期刊
JOURNAL OF THE AMERICAN HEART ASSOCIATION
卷 3, 期 6, 页码 -出版社
WILEY
DOI: 10.1161/JAHA.114.001471
关键词
arrhythmogenic right ventricular dysplasia/cardiomyopathy; desmosome cardiomyopathy; etiology; exercise genetics-human
资金
- Alexandre Suerman Stipend
- St. Jude Medical Inc
- Medtronic Inc
- Leyla Erkan Family Fund for ARVD Research
- Dr. Satish, Rupal, and Robin Shah ARVD Fund at Johns Hopkins
- Dr. Francis P. Chiaramonte Private Foundation
- Bogle Foundation
- Healing Hearts Foundation
- Campanella family
- Patrick J. Harrison Family
- Peter French Memorial Foundation
- Wilmerding Endowments
Background-Exercise is associated with age-related penetrance and arrhythmic risk in carriers of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C)-associated desmosomal mutations; however, its role in patients without desmosomal mutations (gene-elusive) is uncertain. This study investigates whether exercise is (1) associated with onset of gene-elusive ARVD/C and (2) has a differential impact in desmosomal and gene-elusive patients. Methods and Results-Eighty-two ARVD/C patients (39 desmosomal, all probands) were interviewed about regular physical activity from age 10. Participation in endurance athletics, duration (hours/year), and intensity (MET-Hours/year) of exercise prior to clinical presentation were compared between patients with desmosomal and gene-elusive ARVD/C. All gene-elusive patients were endurance athletes. Gene-elusive patients were more likely to be endurance athletes (P<0.001) and had done significantly more intense (MET-Hrs/year) exercise prior to presentation (P<0.001), particularly among cases presenting < age 25 (P=0.027). Family history was less prevalent among gene-elusive patients (9% versus 40% desmosomal, P<0.001), suggesting a greater environmental influence. Gene-elusive patients without family history did considerably more intense exercise than other ARVD/C patients (P=0.004). Gene-elusive patients who had done the most intense (top quartile MET-Hrs/year) exercise prior to presentation had a younger age of presentation (P=0.025), greater likelihood of meeting ARVD/C structural Task Force Criteria (100% versus 43%, P=0.02), and shorter survival free from a ventricular arrhythmia in follow-up (P=0.002). Conclusions-Gene-elusive, non-familial ARVD/C is associated with very high intensity exercise suggesting exercise has a disproportionate role in the pathogenesis of these cases. As exercise negatively modifies cardiac structure and promotes arrhythmias, exercise restriction is warranted.
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