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Cooperative international neuromuscular research group duchenne natural history study demonstrates insufficient diagnosis and treatment of cardiomyopathy in duchenne muscular dystrophy
Author(s) -
Spurney Christopher,
Shimizu Reiko,
Morgenroth Lauren P.,
Kolski Hanna,
GordishDressman Heather,
Clemens Paula R.
Publication year - 2014
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.24163
Subject(s) - duchenne muscular dystrophy , medicine , cardiomyopathy , natural history , ejection fraction , cardiology , muscular dystrophy , cohort , natural history study , heart failure
: 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. Muscle Nerve 50:250–256, 2014