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

摘要

IntroductionHeart failure (HF) is an increasingly recognized outcome in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). Though cardiopulmonary exercise testing (CPET) serves as a prognostic tool in advanced HF, it is rarely used in ARVC/D due to perceived arrhythmia risk and limited data. We sought to determine the safety of CPET and demonstrate utility of Ve/VCO2slope in ARVC/D. MethodsUsing the Johns Hopkins ARVC/D registry, we retrospectively identified and analyzed 46 CPETs from 33 patients meeting 2010 Task Force Criteria. Each patient's most recent CPET was used to evaluate the association of normalized maximal oxygen uptake (peak VO2) and ventilatory efficiency (Ve/VCO2slope) with transplant-free survival using Kaplan-Meier graphs and log-rank test. Baseline characteristics were compared between groups using Mann-Whitney U and Fisher exact tests. ResultsThe majority of the 46 CPETs were performed without complications. Arrhythmia occurred infrequently and was asymptomatic: PVCs (n = 1), bigeminy (n = 1) and NSVT (n = 2). Normalized peak VO2(pVO2) ranged from 9.7 to 54.8 mL/kg/min. Ve/VCO2slope ranged from 21.9 to 65.2. Baseline demographics including race, sex, comorbidities, age at diagnosis, and mutation did not differ among those with pVO2≤14 (n = 8) vs pVO2>14 (n = 25) or Ve/VCO2slope ≥33 (n = 12) vs Ve/VCO2slope < 33 (n = 17). Clinical HF was more common in the group with pVO2≤14 (13/24 (54%) vs. 8/8 (100%),P =0.005) and in the group with Ve/VCO2slope≥33 (8/16 (50%) vs. 11/12 (92%),P =0.005). Decreased transplant-free survival was demonstrated in patients with Ve/VCO2slope≥33 (hazard ratio 11.95, 95% CI 1.40-101.74,P= 0.023; ) but not in patients with pVO2≤14 (hazard ratio 2.32, 95% CI 0.67-8.07,P= 0.186). Additionally, patients with higher Ve/VCO2slope tended to have more severe RV dysfunction by echo (). ConclusionIn patients with ARVC/D, CPET is safe to perform. Additionally, Ve/VCO2slope may have utility in risk stratification in this predominantly RV disease.