Cooperative international neuromuscular research group duchenne natural history study demonstrates insufficient diagnosis and treatment of cardiomyopathy in duchenne muscular dystrophy
Abstract
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) 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. Muscle Nerve 50:250–256, 2014
Document Details
- Document Type
- Pub Defense Publication
- Publication Date
- May 14, 2014
- Source ID
- 10.1002/mus.24163
Entities
People
- Christopher F Spurney
- Hanna Kolski
- Heather Gordish‐dressman
- Lauren P. Morgenroth
- Paula R. Clemens
- Reiko Shimizu
- The Cinrg Investigators
Organizations
- Children's National Hospital
- National Center for Research Resources
- National Institute of Neurological Disorders and Stroke
- National Institute on Disability, Independent Living, and Rehabilitation Research
- United States Department of Defense
- United States Department of Veterans Affairs
- University of Alberta
- University of Pittsburgh