Abstract 12449: Exercise Has a Disproportionate Role in the Pathogenesis of Non-Desmosomal Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy
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 the pathogenesis of nondesmosomal ARVD/C is uncertain.
Methods: Eighty-two patients with ARVD/C (39 desmosomal mutation carriers, all index cases) were interviewed about regular physical activity from age 10. Intensity (MET-Hrs/year) and exercise duration prior to clinical presentation were compared between patients with desmosomal and nondesmosomal ARVD/C. Association of exercise history with age at clinical presentation, family history, right ventricular structural dysfunction, and arrhythmic course was assessed.
Results: Nondesmosomal patients were more likely to be endurance athletes (100% vs. 69% desmosomal, p<0.001) and did significantly more intense (MET-Hrs/year) exercise prior to presentation (p<0.001), particularly among cases presenting age <25 (p=0.027, Figure 1-A). Family history was less prevalent among nondesmosomal patients (9% vs. 40% desmosomal, p<0.001) and nondesmosomal patients without family history did almost twice MET-Hrs/year exercise compared to other ARVD/C patients (p=0.004). Among nondesmosomal cases, patients doing the most intense (top quartile MET-Hrs/year) exercise prior to presentation had greater likelihood of meeting ARVD/C structural criteria at presentation (100% vs. 43%, p=0.02), younger age of presentation (p=0.025), and shorter survival free from a sustained ventricular arrhythmia in follow-up (p=0.002, Figure 1-B).
Conclusion: Exercise has a differential impact on development of ARVD/C among nondesmosomal patients who require higher exercise intensity for developing the disease compared to desmosomal patients. As exercise appears to negatively modify cardiac structure and promote arrhythmias in nondesmosomal ARVD/C patients, exercise restriction is warranted.
Author Disclosures: A.C. Sawant: None. A. Bhonsale: None. A.S. te Riele: None. C. Tichnell: None. B. Murray: None. S. Russell: None. H. Tandri: None. R.J. Tedford: Consultant/Advisory Board; Modest; merck. D.P. Judge: None. H. Calkins: Other Research Support; Significant; Medtronic Inc, St. Jude Medical Inc. C.A. James: None.
- © 2014 by American Heart Association, Inc.