Serial two-dimensional echocardiography in duchenne muscular dystrophy

Patients with Duchenne muscular dystrophy (DMD) are known to have progressive epicardial fibrosis of the free wall of the left ventricle, but standard noninvasive M-mode echocardiographic tests of left ventricular function are relatively insensitive detectors of this cardiomyopathy. We therefore used two-dimensional echocardiography to record three short axis levels of the left ventricle in 13 Duchenne patients. Serial studies were separated by 2 years for most patients. The two-dimensional echocardiographic technique allows qualitative evaluation of segmental contraction of the left ventricle. Four general principles were found during this study: (1) Obvious contraction abnormalities of the left ventricle were present in most patients with DMD. (2) In most patients, the contraction deficit was first noted in the left ventricular posterior free wall behind the mitral valve. (3) Once a contraction deficit was observed, the area of abnormal contraction progressed inferiorly to include additional areas of the left ventricular posterior free wall. (4) Standard M-mode left ventricular function techniques were unreliable for detecting individuals with segmental contraction abnormalities.