Regional expression of myocardial sheet dysfunction in dystrophin-deficient cardiomyopathy elucidated with diffusion tensor MRI and optical calcium mapping
نویسندگان
چکیده
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.
منابع مشابه
Focal but reversible diastolic sheet dysfunction reflects regional calcium mishandling in dystrophic mdx mouse hearts.
Cardiac dysfunction is a primary cause of patient mortality in Duchenne muscular dystrophy, potentially related to elevated cytosolic calcium. However, the regional versus global functional consequences of cellular calcium mishandling have not been defined in the whole heart. Here we sought for the first time to elucidate potential regional dependencies between calcium mishandling and myocardia...
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