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Myotonic dystrophy presenting as severely dilated cardiomyopathy with out-of-hospital cardiac arrest

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A 52-year-old female was admitted after an out-ofhospital cardiac arrest due to ventricular fibrillation. Cardiological evaluation revealed non-ischaemic dilated cardiomyopathy (DCM). Cardiac MRI showed a severely dilated left ventricle with… Click to show full abstract

A 52-year-old female was admitted after an out-ofhospital cardiac arrest due to ventricular fibrillation. Cardiological evaluation revealed non-ischaemic dilated cardiomyopathy (DCM). Cardiac MRI showed a severely dilated left ventricle with an ejection fraction of 17% (Fig. 1a). Normal coronary arteries were seen on the coronary angiogram. Her medical history revealed cataracts at the age of 48 years and diminished strength in her hands. Two of her sisters had been diagnosed with myotonic dystrophy (MD). Their children had a more severe phenotype including clubfeet and developmental delay (Fig. 1b). Analysis

Keywords: dilated cardiomyopathy; cardiac arrest; myotonic dystrophy; severely dilated

Journal Title: Netherlands Heart Journal
Year Published: 2018

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