Serial left ventricular wall measurements in Duchenne's muscular dystrophy

Abstract

In 19 patients with Duchenne's muscular dystrophy, left ventricular wall thickness in end-systole and end-diastole was determined serially every 12 months by echocardiography and compared with ventricular wall growth in normal subjects. In the normal subjects, left ventricular wall thickness increased linearly with increasing body surface area. A control group of wheelchair-bound patients with a variety of neurologic disorders, although not followed serially, had a distribution of end-diastolic wall thickness values similar to that of the normal subjects. In patients with muscular dystrophy, wall thickness increased linearly with respect to body surface area for some time and then began to thin. The time at which thinning began was not directly related to age, although it was more common in older than in younger patients. Those patients who died demonstrated marked deviation from normal wall growth. Free wall thinning is probably a result of fibrosis and loss of myofibrils.