Integrated Backscatter in Becker Muscular Dystrophy Patients With Functionally Normal Heart: Myocardial Ultrasound Tissue Characterization Study

Cardiac involvement (leading to cardiomyopathy and heart failure) is frequent, age-dependent, and unpredictable (1). Since there is no direct relationship between severity of skeletal and cardiac involvement, cardiomyopathy frequently develops in patients with normal skeletal muscle function (2). In BMD patients, differences in the amount of myocardial dystrophin and in the site of the deletion, as well as other still-unidentified factors, are probably the molecular basis of the cardiac involvement that is common in BMD patients, with ECG changes in about 70% of cases (2) and reduced systolic function in up to 60% (1). Since the muscle involvement is often mild and heart disease is a frequent cause of death in BMD (in up to 50% of patients) (5), cardiac transplantation has been successfully performed in these patients.