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Current Cardiac Imaging Approaches in Duchenne Muscular Dystrophy

Authors :
Sabrina Poonja
Steven C. Greenway
Alyssa Power
Jean K. Mah
Nowell M. Fine
Source :
Journal of Clinical Neuromuscular Disease. 20:85-93
Publication Year :
2018
Publisher :
Ovid Technologies (Wolters Kluwer Health), 2018.

Abstract

Duchenne muscular dystrophy (DMD) is an X-linked neuromuscular condition caused by mutations in the dystrophin gene leading to skeletal muscle weakness and dilated cardiomyopathy. The prevalence of DMD-related cardiomyopathy increases with age and is almost universal by the third decade of life. Myocardial fibrosis and progressive left ventricular dysfunction lead to the development of heart failure and premature death. With modern advances in medical and surgical management for patients with DMD increasing their life expectancy, cardiac dysfunction represents an increasing cause of morbidity and mortality in these patients. Early diagnosis of dilated cardiomyopathy before symptom development enables the initiation of potentially disease-modifying therapies, but requires regular dedicated imaging surveillance with sufficient sensitivity to detect subclinical changes in cardiac structure and function. Currently, transthoracic echocardiography (TTE) and cardiac magnetic resonance imaging (CMR) are commonly used and have complementary roles. TTE is rapid and readily available, whereas CMR is the gold standard for the quantification of ventricular structure and function and can detect the presence and extent of myocardial fibrosis, an increasingly appreciated marker for early disease. This review describes the clinical applications, advantages, and disadvantages of cardiac imaging screening and surveillance for the myocardial manifestations of DMD, with a particular focus on TTE and CMR.

Details

ISSN :
15220443
Volume :
20
Database :
OpenAIRE
Journal :
Journal of Clinical Neuromuscular Disease
Accession number :
edsair.doi.dedup.....9cc535594cce441ab2d6c622df96b64e
Full Text :
https://doi.org/10.1097/cnd.0000000000000204