A Rare Case of Double Chamber Right Ventricle Presenting as Right Sided Heart Failure in a 61 Year Old Female

Abstract

Background: Double-chambered right ventricle (DCRV) is an uncommon congenital cardiac anomaly in which anomalous muscular bundles separate the right ventricle into proximal high-pressure and distal low-pressure chambers. Presentation in late adulthood may complicate the evaluation of right-sided heart failure because symptoms overlap with those of acquired cardiac conditions. Case Presentation: A 61-year-old woman with a history of congenital heart disease presented with progressive dyspnoea and generalised oedema affecting the lower limbs, abdomen and face. Examination identified raised jugular venous pressure, hepatomegaly, ascites and a pansystolic murmur at the tricuspid area. Laboratory investigations showed anaemia and hypothyroidism. Electrocardiography demonstrated atrial fibrillation with low-voltage complexes, while chest radiography showed cardiomegaly, right atrial enlargement and moderate pleural effusion. Transthoracic echocardiography demonstrated muscular partitioning of the right ventricle with subpulmonic obstruction and a reported Doppler pressure gradient of approximately 78 mm Hg. Severe tricuspid regurgitation, right atrial and right ventricular dilatation, moderate pericardial effusion and preserved left ventricular function were also documented. Discussion: The coexistence of intracavitary right ventricular obstruction and severe tricuspid regurgitation provides a plausible explanation for the pronounced manifestations of systemic venous congestion. However, the relative contributions of these abnormalities cannot be established from the available investigations. Conclusion: This case highlights the importance of examining right ventricular anatomy and haemodynamics in older adults with otherwise unexplained right-sided heart failure.