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Exercise and arrhythmic risk in TMEM43 p.S358L arrhythmogenic right ventricular cardiomyopathy.
- Source :
-
Heart rhythm [Heart Rhythm] 2020 Jul; Vol. 17 (7), pp. 1159-1166. Date of Electronic Publication: 2020 Feb 29. - Publication Year :
- 2020
-
Abstract
- Background: High-level exercise has been associated with a malignant phenotype in desmosomal and genotype-negative forms of arrhythmogenic right ventricular cardiomyopathy (ARVC). This is the first study to examine this issue with ARVC secondary to the TMEM43 p.S358L mutation.<br />Objective: The purpose of this study was to evaluate the impact of exercise on arrhythmic risk and cardiac death in TMEM43 p.S358L ARVC.<br />Methods: Individuals with the TMEM43 p.S358L mutation enrolled in a prospective registry who had received a primary prevention implantable cardioverter-defibrillator (ICD) were invited to complete the modified Paffenbarger Physical Activity Questionnaire to assess their physical activity in the year before their ICD implantation. Time-to-event analyses using unadjusted and adjusted Cox proportional hazards models evaluated associations between physical activity and first appropriate ICD discharge secondary to malignant ventricular arrhythmia or cardiac death.<br />Results: In 80 subjects with the TMEM43 p.S358L mutation, exercise ≥9.0 metabolic equivalent of task (MET)-hours/day (high level) in the year before ICD implantation was associated with an adjusted 9.1-fold increased hazard of first appropriate ICD discharge (there were no deaths) relative to physical activity <9.0 MET-hours/day (moderate level) (95% confidence interval [CI] 3.3-24.6 MET-hours/day; P < .001). The median age from birth to first appropriate ICD discharge was 58.5 years (95% CI 56.5-60.5 years) vs 35.8 years (95% CI 28.2-43.4 years) (P < .001) in subjects in moderate- and high-level exercise groups, respectively.<br />Conclusion: Exercise ≥9.0 MET-hours/day is associated with an increased risk of malignant ventricular arrhythmias in the TMEM43 p.S358L subtype of ARVC. Extrapolating these data, we suggest molecular testing be offered in early childhood to inform exercise choices reflective of the genotype.<br /> (Copyright © 2020 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)
- Subjects :
- Adult
Arrhythmogenic Right Ventricular Dysplasia genetics
Arrhythmogenic Right Ventricular Dysplasia physiopathology
DNA Mutational Analysis
Female
Humans
Male
Membrane Proteins metabolism
Phenotype
Prospective Studies
Risk Factors
Arrhythmogenic Right Ventricular Dysplasia prevention & control
DNA genetics
Exercise physiology
Membrane Proteins genetics
Mutation
Primary Prevention methods
Subjects
Details
- Language :
- English
- ISSN :
- 1556-3871
- Volume :
- 17
- Issue :
- 7
- Database :
- MEDLINE
- Journal :
- Heart rhythm
- Publication Type :
- Academic Journal
- Accession number :
- 32120009
- Full Text :
- https://doi.org/10.1016/j.hrthm.2020.02.028