Voluntary Muscle Involvement in Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy is generally considered to be a primary disease of cardiac muscle, although several clinical observations suggest that the pathologic process might be more diffuse. To further examine this possibility, electromyography and voluntary muscle biopsies were done on 11 patients with hypertrophic cardiomyopathy. In 10 of 10 patients electromyography showed reductions in mean potential amplitude and duration, with an increased incidence of short-duration polyphasic deflections (findings traditionally accepted as indicative of a myopathic process). Light and electron microscopic studies of the biopsy material showed abnormalities in eight of 11 patients: four had central core or target fibers, or both, and two of these, plus four others, had subsarcolemmal mitochondrial proliferation with or without abnormal ultrastructure. These findings indicate that hypertrophic cardiomyopathy is only one aspect of a larger disease spectrum, with abnormalities in both voluntary and cardiac muscle.