An unoperated 45-year-old man with double-inlet single left ventricle, transposed great arteries, ventricular septal defect, and pulmonary stenosis presented with mitral and pulmonary valvular vegetations unresponsive to antibiotic therapy. The initial diagnosis was made with echocardiography, and the morphology was delineated with computed tomography and magnetic resonance imaging. The patient underwent mitral valve replacement with a mechanical valve and pulmonary valve vegetectomy. He recovered well and he is currently well two years after the operation.
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