Cardiac Arrhythmias in Muscular Dystrophies Associated with Emerinopathy and Laminopathy: A Cohort Study.

Autor: Marchel M; 1st Department of Cardiology, Medical University of Warsaw, 02-097 Warsaw, Poland., Madej-Pilarczyk A; Department of Medical Genetics, The Children's Memorial Health Institute, 04-730 Warsaw, Poland., Tymińska A; 1st Department of Cardiology, Medical University of Warsaw, 02-097 Warsaw, Poland., Steckiewicz R; 1st Department of Cardiology, Medical University of Warsaw, 02-097 Warsaw, Poland., Ostrowska E; 1st Department of Cardiology, Medical University of Warsaw, 02-097 Warsaw, Poland., Wysińska J; 1st Department of Cardiology, Medical University of Warsaw, 02-097 Warsaw, Poland., Russo V; Department of Translational Medical Sciences, University of Campania 'Luigi Vanvitelli'-Monaldi Hospital, 80131 Naples, Italy., Grabowski M; 1st Department of Cardiology, Medical University of Warsaw, 02-097 Warsaw, Poland., Opolski G; 1st Department of Cardiology, Medical University of Warsaw, 02-097 Warsaw, Poland.
Jazyk: angličtina
Zdroj: Journal of clinical medicine [J Clin Med] 2021 Feb 12; Vol. 10 (4). Date of Electronic Publication: 2021 Feb 12.
DOI: 10.3390/jcm10040732
Abstrakt: Introduction: Cardiac involvement in patients with muscular dystrophy associated with Lamin A/C mutations ( LMNA ) is characterized by atrioventricular conduction abnormalities and life-threatening cardiac arrhythmias. Little is known about cardiac involvement in patients with emerin mutation ( EMD ). The aim of our study was to describe and compare the prevalence and time distribution of cardiac arrhythmias at extended follow-up.
Patients and Methods: 45 consecutive patients affected by muscular dystrophy associated to laminopathy or emerinopathy were examined. All patients underwent clinical evaluation, 12-lead surface electrocardiogram (ECG), 24 h electrocardiographic monitoring, and cardiac implanted device interrogation.
Results: At the end of 11 (5.0-16.6) years of follow-up, 89% of the patients showed cardiac arrhythmias. The most prevalent was atrial standstill (AS) (31%), followed by atrial fibrillation/flutter (AF/Afl) (29%) and ventricular tachycardia (22%). EMD patients presented more frequently AF/AFl compared to LMNA (50% vs. 20%, p = 0.06 ). Half of the EMD patients presented with AS, whilst there was no occurrence of such in the LMNA ( p = 0.001). Ventricular arrhythmias were found in 60% of patients with laminopathy compared to 3% in patients with emerinopathy ( p < 0.001). The age of AVB occurrence was higher in the LMNA group (32.8 +/- 10.6 vs. 25.1 +/- 9.1, p = 0.03).
Conclusions: Atrial arrhythmias are common findings in patients with muscular dystrophy associated with EMD / LMNA mutations; however, they occurred earlier in EMD patients. Ventricular arrhythmias were very common (60%) in LMNA and occurred definitely earlier compared to the EMD group.
Databáze: MEDLINE
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