Regional expression of myocardial sheet dysfunction in dystrophin-deficient cardiomyopathy elucidated with diffusion tensor MRI and optical calcium mapping

Summary Duchene Muscular Dystrophy (DMD) is a lethal disease caused by ubiquitous lack of dystrophin, but the interaction with regional cardiac mechanical forces that may facilitate eventual expression of abnormal contractile function is unknown. Diffusion tensor MRI (DTI) was used to evaluate function in Langendorff perfused hearts in the mdx mouse model of DMD. Abnormal calcium kinetics (by optical mapping) and sheet mechanics (by DTMRI) occurred more prominently at the mid-upper ventricle, suggesting that regional mechanics influence the development of heart failure. Background