Abstract #0756

Left Ventricular Distribution of LGE and Intra-myocardial Fat in Boys with Duchenne Muscular Dystrophy and Healthy Controls at 3T

Nyasha G Maforo^1,2, Ashley Prosper¹, Pierangelo Renella^1,3, Nancy Halnon⁴, Holden H Wu^1,2,5, and Daniel B Ennis^6,7,8

¹Radiological Sciences, University of California, Los Angeles, Los Angeles, CA, United States, ²Physics and Biology in Medicine, University of California, Los Angeles, Los Angeles, CA, United States, ³Medicine (Pediatric Cardiology), Children's Hospital of Orange County, Orange, CA, United States, ⁴Pediatrics (Cardiology), University of California, Los Angeles, Los Angeles, CA, United States, ⁵Bioengineering, University of California, Los Angeles, Los Angeles, CA, United States, ⁶Radiology, Stanford University, Stanford, CA, United States, ⁷Cardiovascular Institute, Stanford University, Stanford, CA, United States, ⁸Maternal and Child Health Research Institute, Stanford University, Stanford, CA, United States

Duchenne muscular dystrophy (DMD) – a fatal X-linked genetic disorder – is characterized by progressive muscle weakness, pediatric onset cardiomyopathy, and ultimately heart failure. LGE imaging is the gold standard for detecting myocardial replacement fibrosis, but not fatty infiltration. We used chemical-shift based water-fat separation MRI techniques to investigate myocardial fibro-fatty infiltration and identify the onset of microstructural remodeling in boys with DMD. This study aimed to: 1) report and compare the left-ventricular (LV) intra-myocardial fat content in boys with DMD and healthy controls; and 2) determine whether fatty infiltration precedes the appearance of LGE in boys with DMD.

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