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Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited condition associated with ventricular arrhythmias and myocardial dysfunction; however, limited data exist on identifying patients at highest risk. The purpose of the study was to determine whether measures of right ventricular (RV) dysfunction on echocardiogram including RV strain were predictive of structural disease progression in ARVC. Methods and Results A retrospective analysis of serial echocardiograms from 40 patients fulfilling 2010 task force criteria for ARVC was performed to assess structural progression defined by an increase in proximal RV outflow tract dimensions (parasternal short or long axis) or decrease in RV fractional area change. Echocardiograms were analyzed for RV free‐wall peak longitudinal systolic strain using 2‐dimensional speckle tracking. Risk of structural progression and 5‐year change in RV outflow tract measurements were compared with baseline RV strain. Of the 40 ARVC patients, 61% had structural progression with an increase in the mean parasternal short‐axis RV outflow tract dimension from 36.2 to 38.5 mm (P=0.022) and 68% by increase in parasternal long‐axis RV outflow tract dimension from 36.1 to 39.2 mm (P=0.001). RV fractional area change remained stable over time. Baseline RV strain was significantly associated with the risk of structural progression and 5‐year rate of change. Patients with an RV strain more positive than −20% had a higher risk (odds ratio: 18.4; 95% CI, 2.7–125.8; P=0.003) of structural progression. Conclusions RV free wall strain is associated with the rate of structural progression in patients with ARVC. It may be a useful marker in determining which patients require closer follow‐up and treatment.

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.