title: Safety of American Heart Association-recommended minimum exercise for desmosomal mutation carriers.
abstract: 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).
  
  
  CONCLUSIONS
  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.
authors: Department of Medicine, Division of Cardiology, Johns Hopkins University, Baltimore, Maryland.
authors: Abhishek C
authors: AC
authors: Sawant
authors: Department of Medicine, Division of Cardiology, Johns Hopkins University, Baltimore, Maryland.
authors: Anneline S J M
authors: AS
authors: Te Riele
authors: Department of Medicine, Division of Cardiology, Johns Hopkins University, Baltimore, Maryland.
authors: Crystal
authors: C
authors: Tichnell
authors: Department of Medicine, Division of Cardiology, Johns Hopkins University, Baltimore, Maryland.
authors: Brittney
authors: B
authors: Murray
authors: Department of Medicine, Division of Cardiology, Johns Hopkins University, Baltimore, Maryland.
authors: Aditya
authors: A
authors: Bhonsale
authors: Department of Medicine, Division of Cardiology, Johns Hopkins University, Baltimore, Maryland.
authors: Harikrishna
authors: H
authors: Tandri
authors: Department of Medicine, Division of Cardiology, Johns Hopkins University, Baltimore, Maryland.
authors: Daniel P
authors: DP
authors: Judge
authors: Department of Medicine, Division of Cardiology, Johns Hopkins University, Baltimore, Maryland.
authors: Hugh
authors: H
authors: Calkins
authors: Department of Medicine, Division of Cardiology, Johns Hopkins University, Baltimore, Maryland. Electronic address: cjames7@jhmi.edu.
authors: Cynthia A
authors: CA
authors: James
mesh_terms: D000328:Adult; D000572:American Heart Association; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000150:complications / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology; D003896:Desmosomes / Q000235:genetics*; D015444:Exercise / Q000502:physiology*; D005190:Family; D005260:Female; D006579:Heterozygote; D006801:Humans; D016013:Likelihood Functions; D008297:Male; D008875:Middle Aged; D010807:Physical Endurance / Q000502:physiology*; D005082:Physical Exertion / Q000235:genetics; D051191:Plakophilins / Q000235:genetics*; D011314:Preventive Health Services / Q000379:methods; D018570:Risk Assessment / Q000379:methods; D017180:Tachycardia, Ventricular / Q000175:diagnosis / Q000209:etiology / Q000517:prevention & control; D014481:United States
chemical_list: C495337:PKP2 protein, human; D051191:Plakophilins
keywords: Arrhythmia; Arrhythmogenic right ventricular cardiomyopathy; Arrhythmogenic right ventricular dysplasia; Desmosome; Exercise; Plakophilin-2