Implantable Cardioverter-Defibrillator Therapy in Brugada Syndrome: A 30-Year Single-Center Experience.

IF 8 1区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS
Cinzia Monaco, Maria Cespon-Fernandez, Luigi Pannone, Alvise Del Monte, Domenico Della Rocca, Anais Gauthey, Sahar Mouram, Lorenzo Marcon, Giampaolo Vetta, Charles Audiat, Ioannis Doundoulakis, Antonio Bisignani, Vincenzo Miraglia, Gudrun Pappaert, Ivan Eltsov, Gezim Bala, Antonio Sorgente, Ingrid Overeinder, Alexandre Almorad, Erwin Stroker, Andrea Sarkozy, Francis Wellens, Gian Battista Chierchia, Mark La Meir, Juan Sieira, Pedro Brugada, Carlo de Asmundis
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引用次数: 0

Abstract

Background: Brugada syndrome (BrS) continues to pose clinical challenges, despite 3 decades of dedicated research and therapeutic advancements. The pivotal role of implantable cardioverter-defibrillator (ICD) therapy in safeguarding high-risk BrS patients from sudden cardiac death due to ventricular arrhythmias is undeniable. However, the debate on risk stratification and the use of ICDs for primary prevention remains ongoing.

Objectives: This study aimed to evaluate the clinical features, management, and long-term outcomes of ICD therapy in patients with Brugada syndrome.

Methods: BrS-diagnosed patients were prospectively enrolled. Inclusion criteria were: 1) a Brugada type 1 electrocardiogram pattern, either spontaneous or drug induced; 2) ICD implantation; and 3) consistent follow-up. Risk stratification was based on prior arrhythmic events, and the multiparametric Brussel risk score was used from 2017. High-risk patients underwent video-thoracoscopic epicardial ablation starting in 2016. ICD implantation strategies evolved over time, guided by patients' clinical and demographic characteristics.

Results: A total of 306 consecutive Brugada patients (186 male [61%]; mean age 41 ± 17 years; range: 1-82 years) received ICDs at our institution from 1992 to 2022. ICDs were implanted for secondary prevention in 16% of patients. Over the 3 decades, the proportions of secondary prevention implants and asymptomatic patients remained stable, while risk factors fluctuated in the first two decades before stabilizing. During long-term follow-up (median 103 months [63-147 months]), 14% of patients experienced at least 1 sustained ventricular arrhythmia (VA) (1.59 per 100 person-years), 15% had at least 1 inappropriate ICD shock-unaffected by the presence of single or dual leads-and 27% required device revision and/or lead replacement. Patients with secondary prevention ICDs had a higher incidence of both ventricular and supraventricular arrhythmias compared to those with primary prevention ICDs. Loss-of-function mutations and prior nonsustained VAs were associated with sustained VAs. Among high-risk patients, those who underwent epicardial ablation experienced significantly fewer ventricular events. The overall mortality rate was 5.88%, with 22.2% of deaths attributed to cardiac causes.

Conclusions: This 30-year study highlights ICD therapy's critical role in preventing fatal arrhythmias in Brugada syndrome, but also reveals frequent device-related complications, especially in younger patients. Thoracoscopic epicardial ablation significantly reduced VA in high-risk patients, offering a promising adjunctive therapy. These findings emphasize the need for individualized treatment strategies to balance the benefits of ICDs with their risks, and underscore the potential of ablation to improve long-term outcomes.

Brugada综合征的植入式心律转复除颤器治疗:30年单中心经验。
背景:Brugada综合征(BrS)持续带来临床挑战,尽管有30年的专门研究和治疗进展。植入式心律转复除颤器(ICD)治疗在保护高危BrS患者免于室性心律失常所致心源性猝死中的关键作用是不可否认的。然而,关于风险分层和使用icd进行一级预防的辩论仍在继续。目的:本研究旨在评估Brugada综合征患者ICD治疗的临床特征、管理和长期结果。方法:前瞻性纳入brs诊断患者。纳入标准为:1)自发性或药物诱导的Brugada 1型心电图;2) ICD植入;3)持续跟进。风险分层基于先前的心律失常事件,并从2017年开始使用多参数布鲁塞尔风险评分。高危患者从2016年开始接受胸腔镜心外膜消融。ICD植入策略随着时间的推移而发展,以患者的临床和人口特征为指导。结果:共306例Brugada患者,其中男性186例[61%];平均年龄41±17岁;范围:1-82岁)于1992年至2022年在本机构获得icd。16%的患者植入icd用于二级预防。30年来,二级预防种植体和无症状患者的比例保持稳定,而危险因素在前20年波动,然后趋于稳定。在长期随访期间(中位103个月[63-147个月]),14%的患者经历了至少1次持续性室性心律失常(VA)(1.59 / 100人年),15%的患者至少有1次不适当的ICD休克-不受单导联或双导联的影响,27%的患者需要设备修改和/或更换导联。二级预防icd患者与一级预防icd患者相比,室性心律失常和室上心律失常的发生率更高。功能丧失突变和先前的非持续VAs与持续VAs相关。在高危患者中,接受心外膜消融的患者发生的心室事件显著减少。总死亡率为5.88%,其中22.2%的死亡归因于心脏原因。结论:这项为期30年的研究强调了ICD治疗在预防Brugada综合征致死性心律失常中的关键作用,但也揭示了频繁的设备相关并发症,特别是在年轻患者中。胸腔镜心外膜消融可显著降低高危患者的VA,是一种很有前景的辅助治疗方法。这些发现强调了个性化治疗策略的必要性,以平衡icd的益处和风险,并强调了消融术改善长期预后的潜力。
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来源期刊
JACC. Clinical electrophysiology
JACC. Clinical electrophysiology CARDIAC & CARDIOVASCULAR SYSTEMS-
CiteScore
10.30
自引率
5.70%
发文量
250
期刊介绍: JACC: Clinical Electrophysiology is one of a family of specialist journals launched by the renowned Journal of the American College of Cardiology (JACC). It encompasses all aspects of the epidemiology, pathogenesis, diagnosis and treatment of cardiac arrhythmias. Submissions of original research and state-of-the-art reviews from cardiology, cardiovascular surgery, neurology, outcomes research, and related fields are encouraged. Experimental and preclinical work that directly relates to diagnostic or therapeutic interventions are also encouraged. In general, case reports will not be considered for publication.
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