Double outlet right ventricle with infective endocarditis

Double outlet right ventricle (DORV) is an uncommon congenital heart disease with a poor prognosis. We report a rare case of a girl with untreated DORV who survived until adolescence, but then developed infective endocarditis with florid complications and succumbed to it. Although infective endocarditis is seldom encountered in children in developed countries, a high index of suspicion is required for those with congenital heart disease. The roles of cross-sectional imaging are demonstrated, including the usefulness of magnetic resonance imaging not only in evaluating congenital heart disease, but also in detecting incidental lesions in the extracardiac structures.