The Effect of Adiposity on Cardiovascular Function and Myocardial Fibrosis in Patients With Duchenne Muscular Dystrophy

Background Patients with Duchenne muscular dystrophy (DMD) develop cardiomyopathy because of a dystrophin deficiency causing fibrofatty replacement of the myocardium. Corticosteroid use and mobility limitations place these patients at risk for increased adiposity. We sought to determine the associat...

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Bibliographic Details
Published in:Journal of the American Heart Association 2021-10, Vol.10 (19), p.e021037-e021037
Main Authors: Henson, Sarah E, Lang, Sean M, Khoury, Philip R, Tian, Cuixia, Rutter, Meilan M, Urbina, Elaine M, Ryan, Thomas D, Taylor, Michael D, Alsaied, Tarek
Format: Article
Language:English
Subjects:
Adiposity
Cardiomyopathies - diagnostic imaging
Cardiomyopathies - epidemiology
Cardiomyopathies - etiology
Contrast Media
Duchenne muscular dystrophy
Fibrosis
Gadolinium
Heart Diseases - diagnostic imaging
Heart Diseases - epidemiology
Heart Diseases - etiology
Humans
Magnetic Resonance Imaging, Cine
Muscular Dystrophy, Duchenne - complications
Muscular Dystrophy, Duchenne - diagnostic imaging
Muscular Dystrophy, Duchenne - pathology
Myocardium - pathology
Original Research
Retrospective Studies
Stroke Volume
ventricular dysfunction
Ventricular Function, Left
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Summary:Background Patients with Duchenne muscular dystrophy (DMD) develop cardiomyopathy because of a dystrophin deficiency causing fibrofatty replacement of the myocardium. Corticosteroid use and mobility limitations place these patients at risk for increased adiposity. We sought to determine the association of adiposity with cardiovascular dysfunction in patients with DMD. Methods and Results This was a retrospective review of patients with DMD who underwent both cardiac magnetic resonance imaging and dual-energy x-ray absorptiometry within 1 year. The cardiac magnetic resonance imaging parameters included left ventricular ejection fraction and the presence of late gadolinium enhancement (LGE positive [LGE+]). The adiposity indices, measured by dual-energy x-ray absorptiometry, included percentage of body fat, whole body fat mass indexed to height, and body mass index. A total of 324 patients were identified. Fifty-two percent had LGE+, and 36% had cardiac dysfunction (left ventricular ejection fraction
ISSN:2047-9980
2047-9980
DOI:10.1161/JAHA.121.021037