1. Evaluation of the cardiomyopathy in becker muscular dystrophy
- Author
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Francesco Mario Limongelli, Luciano Fattore, L. Passamano, Maria Luisa De Rimini, Maria Esposito, Giugliano Ma, Pasquale Raia, Comi Li, Francesco De Luca, Vito R. Petretta, Luisa Politano, Brunella Restucci, Giovanni Nigro, Vincenzo Nigro, Luigi Comi, Konstantine Tebloev, G., Nigro, L. I., Comi, L., Politano, F. M., Limongelli, V., Nigro, M. L., Derimini, M. A., M., V. R., Petretta, L., Passamano, Restucci, Brunella, L., Fattore, K., Tebloev, L., Comi, F., Deluca, P., Raia, M. G., Esposito, Nigro, G, Comi, Li, Politano, Luisa, Limongelli, Fm, Nigro, Vincenzo, DE RIMINI, Ml, Giugliano, Ma, Petretta, Vr, Passamano, L, and Restucci, B.
- Subjects
Adult ,Aging ,medicine.medical_specialty ,Adolescent ,Heart disease ,Physiology ,Cardiomyopathy ,Muscular Dystrophies ,Electrocardiography ,Cellular and Molecular Neuroscience ,Physiology (medical) ,Internal medicine ,medicine ,Humans ,In patient ,Muscular dystrophy ,Skeletal muscle damage ,Ultrasonography ,Tomography, Emission-Computed, Single-Photon ,biology ,business.industry ,Incidence ,Heart ,Dilated cardiomyopathy ,Middle Aged ,medicine.disease ,Surgery ,Italy ,Cardiology ,biology.protein ,Neurology (clinical) ,Cardiomyopathies ,business ,Complication ,Dystrophin - Abstract
To evaluate the features and the course of cardiomyopathy in Becker muscular dystrophy, 68 patients-identified by clinical assessment and by reduced dystrophin labeling and/or DNA analysis-were followed in the years 1976-1993, for periods ranging from 3 to 18 years (mean 8). Patients periodically underwent clinical, electrocardiographic, echocardiographic, nuclear, and radiological assessments. Preclinical cardiac involvement was found in 67.4% of patients under 16 years of age, decreasing to 30% in patients older than 40. Clinically evident cardiomyopathy was found in 15% of patients under 16 years of age, increasing to 73% in patients older than 40. A real, dilated cardiomyopathy is the most frequent type of myocardial involvement after the age of 20. Results show that the severity of cardiac involvement can be unrelated to the severity of skeletal muscle damage and confirm that cardiac dysfunction is a primary feature of Becker muscular dystrophy. (C) 1995 John Wiley & Sons, Inc.
- Published
- 1995