Department of Medicine, Division of Cardiology, Johns Hopkins University, Baltimore, MD, USA.
J Cardiovasc Electrophysiol. 2018 Oct;29(10):1396-1402. doi: 10.1111/jce.13668. Epub 2018 Jun 27.
Implantable defibrillators (ICD) are an important therapy for arrhythmogenic right ventricular cardiomyopathy (ARVC) patients at high risk of sudden death. Given the high appropriate ICD therapy rate, some have argued that the mere act of implanting an ICD inflates the malignant arrhythmia rate in ARVC.
To report the arrhythmic course of ARVC patients without ICDs at the fulfillment of the 2010 Task Force Criteria and explore predictors of malignant ventricular arrhythmias.
We included 131 definite ARVC patients (age 32 ± 15 years, male 39%, proband 50%) either without ICDs (N = 47) or receiving an ICD at least 6 months after the fulfillment of the diagnostic criteria. The primary outcome was a composite of cardiac arrest (both resuscitated successfully and unsuccessfully) and sustained ventricular tachyarrhythmias (cycle length< 600 milliseconds, at least 30 seconds or requiring an intervention for termination).
At the fulfillment of the diagnostic criteria, ICDs were not recommended to 59 (45%) patients and declined by 22 (17%) patients. Forty (31%) patients were not recognized as having ARVC by the treating physicians. Over 8 (interquartile interval: 3-12) years, 38 (29%) patients had primary outcomes (8 cardiac arrests [3 died] and 30 sustained ventricular arrhythmias) while not having ICDs. The 1-year and 5-year event-free survival was 92% and 72%. Spontaneous sustained ventricular arrhythmias, cardiac syncope, men, proband, and inducibility in electrophysiology study were significantly associated with the primary outcome.
In a contemporary cohort, a considerable risk of malignant arrhythmias existed in ARVC when ICDs were not implanted.
植入式心脏除颤器（ICD）是高危致心律失常性右心室心肌病（ARVC）患者猝死的重要治疗手段。鉴于适当 ICD 治疗率较高，有人认为植入 ICD 本身就会夸大 ARVC 的恶性心律失常发生率。
报告满足 2010 年工作组标准的 ARVC 患者在未植入 ICD 时的心律失常病程，并探讨恶性室性心律失常的预测因素。
我们纳入了 131 例明确的 ARVC 患者（年龄 32±15 岁，男性占 39%，先证者占 50%），分为未植入 ICD 组（n=47）和植入 ICD 组（植入时间在满足诊断标准后至少 6 个月）。主要终点为心脏骤停（复苏成功和失败）和持续性室性心动过速（周长<600 毫秒，至少 30 秒或需要干预终止）的复合终点。
在满足诊断标准时，有 59 例（45%）患者不建议植入 ICD，22 例（17%）患者拒绝植入。40 例（31%）患者未被治疗医生识别为 ARVC。在 8 年（四分位间距：3-12 年）的随访中，38 例（29%）未植入 ICD 的患者出现了主要终点事件（8 例心脏骤停[3 例死亡]和 30 例持续性室性心律失常）。1 年和 5 年无事件生存率分别为 92%和 72%。自发性持续性室性心律失常、心脏性晕厥、男性、先证者和电生理检查的诱发性与主要终点显著相关。
在当代队列中，未植入 ICD 的 ARVC 患者存在相当大的恶性心律失常风险。
