Cardiac involvement in Duchenne and Becker muscular dystrophy

Core Tip

Core tip: Duchenne and Becker muscular dystrophy are the commonest X-linked muscular diseases. Death is usually due to cardiac disease including ventricular dysfunction, heart block or malignant arrhythmias. Female carriers may also present cardiac involvement. Overt heart failure may be delayed or absent. Electrocardiography findings include conduction defects, arrhythmias and myocardial necrosis. Echocardiography assesses a marked variability of left ventricular dysfunction. Epicardial fibrosis in both patients and carriers has been documented by Cardiovascular Magnetic Resonance (CMR), even if overt muscular disease is absent. A combined approach using clinical and CMR assessment may motivate early cardioprotective treatment and delay serious cardiac complications.