Cardiac involvement in progressive muscular dystrophy

Abstract

1. 1. Forty-four patients with muscular dystrophy have been described. In general, no relationship between the duration and severity of the skeletal muscular lesions and those of the heart was noted. 2. 2. The necropsy data from one of the six patients on whom histological data were available have been described in detail. The similarity of cardiac and skeletal muscle lesions was noteworthy in all our cases. 3. 3. Variations in the electrocardiogram suggestive of abnormality were frequent. Tachycardia, abnormalities of the P waves, and tall R waves in V 1 and V 2 occurred frequently. Although no specific pattern was indicative of dystrophic involvement of the heart, patients with progressive muscular dystrophy on whom unusual electrocardiograms have been obtained should be suspected of having dystrophic heart disease. 4. 4. In patients on whom the diagnosis of progressive muscular dystrophy (pseudohypertrophic or otherwise) has been made, the etiological diagnosis of “possible dystrophic heart disease” must always be considered. By “dystrophic heart disease” it is inferred that the heart has been pathologically involved by a histological process pathognomonic of the skeletal muscular disease.