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AIMS: Arrhythmogenic right ventricular cardiomyopathy (ARVC), an inherited heart muscle abnormality, is a major cause of sudden cardiac death (SCD). However, the burden of SCD and risk factors in ARVC are not clearly described. Thus, we estimated the rates and predictors of SCD in ARVC in a meta-analysis. METHODS AND RESULTS: PubMed, Embase, and Web of Science were searched through 7 April 2021. Prospective studies reporting SCD from ARVC cohorts were included. Data were independently extracted by two reviewers and pooled in a random-effects meta-analysis. Fifty-two studies (n = 5485 patients) with moderate-to-low risk of bias were included. The pooled annualized rates of SCD were 0.65 per 1000 [95% confidence interval 0.00-6.43, I2 0.00%] in those with an implantable cardioverter-defibrillator (ICD) and 7.21 (2.38-13.79, I2 0.0%) in non-ICD cohorts: 7.14 in probands and 8.44 for 2010 Task Force Criteria (TFC). Multivariable predictors of life-threatening arrhythmic events including SCD were: age at presentation [adjusted hazard ratio 0.98 (0.97-0.99)], male sex [2.08 (1.29-3.36)], right ventricular (RV) dysfunction [6.99 (2.17-22.49)], QRS fragmentation [6.55 (3.33-12.90)], T-wave inversion [1.12 (1.02-1.24)], syncope at presentation [2.83 (2.40-4.08)], previous non-sustained ventricular tachyarrhythmia [2.53 (1.44-4.45)], and the TFC score [1.96 (1.02-3.76)], (P < 0.05). Predictors of appropriate ICD therapy were RV dysfunction, syncope, and inducible ventricular arrhythmia (P < 0.01). CONCLUSION: This meta-analysis demonstrates a high burden of SCD in ARVC patients, especially among probands and ARVC defined by the modified TFC. Better strategies are required to improve patient management and prevent SCD in ARVC. PROSPERO ID: CRD42020211761.

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Arrhythmogenic right ventricular cardiomyopathy, Implantable cardioverter-defibrillator, International task force criteria, Meta-analysis, Risk prediction, Risk stratification, Sudden cardiac death, Ventricular fibrillation, Ventricular tachycardia