Emery-Dreifuss muscular dystrophy Type 1 is associated with a high risk of malignant ventricular arrhythmias and end-stage heart failure.
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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
Аннотация
METHODS: Consecutively referred EMD variant-carriers were retrospectively recruited from 12 international cardiomyopathy units. MVA and ESHF incidences in male and female variant-carriers were determined. Male EMD variant-carriers with a cardiac phenotype at baseline (EMDCARDIAC) were compared with consecutively recruited male LMNA variant-carriers with a cardiac phenotype at baseline (LMNACARDIAC).
RESULTS: Longitudinal follow-up data were available for 38 male and 21 female EMD variant-carriers [mean (SD) ages 33.4 (13.3) and 43.3 (16.8) years, respectively]. Nine (23.7%) males developed MVA and five (13.2%) developed ESHF during a median (inter-quartile range) follow-up of 65.0 (24.3-109.5) months. No female EMD variant-carrier had MVA or ESHF, but nine (42.8%) developed a cardiac phenotype at a median (inter-quartile range) age of 58.6 (53.2-60.4) years. Incidence rates for MVA were similar for EMDCARDIAC and LMNACARDIAC (4.8 and 6.6 per 100 person-years, respectively; log-rank P = .49). Incidence rates for ESHF were 2.4 and 5.9 per 100 person-years for EMDCARDIAC and LMNACARDIAC, respectively (log-rank P = .09).
CONCLUSIONS: Male EMD variant-carriers have a risk of progressive heart failure and ventricular arrhythmias similar to that of male LMNA variant-carriers. Early implantable cardioverter defibrillator implantation and heart failure drug therapy should be considered in male EMD variant-carriers with cardiac disease.