ventricular septal defects as underlying diagnosis. Eight patients remained completely asymptomatic during follow-up and have been managed conservatively thus far. The remaining patients developed symptoms at a median age of 26years. Surgical correction was performed in 33 patients (median age at operation 27years). No residual intraventricular gradient was present at the latest follow-up in 91% of operated patients and functional class improved significantly with only 6 patients remaining in NYHA class 2. There was no early or late operative mortality and no patient required re-operation for DCRV during a median follow-up of 8years. Conclusions Contemporary adult DCRV patients have good survival prospects and low long-term morbidity. Despite occasionally presenting with considerable intraventricular gradients we could not identify any case of sudden death in our unoperated adult sub-population. Although asymptomatic adults may be encountered even with severe obstruction, symptom development is common during adult life. Cardiac surgery in this cohort is inherently low risk and offers good long-term haemodynamic and functional results justifying early intervention in consideration of the progressive nature of this rare congenital lesion.