ACE inhibition to slow progression of myocardial fibrosis in muscular dystrophies

Abstract

Muscular dystrophy (MD) connotes a heterogeneous group of inherited disordersaffecting skeletal and cardiac muscle. Inseveral forms of MD, the cardiac disease may be the predominant manifestationof the underlying genetic myopathy. The cardiacinvolvement is due to progressive interstitial fibrosis and fatty replacement inboth the atria and ventricles, which may lead to cardiomyopathy, conductiondefects and tachyarrhythmias. Angiotensin-convertingenzyme inhibitors (ACE-Is) modulate the production of angiotensin II and limitthe amount of fibrosis in the myocardium, reducing mortality andhospitalization in cardiac patients. The aim of present review is to describethe antifibrotic proprieties of ACE inhibitor therapy and to summarize thecurrent body of scientific literature relating to the use of ACE-Is for theprevention and treatment of cardiomyopathy in patients with musculardystrophies.

Keywords: Angiotensin-converting enzyme inhibitor; Becker muscular dystrophy; Duchenne muscular dystrophy; Dystrophinopathic cardiomyopathy; Emery–Dreifuss muscular dystrophy; Fibrosis; Heart failure; Muscular dystrophy; Myotonic dystrophy.