A patient with Emery-Dreifuss muscular dystrophy was followed up from 9 to 26 years of age. Serial electrocardiographic studies showed changes ranging from early cardiac conduction abnormality as first degree atrioventricular block to sick sinus syndrome. In addition the development of cardiomyopathy was documented by electrocardiographic, echocardiographic and radionuclide studies of the heart.
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