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Comment & Response
September 2017

Myocardial Fibrosis in Duchenne and Becker Muscular Dystrophy

Author Affiliations
  • 1Heart and Vascular Center, Department of Medicine, Case Western Reserve University School of Medicine at MetroHealth Medical Center, Cleveland, Ohio
JAMA Cardiol. 2017;2(9):1045-1046. doi:10.1001/jamacardio.2017.1013

To the Editor In their recent article in JAMA Cardiology, Silva et al1 address an important question: does early use of angiotensin-converting enzyme inhibitor therapy in patients with Duchenne or Becker muscular dystrophy affect the progression of myocardial fibrosis (MF), as identified by cardiovascular magnetic resonance? Although this 2-year study could not focus on actual outcomes owing to both the short-term period of observation and the modest number of randomized patients, the authors confirmed that MF in patients with Duchenne muscular dystrophy provides long-term prognostic information and that left ventricular ejection fraction is often normal when MF is already evident.1,2 Thus, if the progression of MF could be slowed, long-term outcomes might be improved.