Magnetic resonance imaging findings of isolated right ventricular hypoplasia

Isolated right ventricular hypoplasia, unassociated with severe pulmonary or tricuspid valvular malformation, is a rare congenital heart disease. Herein the authors report a case of a 6-year-old girl having isolated right ventricular hypoplasia treated with one and a half ventricular repair, and describe the cardiac magnetic resonance (CMR) imaging findings. CMR imaging revealed diffuse subendocardial or transmural late gadolinium enhancement of the right ventricle with a thin, smooth wall due to the absence of trabeculations. Noncompaction of the left ventricle was also demonstrated, although the relevance to isolated right ventricular hypoplasia is not obviously explicable.