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-  2018 

Original research article: Association and diagnostic utility of diastolic dysfunction and myocardial fibrosis in patients with Fabry disease

DOI: 10.1136/openhrt-2018-000803

Keywords: echocardiography, Fabry, MRI

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Abstract:

Current guidelines highlight important therapy implications of cardiac fibrosis in patients with Fabry disease (FD). However, association between morphological and functional impairments with cardiac fibrosis in hereditary cardiomyopathies remains elusive. We investigated the association between echocardiography-determined cardiac dysfunction and cardiac MRI (cMRI)-detected myocardial fibrosis (late gadolinium enhancement, LE) in patients with FD with preserved left ventricular ejection fraction (≥50%)

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