Risk stratification of arrhythmogenic right ventricular cardiomyopathy based on signal averaged electrocardiograms

•3 + SAECG predicts worse prognosis when ARVC is suspected.•3 + SAECG predicts even worse prognosis in definite ARVC.•Non-definite ARVC patients have excellent prognosis if they have no 3 + SAECG.•SAECG should be valuable in the risk stratification of ARVC.

BackgroundSignal averaged electrocardiogram (SAECG) is a specific and non-invasive tool useful for arrhythmogenic right ventricular cardiomyopathy (ARVC) diagnosis. However, its role in risk stratification of patients with ARVC remains largely undefined.MethodsSixty-four patients fulfilling Task Force ARVC criteria (mean age: 47 ± 14 years-old, 56% male, 50% definite ARVC) were enrolled. The baseline demographic, electrocardiographic, structural, and electrophysiological characteristics were collected. Patients with SAECG fulfilling all 3 Task Force criteria (3 + SAECG) were categorized into group 1, and those fulfilled 2 or less criterion were categorized into group 2. The study endpoints were unstable ventricular arrhythmia (VA), device detectable sustained fast VA (cycle lengths < 240 ms) and cardiovascular death.ResultsDuring a mean follow-up of 21 ± 20 months, 15 primary endpoints including 12 unstable VAs and 3 device-detected fast VAs were met. One patient died of electrical storm, and one patient underwent heart transplantation. The presence of 3 + SAECG predicted malignant events in all patients with definite and non-definite ARVC (p < 0.01, OR = 30.5, 95% CI = 2.5-373.7) and in patients with definite ARVC alone (p = 0.03, OR = 11.1, 95% CI = 1.3-93.9). Patients diagnosed with non-definite ARVC without 3 + SAECG were free from malignant events.ConclusionsSAECG fulfilling all 3 Task Force criteria was an independent risk predictor of malignant events in ARVC patients. SAECG may play a valuable role in ARVC risk stratification.