Key result
Histological examination of hearts from patients with chronic myopathy revealed fibre hypertrophy, interstitial fibrosis, vacuolar degeneration, and focal groups of aberrantly orientated myofibres.
Case Report (n=3)
Identifies specific histological changes, including aberrantly orientated myofibres, in the hearts of patients with chronic myopathies.
May indicate cardiac involvement in chronic myopathy; case report leaves open prognostic value and screening implications.
Histological changes in the hearts of two patients with dystrophia myotonica and one patient with a familial scapulo-peroneal myopathy are described. The myocardium showed fibre hypertrophy, interstitial fibrosis, vacuolar degeneration of myocardial cells and focal groups of aberrantly orientated myofibres. The AV node in the case of scapulo-peroneal myopathy showed vacuolar fibre degeneration and fatty infiltration. The aberrantly orientated myofibres have not previously been described in this context, and their relationship to similar aberrant fibres in hypertrophic obstructive cardio-myopathy and the electrophysiological and clinical manifestations of cardiac involvement in myotonic dystrophy are discussed.
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Andrea Franks (1978) conducted a case report in Dystrophia myotonica and familial scapulo-peroneal myopathy (n=3). Chronic myopathy was evaluated on Histological changes in the heart. Histological examination of hearts from patients with chronic myopathy revealed fibre hypertrophy, interstitial fibrosis, vacuolar degeneration, and focal groups of aberrantly orientated myofibres.
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