Sudden Cardiac Death Prediction in Arrhythmogenic Right Ventricular Cardiomyopathy

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with arrhythmias (VA) and sudden cardiac death (SCD). A model was recently developed to predict incident sustained VA in patients ARVC. However, since this outcome may overestimate the risk for SCD, we aimed specifically life-threatening (LTVA) as a closer surrogate SCD. Methods: We assembled retrospective cohort of definite ARVC cases from 15 centers North America Europe. Association 8 prespecified clinical predictors LTVA (SCD, aborted sustained, or implantable cardioverter-defibrillator treated tachycardia >250 beats per minute) follow-up assessed by Cox regression backward selection. Candidate variables included age, sex, prior (?30s, hemodynamically unstable, tachycardia; SCD), syncope, 24-hour premature complexes count, number anterior inferior leads T-wave inversion, left ejection fraction. The resulting internally validated using bootstrapping. Results: total 864 (40±16 years; 53% male) were included. Over 5.75 years (interquartile range, 2.77–10.58) follow-up, 93 (10.8%) experienced including SCD/aborted SCD (1.7%). Of predictors, only 4 (younger male complex inversion) LTVA. Notably, did not subsequent ( P =0.850). these had an optimism-corrected C-index 0.74 (95% CI, 0.69–0.80) calibration slope 0.95 0.94–0.98) indicating minimal over-optimism. Conclusions: events can be predicted novel simple prediction predictors. Prior extent functional heart disease are events.