Safety and Utility of Cardiopulmonary Exercise Testing in Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

Paul J. Scheel, Roberta Florido, Steven Hsu, Brittney Murray, Crystal Tichnell, Cynthia A. James, Julia Agafonova, Harikrishna Tandri, Daniel P. Judge, Stuart D. Russell, Ryan J. Tedford, Hugh Calkins, Nisha A. Gilotra

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2 Scopus citations


Background: Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is characterized by high arrhythmic burden and progressive heart failure, which can prompt referral for heart transplantation. Cardiopulmonary exercise testing (CPET) has an established role in risk stratification for advanced heart failure therapies, but has not been described in ARVC/D. This study sought to determine the safety and prognostic utility of CPET in patients with ARVC/D. Methods and Results: Using the Johns Hopkins ARVC/D Registry, we examined patients with ARVC/D undergoing CPET. Baseline characteristics and transplant-free survival were compared on the basis of peak oxygen consumption (pVO2) (≤14 or >14 mL/kg per minute) and ventilatory efficiency (Ve/VCO2 slope ≤34 or >34). Thirty-eight patients underwent 50 CPETs. There were no sustained arrhythmic events. Twenty-nine patients achieved a maximal test. Patients with pVO2 ≤14 mL/kg per minute were more often men (P=0.042) compared with patients with pVO2 >14 mL/kg per minute. Patients with Ve/VCO2 slope >34 tended to have more moderate/severe right ventricular dilation (7/9 [78%] versus 10/26 [38%]; P=0.060) and clinical heart failure (8/9 [89%] versus 13/26 [50%]; P=0.056) compared with patients with Ve/VCO2 slope ≤34. Patients who underwent heart transplantation were more likely to have clinical heart failure (10/10 [100%] versus 13/28 [46%]; P=0.003). Patients with Ve/VCO2 slope >34 had worse transplant-free survival compared with patients with Ve/VCO2 slope ≤34 (n=35; hazard ratio, 6.57 [95% CI, 1.28–33.72]; log-rank P=0.010), whereas transplant-free survival was similar on the basis of pVO2 groups (n=29; hazard ratio, 3.38 [95% CI, 0.75–15.19]; log-rank P=0.092). Conclusions: CPET is safe to perform in patients with ARVC/D. Ve/VCO2 slope may be used for risk stratification and guide referral for heart transplantation in ARVC/D.

Original languageEnglish (US)
Article numbere013695
JournalJournal of the American Heart Association
Issue number3
StatePublished - Feb 4 2020


  • arrhythmias
  • cardiomyopathy
  • exercise testing
  • genetics
  • heart failure
  • transplantation

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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