Impaired Atrial and Ventricular Strain Predicts Heart Failure in Arrhythmogenic Right Ventricular Cardiomyopathy

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) increases the risk of heart failure (HF) and arrhythmias. Speckle-tracking echocardiography (STE) detects myocardial dysfunction, but its predictive role for HF in this population remains unclear. Methods: Seventy-one patients with ARVC (age 43.7 ± 14.8 years, 53.5% male) without prevalent HF at baseline who were enrolled in the Johns Hopkins ARVC Registry were retrospectively included. Global strain (GS) and strain rate (SR) of the left ventricle (LV), right ventricle free wall (RVFW), left atrium (LA), and right atrium (RA) were measured by a blinded operator. Cox regression models assessed their association with incident HF. Results: Incident HF developed in 23 patients (age 49.3 ± 12.5 years, 52.2% male) during a median follow-up of 2.7 years. Decreases in strain were significantly associated with HF: LV peak global longitudinal systolic strain (GLS; hazard ratio [HR] 1.20, 95% confidence interval [CI] 1.06-1.35; P = 0.003), RVFW strain (HR 1.11, 95% CI 1.04-1.18; P = 0.003), LA GS (HR 1.05, 95% CI 1.00-1.09; P = 0.030), and RA GS (HR 1.07, 95% CI 1.03-1.12; P < 0.001). Associations for LV GLS, RVFW strain, and RA GS remained significant after adjusting for age and sex. Strain values frequently fell below established reference ranges. Any strain value (LV GLS, RVFW strain, LA GS, or RA GS) below the normal limit was associated with an 8-fold increase in HF (HR 8.43, 95% CI 1.97-36.02; P = 0.004), and each individual component below the normal threshold doubled the risk (HR 2.35, 95% CI 1.60-3.45; P < 0.001). Conclusions: STE deformation abnormalities are associated with incident HF in ARVC patients. Echocardiographic strain may aid in identifying patients at risk of HF for closer follow-up and management.