Department of Cardiology, Thoraxcenter, Erasmus MC, Rotterdam, The Netherlands.
Circ Arrhythm Electrophysiol. 2013 Jun;6(3):562-8. doi: 10.1161/CIRCEP.113.000392. Epub 2013 May 14.
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a cardiomyopathy characterized by ventricular arrhythmias and an abnormal right ventricle. Implantable cardioverter defibrillator (ICD) therapy may prevent sudden cardiac death in patients with ARVD/C. Currently, an overview of outcomes, appropriate and inappropriate interventions, and complications of ICD therapy in ARVD/C is lacking.
A literature search was performed to identify studies reporting outcome and complications in patients with ARVD/C who underwent ICD implantation. Of 641 articles screened, 24 studies on 18 cohorts were eligible for inclusion. In case of multiple publications on a cohort, the most recent publication was included in the meta-analysis. There were 610 patients (mean age, 40.4 years; 42% women), who had an ICD for primary or secondary prevention of sudden cardiac death. Risk factors for sudden cardiac death were presyncope (61%), syncope (31%), previous cardiac arrest (14%), ventricular tachycardia (58%), and ventricular fibrillation (6%). Antiarrhythmic medication consisted mostly of β-blockers (38%), amiodarone (14%), or sotalol (30%). During the 3.8-year follow-up, annualized cardiac mortality rate was 0.9%, annualized noncardiac mortality rate was 0.8%, and annualized heart transplant rate was 0.9%. The annualized appropriate and inappropriate ICD intervention rates were 9.5% and 3.7%, respectively. ICD-related complications consisted of difficult lead placement (18.4%), lead malfunction (9.8%), infection (1.4%), lead displacement (3.3%), and any complication (20.3%).
Cardiac and noncardiac mortality rates after ICD implantation in patients with ARVD/C are low. Appropriate ICD interventions occur at a rate of 9.5%/y. Inappropriate ICD interventions and complications lead to considerable ICD-related morbidity.
致心律失常性右室心肌病（ARVD/C）是一种以室性心律失常和右心室异常为特征的心肌病。植入式心脏复律除颤器（ICD）治疗可预防 ARVD/C 患者的心脏性猝死。目前，缺乏 ARVD/C 患者 ICD 治疗的结局、适当和不适当干预以及并发症的概述。
进行了文献检索，以确定报道 ARVD/C 患者接受 ICD 植入后结局和并发症的研究。在筛选出的 641 篇文章中，有 24 项研究纳入了 18 个队列。如果一个队列有多篇文献发表，那么包含最新信息的文献将被纳入荟萃分析。共有 610 例患者（平均年龄 40.4 岁，42%为女性）因心脏性猝死的一级或二级预防而植入 ICD。心脏性猝死的危险因素包括先兆晕厥（61%）、晕厥（31%）、既往心脏骤停（14%）、室性心动过速（58%）和心室颤动（6%）。抗心律失常药物主要包括β受体阻滞剂（38%）、胺碘酮（14%）或索他洛尔（30%）。在 3.8 年的随访期间，每年的心脏死亡率为 0.9%，每年的非心脏死亡率为 0.8%，每年的心脏移植率为 0.9%。每年适当和不适当的 ICD 干预率分别为 9.5%和 3.7%。ICD 相关并发症包括导线放置困难（18.4%）、导线故障（9.8%）、感染（1.4%）、导线移位（3.3%）和任何并发症（20.3%）。
ARVD/C 患者植入 ICD 后的心脏和非心脏死亡率较低。适当的 ICD 干预率为 9.5%/年。不适当的 ICD 干预和并发症导致相当大的 ICD 相关发病率。
