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Peter D. Donofrio, MD; Venkata R. Challa, MD; Barry T. Hackshaw, MD; Stephen A. Mills, MD; A. Robert Cordell, MD

Arch Neurol. 1989;46(6):705-707.

Abstract
• A 17-year-old boy with muscular dystrophy developed a cardiomyopathy. His brother died of a cardiomyopathy, and muscle enzyme levels were elevated in asymptomatic family members. Examination revealed cardiomegaly, hepatomegaly, proximal muscle atrophy and weakness, and calf hypertrophy. Skeletal muscle and endomyocardial biopsy specimens were consistent with Becker's muscular dystrophy. Because of intractable heart failure, orthotopic cardiac transplantation was performed. Two years after transplantation, the patient has returned to work and regained previous exercise tolerance. Heart transplantation can be an acceptable treatment of patients who have muscular dystrophy, with preserved ambulation and favorable life expectancy, and also life-threatening cardiomyopathy refractory to medical management.

Author Affiliations
From the Departments of Neurology (Dr Donofrio), Pathology (Dr Challa), Cardiology (Dr Hackshaw), and Surgery (Drs Mills and Cordell), Wake Forest University, The Bowman Gray School of Medicine, Winston-Salem, NC.

Footnotes
Accepted for publication Sept 15, 1988.

Presented in part at the 39th Annual Meeting of the American Academy of Neurology, New York, April 8, 1987.

Reprint requests to Department of Neurology, The Bowman Gray School of Medicine, 300 S Hawthorne Rd, Winston-Salem, NC 27103 (Dr Donofrio).

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