Article
Emery-Dreifuss muscular dystrophy Type 1 is associated with a high risk of malignant ventricular arrhythmias and end-stage heart failure.
European heart journal - 21 Dec 2023
Cannie Douglas E, Syrris Petros, Protonotarios Alexandros, Bakalakos Athanasios, Pruny Jean-François, Ditaranto Raffaello, Martinez-Veira Cristina, Larrañaga-Moreira Jose M, Medo Kristen, Bermúdez-Jiménez Francisco José, Ben Yaou Rabah, Leturcq France, Mezcua Ainhoa Robles, Marini-Bettolo Chiara, Cabrera Eva, Reuter Chloe, Limeres Freire Javier, Rodríguez-Palomares José F, Mestroni Luisa, Taylor Matthew R G, Parikh Victoria N, Ashley Euan A, Barriales-Villa Roberto, Jiménez-Jáimez Juan, Garcia-Pavia Pablo, Charron Philippe, Biagini Elena, García Pinilla José M, Bourke John, Savvatis Konstantinos, Wahbi Karim, Elliott Perry M
Abstract excerpt
BACKGROUND AND AIMS: Emery-Dreifuss muscular dystrophy (EDMD) is caused by variants in EMD (EDMD1) and LMNA (EDMD2). Cardiac conduction defects and atrial arrhythmia are common to both, but LMNA variants also cause end-stage heart failure (ESHF) and malignant ventricular arrhythmia (MVA). This study aimed to better characterize the cardiac complications of EMD variants. METHODS: Consecutively referred EMD...
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