Wellcome Centre for Human Genetics, Nuffield Department of Medicine, University of Oxford, Oxford, UK.
Adelaide Medical School, Faculty of Health and Medical Sciences, The University of Adelaide, Adelaide, Australia.
Europace. 2022 Oct 13;24(10):1665-1674. doi: 10.1093/europace/euac014.
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.
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).
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.
致心律失常性右室心肌病（ARVC）是一种遗传性心肌异常，是心脏性猝死（SCD）的主要原因。然而，ARVC 中的 SCD 负担和危险因素尚不清楚。因此，我们通过荟萃分析来评估 ARVC 中的 SCD 发生率和预测因素。
我们通过 2021 年 7 月 7 日检索 PubMed、Embase 和 Web of Science，纳入了报告 ARVC 队列中 SCD 的前瞻性研究。两名评审员独立提取数据，并进行随机效应荟萃分析。纳入了 52 项研究（n=5485 例患者），其偏倚风险为中低水平。植入式心脏复律除颤器（ICD）组的 SCD 年化发生率为 0.65/1000（95%置信区间 0.00-6.43，I2 0.00%），非 ICD 组为 7.21（2.38-13.79，I2 0.0%）：先证者为 7.14，2010 年工作组标准（TFC）为 8.44。包括 SCD 在内的危及生命的心律失常事件的多变量预测因素为：发病年龄[校正后的危险比 0.98（0.97-0.99）]、男性[2.08（1.29-3.36）]、右心室（RV）功能障碍[6.99（2.17-22.49）]、QRS 碎裂[6.55（3.33-12.90）]、T 波倒置[1.12（1.02-1.24）]、首发晕厥[2.83（2.40-4.08）]、既往非持续性室性心动过速[2.53（1.44-4.45）]和 TFC 评分[1.96（1.02-3.76）]（P<0.05）。RV 功能障碍、晕厥和诱发性室性心律失常是 ICD 治疗恰当的预测因素（P<0.01）。
这项荟萃分析表明 ARVC 患者的 SCD 负担很高，尤其是在经修正的 TFC 定义的先证者和 ARVC 患者中。需要更好的策略来改善患者管理，预防 ARVC 中的 SCD。PROSPERO 注册号：CRD42020211761。
