Journal article

Cooperative international neuromuscular research group duchenne natural history study demonstrates insufficient diagnosis and treatment of cardiomyopathy in duchenne muscular dystrophy

C Spurney, R Shimizu, LP Morgenroth, H Kolski, H Gordish-Dressman, PR Clemens, M Cregan, E Goude, M Glick, L Johnson, J Han, N Joyce, D Kilmer, A Nicorici, C Chidambaranathan, S Kumar, L Eliasoph, E Hosaki, A Gonzales, V Harris Show all

Muscle and Nerve | Published : 2014

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) <28% or ejection fraction (EF) 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 underutilize..

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University of Melbourne Researchers