Journal
MUSCLE & NERVE
Volume 50, Issue 2, Pages 250-256Publisher
WILEY
DOI: 10.1002/mus.24163
Keywords
cardiomyopathy; Duchenne muscular dystrophy; echocardiogram; glucocorticoid; natural history
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Funding
- U.S. Department of Education/NIDRR [H133B031118, H133B090001]
- NIH/NINDS Paul D. Wellstone Muscular Dystrophy Research Centers [U54HD053177]
- U.S. Department of Defense [W81XWH-09-1-0592]
- NIH/NCRR [UL1RR031988]
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Introduction: Cardiomyopathy is a common cause of morbidity and death in patients with Duchenne muscular dystrophy (DMD). Methods: This investigation was a cross-sectional cross-sectional analysis of clinical data from the multi-institutional Cooperative International Neuromuscular Research Group (CINRG) DMD Natural History Study of 340 DMD patients aged 2-28 years. Cardiomyopathy was defined as shortening fraction (SF) <28% or ejection fraction (EF) <55%. Results: Two hundred thirty-one participants reported a prior clinical echocardiogram study, and 174 had data for SF or EF. The prevalence of cardiomyopathy was 27% (47 of 174), and it was associated significantly with age and clinical stage. The association of cardiomyopathy with age and clinical stage was not changed by glucocorticoid use as a covariate (P>0.68). In patients with cardiomyopathy, 57% (27 of 47) reported not taking any cardiac medications. Cardiac medications were used in 12% (15 of 127) of patients without cardiomyopathy. Conclusions: We found that echocardiograms were underutilized, and cardiomyopathy was undertreated in this DMD natural history cohort.
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