Cardiopathy in Muscular Dystrophy

SUMMARY In previous communications 12 , 13 certain electrocardiographic changes, described under myopathic pattern, were reported in a series of patients suffering from various types of muscular dystrophy . The present report is a further elaboration of the cardiac status in these patients. One hundred and sixty cases of various forms of myopathy were studied. Findings suggestive of cardiopathy were limited only to pseudohypertrophic muscular dystrophy (sex linked Duchenne type myopathy). Except for the coincidental finding of congenital or rheumatic heart disease in four cases, only two showed clinically recognizable cardiopathy in the form of congestive cardiac failure . This was confirmed in one of the cases with histopathologic evidence on necropsy. Another three cases had cardiac enlargement on radiologic studies . Seventy-five per cent of the cases of pseudohypertrophic muscular dystrophy showed “myopathic pattern” 13 in the electrocardiogram. Right heart catheterization done in nine cases confirmed the congenital cardiac lesion in one and showed incipient right heart failure in another. Findings were in the upper limits of normal in two more cases. This was shown when pressure was applied in the right hypochondrium . However, resting studies showed normal findings in all these cases. Various lacunae in our knowledge regarding the limitation of “myopathic pattern” to pseudohypertrophic muscular dystrophy group, the genesis of electrocardiographic changes and the relative rarity of congestive cardiac failure are emphasized.