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The heart in becker muscular dystrophy, facioscapulohumeral dystrophy, and bethlem myopathy
Author(s) -
de Visser Marianne,
de Voogt Willem G.,
la Rivière Guusje V.
Publication year - 1992
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.880150510
Subject(s) - facioscapulohumeral muscular dystrophy , muscular dystrophy , medicine , myopathy , dystrophy , cardiomyopathy , cardiology , heart failure , pathology
We report a study, assessing involvement of the heart in 33 familial cases of Becker muscular dystrophy (BMD), 31 familiar cases of facioscapulohumeral (FSH) dystrophy, and 27 familial cases of Bethlem myopathy. In the patients with BMD, correlations of myocardial involvement with age and extent of musculoskeletal involvement were made. We performed physical examination, chest X‐ray, electrocardiographic (EKG), and echocardiographic extients with FSH dystrophy. Thirteen patients with BMD (45%) showed EKG changes similar to those found in Duchenne muscular dystrophy. Only 1 of the 13 individuals with cardiac invovement was wheelchairbound. We found no evidence of cardiac changes in the patients with FSH dystrophy. In Bethlem myopathy, only 1 patient had a form of hypertrophic cardiomyopathy (asymmetrical septal hypertrophy).