Safety of American Heart Association-recommended minimum exercise for desmosomal mutation carriers

Heart Rhythm. 2016 Jan;13(1):199-207. doi: 10.1016/j.hrthm.2015.08.035. Epub 2015 Aug 29.


Background: Endurance exercise is associated with adverse outcomes in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). Exercise recommendations for family members remain undetermined.

Objective: The purposes of this study were to determine if (1) endurance exercise (Bethesda class C) and exercise intensity (metabolic equivalent hours per year [MET-Hr/year]) increase the likelihood of fulfilling 2010 Task Force Criteria and ventricular arrhythmias/implantable cardioverter-defibrillator shock (ventricular tachycardia/ventricular fibrillation [VT/VF]), and (2) exercise restriction to the American Heart Association (AHA)-recommended minimum for healthy adults is associated with favorable outcomes of at-risk family members.

Methods: Twenty-eight family members of 10 probands inheriting a PKP2 mutation were interviewed about exercise from age 10. Exercise threshold to maintain overall health was based on the 2007 AHA guidelines of a minimum 390 to 650 MET-Hr/year.

Results: After adjustment for age, sex, and family membership, both participation in endurance athletics (odds ratio [OR] 7.4, P = .03) and higher-intensity exercise (OR = 4.2, P = .004) were associated with diagnosis (n = 13). Endurance athletes were also significantly more likely to develop VT/VF (n = 6, P = .02). Family members who restricted exercise at or below the upper bound of the AHA goal (≤650 MET-Hr/year) were significantly less likely to be diagnosed (OR = 0.07, P = .002) and had no VT/VF. At diagnosis and first VT/VF, family members had accumulated 2.8-fold (P = .002) and 3.5-fold (P = .03), respectively, greater MET-Hr exercise than the AHA-recommended minimum. Those who developed VT/VF had performed particularly high-intensity exercise in adolescence compared to unaffected family members (age 10-14: P = .04; age 14-19: P = .02).

Conclusion: The results of this study suggest restricting unaffected desmosomal mutation carriers from endurance and high-intensity athletics but potentially not from AHA-recommended minimum levels of exercise for healthy adults.

Keywords: Arrhythmia; Arrhythmogenic right ventricular cardiomyopathy; Arrhythmogenic right ventricular dysplasia; Desmosome; Exercise; Plakophilin-2.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • American Heart Association
  • Arrhythmogenic Right Ventricular Dysplasia* / complications
  • Arrhythmogenic Right Ventricular Dysplasia* / diagnosis
  • Arrhythmogenic Right Ventricular Dysplasia* / genetics
  • Arrhythmogenic Right Ventricular Dysplasia* / physiopathology
  • Desmosomes / genetics*
  • Exercise / physiology*
  • Family
  • Female
  • Heterozygote
  • Humans
  • Likelihood Functions
  • Male
  • Middle Aged
  • Physical Endurance / physiology*
  • Physical Exertion / genetics
  • Plakophilins / genetics*
  • Preventive Health Services / methods
  • Risk Assessment / methods
  • Tachycardia, Ventricular* / diagnosis
  • Tachycardia, Ventricular* / etiology
  • Tachycardia, Ventricular* / prevention & control
  • United States


  • PKP2 protein, human
  • Plakophilins