Progression of myocardial fibrosis by magnetic resonance imaging in patients with Duchenne and Becker muscular dystrophy and preserved left ventricular ejection fraction - a randomized clinical trial for treatment with ACE inhibitors

Background Duchenne (DMD) and Becker (BMD) muscular dystrophies ( MD) are inherited X-linked diseases characterized by absence or decrease of dystrophin, a sarcolemal protein that is essential for maintenance of the muscular membrane integrity during muscular contraction. Cardiac involvement is as high, it can be clinically silent, but is often complicated by severe heart failure and high mortality. Angiotensin-converting enzyme inhibitors (ACEI) is recommended for patients with left ventricular dysfunction. We previously described that CMR can identify myocardial fibrosis (MF) even in the early stages of cardiomyopathy in MD before overt LV dysfunction (J Am Coll Cardiol 2007;49:1874-9). The impact of treatment with ACE inhibitors in the progression of fibrosis in patients with MD and preserved LV function is still unknown.