Abstract 9370: Myocardial Tissue Characterization of Pediatric Patients With Duchenne Muscular Dystrophy

• Published in: Circulation (New York, N.Y.) N.Y.), 2021-11, Vol.144 (Suppl_1), p.A9370-A9370
• Main Authors: Sunthankar, Sudeep, George-Durrett, Kristen, Crum, Kim, Markham, Larry W, Soslow, Jonathan H
• Format: Article
• Language: English
• ISSN: 0009-7322; 1524-4539
• DOI: 10.1161/circ.144.suppl_1.9370

IntroductionCardiomyopathy (CM) is the leading cause of death in Duchenne muscular dystrophy (DMD). Cardiac magnetic resonance (CMR) parametric mapping sequences, Native T1, T2, and extracellular volume (ECV), can offer insights into disease pathophysiology. The objective of this abstract was to leverage parametric mapping sequences to define the tissue characteristics of DMD CM progression. We hypothesized DMD subjects could be categorized based on fibrosis, myocardial edema, and fat infiltration. MethodsDMD patients (n=52; median 12.1 years) underwent CMR including T1, T2, and ECV at the base, mid, and apex. Categories were defined as normal, early fibrosis (normal ECV, high T1, normal T2), fibrosis (high ECV, normal or high T1, normal T2), edema (normal or high ECV, normal or high T1, high T2), fat (normal ECV, low T1, high T2) or fibrofatty (high ECV, normal or low T1, high T2). Statistical analysis included Chi-square, Wilcoxon rank sum, Wilcoxon signed rank, and Spearman’s rho. ResultsMedian left ventricular ejection fraction (LVEF) was 59% and 15 (29%) had LVEF < 55%; 18 subjects (35%) had normal LVEF and no late gadolinium enhancement. These subjects were younger (10.2 years [8.4,11.6] vs 14.7 [11.5,17.8], p