PT - JOURNAL ARTICLE AU - Olgierd Woźniak AU - Karolina Borowiec AU - Marek Konka AU - Alicja Cicha-Mikołajczyk AU - Andrzej Przybylski AU - Łukasz Szumowski AU - Piotr Hoffman AU - Krzysztof Poślednik AU - Elżbieta Katarzyna Biernacka TI - Implantable cardiac defibrillator events in patients with arrhythmogenic right ventricular cardiomyopathy AID - 10.1136/heartjnl-2020-318415 DP - 2021 Mar 04 TA - Heart PG - heartjnl-2020-318415 4099 - http://heart.bmj.com/content/early/2021/03/03/heartjnl-2020-318415.short 4100 - http://heart.bmj.com/content/early/2021/03/03/heartjnl-2020-318415.full AB - Objective Arrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with a risk of sudden cardiac death. Optimal risk stratification is still under debate. The main purpose of this long-term, single-centre observation was to analyse predictors of appropriate and inappropriate implantable cardioverter-defibrillator (ICD) interventions in the population of patients with ARVC with a high risk of life-threatening arrhythmias.Methods The study comprised 65 adult patients (median age 40 years, 48 men) with a definite diagnosis of ARVC who received ICD over a time span of 20 years in primary (40%) or secondary (60%) prevention of sudden cardiac death. The study endpoints were first appropriate and inappropriate ICD interventions (shock or antitachycardia pacing) after device implantation.Results During a median follow-up of 7.75 years after ICD implantation, nine patients died and six individuals underwent heart transplantation. Appropriate ICD interventions occurred in 43 patients (66.2%) and inappropriate ICD interventions in 18 patients (27.7%). Multivariable analysis using cause-specific hazard model identified three predictors of appropriate ICD interventions: right ventricle dysfunction (cause-specific HR 2.85, 95% CI 1.56 to 5.21, p<0.001), age <40 years at ICD implantation (cause-specific HR 2.37, 95% CI 1.13 to 4.94, p=0.022) and a history of sustained ventricular tachycardia (cause-specific HR 2.55, 95% CI 1.16 to 5.63, p=0.020). Predictors of inappropriate ICD therapy were not found. Complications related to ICD implantation occurred in 12 patients.Conclusions Right ventricle dysfunction, age <40 years and a history of sustained ventricular tachycardia were predictors of appropriate ICD interventions in patients with ARVC. The results may be used to improve risk stratification before ICD implantation.