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  - 2022 Jan 01
TA  - Heart
PG  - 22--28
VI  - 108
IP  - 1
4099  - http://heart.bmj.com/content/108/1/22.short
4100  - http://heart.bmj.com/content/108/1/22.full
SO  - Heart2022 Jan 01; 108
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.All data relevant to the study are included in the article or uploaded as supplementary information.