source: Journal of Cardiovascular Magnetic Resonance
Jonathan H Soslow, Stephen M Damon, Kimberly Crum, David Parra, Andrew E Arai, Bruce M Damon, Larry W Markham
In the current era, cardiovascular disease is the leading cause of death in patients with Duchenne muscular dystrophy (DMD). Pathological studies demonstrate myocardial fibrosis that begins in the subepicardium of the left ventricular free wall. Late gadolinium enhancement (LGE) images demonstrate a similar pattern of extracellular matrix (ECM) expansion. However, based on a baseline pro-inflammatory state and reports of possible myocarditis in DMD subjects, it is unclear whether this LGE represents edema or fibrosis. Our objective was to use extracellular volume (ECV) and T2 mapping to better characterize LGE in boys with DMD.
21 DMD subjects and 11 healthy male controls were prospectively enrolled. Subjects underwent cardiac MRI including standard functional analysis and LGE as well as T1 and T2 mapping in the short axis at the level of the papillary muscles. Pre and post-contrast T1 maps and a hematocrit were used to calculate ECV maps. Manual contours were traced on ECV and T2 maps to determine global and segmental values. Focal regions of interest (ROI) in areas of significant LGE were traced on ECV maps. Values were compared between DMD and controls using a Wilcoxon rank-sum test.
National Heart, Lung, and Blood Institute, National Institutes of Health, USA; Vanderbilt University Medical Center, USA