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In a patient with complete atrioventricular block and restrictive cardiomyopathy, the cardiomyopathy was found to be part of a generalized disorder affecting both cardiac and skeletal muscle.
Case Report (n=1)
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Restrictive cardiomyopathy presenting with complete AV block can be a manifestation of a more generalized myopathy affecting both cardiac and skeletal muscle.
Two-dimensional echocardiography, cardiac catheterization, electrophysiologic studies, and biopsies of cardiac and skeletal muscle were performed in a patient with complete atrioventricular block as the initial manifestation of restrictive cardiomyopathy. The patient also showed distal myopathy with rimmed vacuoles. Weakness of the distal leg muscles occurred several years after the initial cardiac manifestations and the patient developed congestive heart failure 1 year before the second admission. Endomyocardial biopsy showed patchy myocardial fibrosis without endomyocardial fibrosis or any other specific findings of myocardial disease. Histochemical examination of skeletal muscle showed the typical features of distal myopathy with rimmed vacuoles. In some patients with atrioventricular block as the initial manifestation of restrictive cardiomyopathy, the cardiomyopathy may be just 1 manifestation of a more generalized disorder affecting both cardiac and skeletal muscle.
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Ishiwata et al. (1993) conducted a case report in Restrictive cardiomyopathy with complete atrioventricular block and distal myopathy with rimmed vacuoles (n=1). In a patient with complete atrioventricular block and restrictive cardiomyopathy, the cardiomyopathy was found to be part of a generalized disorder affecting both cardiac and skeletal muscle.
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