Key result
Orthotopic cardiac transplantation in a 17-year-old boy with Becker's muscular dystrophy and intractable heart failure resulted in return to work and previous exercise tolerance at 2 years.
Why the study?
Can orthotopic cardiac transplantation be an acceptable treatment for patients with muscular dystrophy and life-threatening cardiomyopathy?
Case Report (n=1)
Can orthotopic cardiac transplantation be an acceptable treatment for patients with muscular dystrophy and life-threatening cardiomyopathy?
Heart transplantation can be an acceptable treatment for patients with muscular dystrophy and life-threatening cardiomyopathy refractory to medical management, provided they have preserved ambulation and favorable life expectancy.
May support cardiac transplantation in Becker muscular dystrophy cardiomyopathy; leaves open long-term outcomes and selection criteria.
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.
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Donofrio et al. (1989) conducted a case report in Muscular dystrophy and cardiomyopathy (n=1). Orthotopic cardiac transplantation was evaluated on Clinical status and survival. Orthotopic cardiac transplantation in a 17-year-old boy with Becker's muscular dystrophy and intractable heart failure resulted in return to work and previous exercise tolerance at 2 years.
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