Abstract
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.
Highlights
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
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)
Patients with MF and preserved LVEF that were randomized for treating with Angiotensin-converting enzyme inhibitors (ACEI) had lower evolution of MF than those who were randomized to untreated group (3.1 ± 7.4% vs. 10.0 ± 6.2%, respectively, p = 0.001)
Summary
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
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