Netherlands Heart Institute, Utrecht, The Netherlands; Division Heart and Lungs, Department of Cardiology, University Medical Center Utrecht, University of Utrecht, Utrecht, The Netherlands.
Division Heart and Lungs, Department of Cardiology, University Medical Center Utrecht, University of Utrecht, Utrecht, The Netherlands.
Heart Rhythm. 2018 Jul;15(7):1097-1107. doi: 10.1016/j.hrthm.2018.01.031. Epub 2018 Feb 3.
While many studies evaluate predictors of ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy (ARVC), a systematic review consolidating this evidence is currently lacking. Therefore, we searched MEDLINE and Embase for studies analyzing predictors of ventricular arrhythmias (sustained ventricular tachycardia/fibrillation (VT/VF), appropriate implantable cardioverter-defibrillator therapy, or sudden cardiac death) in patients with definite ARVC, patients with borderline ARVC, and ARVC-associated mutation carriers. In the case of multiple publications on the same cohort, the study with the largest population was included. This yielded 45 studies with a median cohort size of 70 patients (interquartile range 60 patients) and a median follow-up of 5.0 years (interquartile range 3.3 - 6.7 years). The average proportion of arrhythmic events observed was 10.6%/y in patients with definite ARVC, 10.0%/y in patients with borderline ARVC, and 3.7%/y with mutation carriers. Predictors of ventricular arrhythmias were population dependent: consistently predictive risk factors in patients with definite ARVC were male sex, syncope, T-wave inversion in lead >V, right ventricular dysfunction, and prior (non)sustained VT/VF; in patients with borderline ARVC, 2 additional predictors-inducibility during electrophysiology study and strenuous exercise-were identified; and with mutation carriers, all aforementioned predictors as well as ventricular ectopy, multiple ARVC-related pathogenic mutations, left ventricular dysfunction, and palpitations/presyncope determined arrhythmic risk. Most evidence originated from small observational cohort studies, with a moderate quality of evidence. In conclusion, the average risk of ventricular arrhythmia ranged from 3.7 to 10.6%/y depending on the population with ARVC. Male sex, syncope, T-wave inversion in lead >V, right ventricular dysfunction, and prior (non)sustained VT/VF consistently predict ventricular arrhythmias in all populations with ARVC.
虽然许多研究评估了致心律失常性右心室心肌病 (ARVC) 中心律失常的预测因素，但目前缺乏对这些证据进行系统综述。因此，我们在 MEDLINE 和 Embase 中搜索了分析明确 ARVC 患者、边界性 ARVC 患者和 ARVC 相关突变携带者的心律失常预测因素（持续性室性心动过速/颤动 (VT/VF)、适当的植入式心脏复律除颤器治疗或心源性猝死）的研究。在同一队列有多份出版物的情况下，我们纳入了队列规模最大的研究。这产生了 45 项研究，中位数队列规模为 70 例患者（四分位间距为 60 例患者），中位数随访时间为 5.0 年（四分位间距为 3.3-6.7 年）。明确 ARVC 患者的心律失常事件发生率平均为 10.6%/年，边界性 ARVC 患者为 10.0%/年，突变携带者为 3.7%/年。心律失常的预测因素与人群有关：在明确 ARVC 患者中，一致具有预测意义的危险因素为男性、晕厥、导联 >V 的 T 波倒置、右心室功能障碍和既往（非）持续性 VT/VF；在边界性 ARVC 患者中，另外两个预测因素-电生理研究中的诱发性和剧烈运动-被识别；在突变携带者中，所有上述预测因素以及室性早搏、多个 ARVC 相关致病性突变、左心室功能障碍和心悸/头晕确定了心律失常风险。大多数证据来自小型观察性队列研究，证据质量中等。总之，根据 ARVC 人群，室性心律失常的平均风险范围为 3.7%至 10.6%/年。男性、晕厥、导联 >V 的 T 波倒置、右心室功能障碍和既往（非）持续性 VT/VF 在所有 ARVC 人群中一致预测心律失常。
