Compressed heart and mitral valve prolapse in a case of Duchenne's progressive muscular dystrophy with thorax...

We report on a 15 year old patient with Duchenne's progressive muscular dystrophy who demonstrated a narrowing of the left ventricular inflow and outflow tracts due to compression by a highly deformed thoracic spine. A systolic murmur (4/6) with thrill and a diastolic murmur (2/6) were heard, with these murmurs being louder in the expiratory phase as compared with the inspiratory phase. The second heart sound showed a paradoxical splitting. Echocardiograms revealed a compressed and narrowed left ventricle and a prolapsed mitral valve. The intensities of the heart murmurs changed synchronously with the chamber's narrowing due to respiration. A narrowed left ventricle occurring as a result of the compression by the deformed thoracic spine is thought to be the cause of these cardiac findings.