title: [Generalized lentigo in a case of hypertrophic cardiomyopathy with right ventricular outflow tract obstruction].
abstract: A case was reported in which generalized lentigo was associated with hypertrophic cardiomyopathy and right ventricular outflow tract obstruction. A 34-year-old man was admitted to our hospital for cardiac evaluation. He had been noted to have a heart murmur since his childhood and had had a diagnosis of pulmonary stenosis in his high school age. Physical examination revealed precordial murmurs and numerous lentigines over his whole body surface including palms and soles. Two-dimensional and M-mode echocardiography disclosed asymmetric septal hypertrophy, systolic anterior motion of the mitral valve (SAM) and systolic semiclosure of both semilunar valves. A systolic turbulent flow was recorded at the outflow tracts of both ventricles by pulsed Doppler technique. Cardiac catheterization demonstrated a pressure gradient of 13 mmHg across the right ventricular outflow tract. We considered that the present case was an atypical LEOPARD syndrome with predominant right ventricular outflow tract obstruction which was precisely evaluated by noninvasive techniques.
mesh_terms: D000328:Adult; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications* / Q000175:diagnosis; D004452:Echocardiography; D004562:Electrocardiography; D006346:Heart Septum / Q000000981:diagnostic imaging; D006801:Humans; D007911:Lentigo / Q000150:complications*; D008297:Male; D010701:Phonocardiography; D011666:Pulmonary Valve Stenosis / Q000150:complications*; D011859:Radiography