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SYMPOSIUM – LAMINOPATHIES. Emery-Dreifuss muscular dystrophy – a cardiologist’s perspective

 Michał Marchel, Roman Steckiewicz, Grzegorz Opolski

Affiliation and address for correspondence
Aktualn Neurol 2006, 6 (1), p. 39-43
Abstract

Emery-Dreifuss muscular dystrophy (EDMD) is characterized by various degrees of cardiac involvement. It concerns clinically asymptomatic patients, patients with arrhythmia, and patients with heart failure due to systolic dysfunction. Cardiomyopathy due to EDMD may lead to sudden cardiac death. The variations in the clinical picture of cardiomyopathy in the two forms of EDMD with regard to the type of inheritance is still unclear. The following paper discusses the most recent papers concerning cardiac complications in EDMD and possibilities of their prevention and therapy. Various cardiologic complications in context of different laminopathies have been presented. It lays particular emphasis on the comprehensiveness of treatment and the interdisciplinary nature of the issue. 

Keywords
Emery-Dreifuss muscular dystrophy, cardiomyopathy, arrhythmias, heart failure, laminopathies

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