156 MITRAL VALVE PROLAPSE (MVP) SYNDROME IN DUCHENNE'S PROGRESSIVE MUSCULAR DYSTROPHY

Cardiac status was prospectively evaluated non-invasively in 20 male children, each with clinical, biochemical, electromyographic and muscle-biopsy evidence of Duchenne's progressive muscular dystrophy. A nonejection systolic click, confirmed by phonocardiography, was identified in 7 patients (3 with midsystolic murmur as well) and suggested the presence of MVP syndrome. Echocardiography supported this diagnosis in all 7 patients and in 4 others without systolic click. An abrupt midsystolic posterior motion (> 3mm) was noted in 5 patients, while a smooth, pansystolic, anteriorly concave (“hammock-like”) posterior motion of the leaflet > 3mm from the CD line was recorded for all 11 patients. Additional findings were: multiple sequence lines in 6 patients and posterior coaptation of the mitral leaflet near the left atrial wall in 6.