Cardiac transplantation in a patient with muscular dystrophy and cardiomyopathy
P. D. Donofrio, V. R. Challa, B. T. Hackshaw, S. A. Mills and A. R. Cordell
Department of Neurology, Wake Forest University, Bowman Gray School of Medicine, Winston-Salem, NC 27103.
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.
