Complex Adult Congenital Heart Disease: A Rare Case of Dextrocardia with Double-outlet Right Ventricle

Abstract Double-outlet right ventricle (DORV) is a complex and rare congenital heart disease (CHD), which is usually symptomatic early in childhood and thus diagnosed early. Dextrocardia is another rare CHD that can occur either as an isolated cardiac abnormality or may be associated with other complex cardiac defects. However, the co-occurrence of DORV and dextrocardia is rare, especially in adult life. We present a 35-year-old Nigerian lady with situs inversus totalis and complex CHD, which included dextrocardia with multiple congenital intracardiac shunts, DORV occurring with L-malpositioning of the great vessels, atrial septal defect, and ventricular septal defect with partial anomalous pulmonary venous drainage, persistent left superior vena cava as well as severe pulmonary stenosis.