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Abstract #3803

Early Signs of Cardiomyopathy in Delta-Sarcoglycan Null Mice

Anne-Cecile Huby1, Sumeda Abeykoon2, Scott Dunn2, Janaka Wansapura2

1The Heart Institute, Cincinnati Children's Hospital, Cincinnati, OH, United States; 2Imaging Research Center, Cincinnati Children's Hospital, Cincinnati, OH, United States

Delta-sarcoglycan null mice (DSG) develop cardiac and skeletal muscle histopathological alterations similar to those in humans with limb girdle muscular dystrophy. In this study we performed cardiac MRI on 12 week old mice compared to wild type (WT). There was no difference in the ejection fraction between DSG and WT. However the circumferential strain of DSG mice was significantly less than that of the WT. There was an increase in the gene expression of collagen markers. No delayed enhancement was observed but ex vivo T1 and T2 maps showed decreased relaxation times that correlated with mild diffused fibrosis in histopathology.