Exercise Increases Age-Related Penetrance and Arrhythmic Risk in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Associated Desmosomal Mutation Carriers
By C. James, A. Bhonsale, C. Tichnell, B. Murray, S. Russell, H. Tandri, R. Tedford, D. Judge, H. Calkins. Journal of the American College of Cardiology, 2013
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Objectives To determine how exercise influences penetrance of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) among patients with desmosomal mutations. Background While animal models and anecdotal evidence suggest exercise is a risk factor for ARVD/C, there have been no systematic human studies. Methods Eighty-seven carriers (46 male, mean age 44±18) were interviewed about regular physical activity from age ten. The relationship of exercise with; 1) sustained ventricular arrhythmia (VT/VF), 2) stage C heart failure (HF), and 3) meeting diagnostic criteria for ARVD/C (TFC) was studied. Results Endurance athletes (n=56) developed symptoms at a younger age (30.1±13.0 vs. 40.6±21.1 years, p=0.05), were more likely to meet TFC at last follow-up (82% vs. 35%, p<0.001), and had a lower lifetime survival free from VT/VF (p=0.013) and HF (p=0.004). Compared to those who did the least (lowest quartile) exercise per year prior to presentation, those in the second (OR=6.64, p=0.013), third (OR=16.7, p=0.001), and top (OR=25.3, p<0.0001) quartiles were increasingly likely to meet TFC. Among 61 who did not present with VT/VF, the 13 subjects experiencing a first VT/VF event over a mean 8.4±6.7 year follow-up were all endurance athletes (p=0.002). Survival from first VT/VF was lowest among those who exercised most (top quartile) both prior to (p=0.036) and after (p=0.005) clinical presentation. Among individuals in the top quartile, a reduction in exercise decreased VT/VF risk (p=0.04). Conclusions Endurance exercise and frequent exercise increase risk of VT/VF, HF, and ARVD/C in desmosomal mutation carriers. These findings support exercise restriction for these patients.
Subfields: Cardiology and Cardiovascular Medicine
View Paper: https://www.semanticscholar.org/paper/037139e68618e2aa9277729a7ed28b1a129fec10
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In Cardiology and Cardiovascular Medicine
Citations in the corpus
- 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the preventionof sudden cardiac death (2015, neutral): “2 Exercise restriction Endurance training at a competitive level probably exacerbates the phenotype of ARVC.(81,403) Therefore, while there are no controlled trials demonstrating a beneficial effect, avoidance of high-level endurance training is recommended.”
- 2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy. (2019, background): “the clinical presentation had a lower incidence of ventricular arrhythmia compared with patients who continued to participate in intense and/or long-duration exercise.(253) This finding was replicated in a study of 108 probands from the North American ARVC Registry that showed patients who continued”