Double-Chambered Right Ventricle-Case Report and Review of Literature

Double-chambered right ventricle (DCRV) is a rare condition seen in only 0.5-2.0% of all case of congenital heart disease (CHD). An isolated DCRV is very rare, while approximately 80-90% of DCRV cases are associated with various other congenital heart defects, with VSD, in particular, a perimembranous type VSD, being the most common. In DCRV right ventricle is separated into a proximal high-pressure and distal low pressure chamber. It can be caused either by the presence of anomalous muscle bundle (AMB), by hypertrophy of endogenous trabecular tissue, or occasionally by an aberrant moderator band. DCRV is characterised by intraventricular pressure gradients greater than 20 mmHg, turbulent flow patterns in the ventricle, and increased pulmonary blood flow. Currently the methods for detection of DCRV with VSD are: Colour echocardiography, Cardiac catheterization, Cardiac CT and Cardiac MRI. This anomaly is often diagnosed during childhood and adolescence, while very few are found in adults. Here, we are presenting an extremely rare case report of a 7 month old male child afflicted with symptomatic DCRV, unusually associated with a large apical muscular VSD.