Cardiac pathology in chronic myopathy, with particular reference to dystrophia myotonica

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Abstract
Histological changes in the hearts of 2 patients with dystrophia myotonica and 1 patient with a familial scapulo-peroneal myopathy are described. The myocardium showed fiber hypertrophy, interstitial fibrosis, vacuolar degeneration of myocardial cells and focal groups of aberrantly orientated myofibers. The AV [atrioventricular] node in the case of scapulo-peroneal myopathy showed vacuolar fiber degeneration and fatty infiltration. The aberrantly orientated myofibers were not previously described in this context, and their relationship to similar aberrant fibers in hypertrophic obstructive cardio-myopathy and the electrophysiological and clinical manifestations of cardiac involvement in myotonic dystrophy are discussed.

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