Multimodality cardiac imaging of a double chambered right ventricle with intrapulmonary shunting: a case report

BACKGROUND: Double chambered right ventricle (DCRV) is a relatively rare congenital heart disease, characterized by the abnormal division of the right ventricle into a high-pressure inlet and low-pressure outlet by anomalous muscle bundles. Extra-cardiac right-to-left shunts may present with clinical symptoms in adulthood and should be sought in patients with previous cavo-pulmonary shunt procedures. CASE PRESENTATION: We report a case of DCRV in a 29 year old Caucasian male presenting in adulthood with a right-to-left shunt secondary to venous collaterals, following cavopulmonary anastomosis for congenital pulmonary atresia and hypoplastic right ventricle. CONCLUSION: Multimodality cardiac imaging using echocardiography, cardiac CT, cardiac MRI and cardiac catheterization is often required for complete characterization of complex congenital heart anomalies in adulthood.