关键词: Brugada syndrome Implantable cardioverter–defibrillator Sudden cardiac death Ventricular fibrillation

来  源:   DOI:10.1186/s43044-024-00485-3   PDF(Pubmed)

Abstract:
BACKGROUND: Brugada syndrome (BrS) is an inherited arrhythmogenic syndrome characterized by cove-shaped ST-segment elevation in leads V1-V3 and incomplete or complete right bundle branch block. BrS exhibits autosomal dominant inheritance with incomplete penetrance and a male predominance. It carries a significant risk of sudden cardiac death due to ventricular fibrillation (VF).
METHODS: Recent studies have highlighted the presence of epicardial fibrosis as a proarrhythmic substrate in BrS, revolutionizing our understanding of the disease\'s pathophysiology. Catheter ablation has emerged as a crucial intervention for symptomatic BrS patients experiencing recurrent episodes of ventricular tachycardia (VT) or VF. By potentially obviating the need for implantable cardioverter-defibrillator (ICD) implantation, epicardial ablation offers a promising therapeutic approach.
CONCLUSIONS: This review emphasizes the significance of current evidence and ongoing research in shaping the role of epicardial ablation as a curative strategy in BrS management, highlighting its potential benefits and the necessity for further investigation.
摘要:
背景:Brugada综合征(BrS)是一种遗传性致心律失常综合征,其特征是V1-V3导联的卵形ST段抬高和不完全或完全的右束支传导阻滞。BrS表现出常染色体显性遗传,外显率不完全,男性占优势。由于心室纤颤(VF),它具有严重的心脏猝死风险。
方法:最近的研究强调了在BrS中存在心外膜纤维化作为心律失常基质,彻底改变了我们对疾病病理生理学的理解。导管消融已成为有症状的BrS患者发生室性心动过速(VT)或VF反复发作的关键干预措施。通过潜在地消除植入式心律转复除颤器(ICD)植入的需要,心外膜消融提供了一种有希望的治疗方法。
结论:这篇综述强调了当前证据和正在进行的研究在塑造心外膜消融作为BrS治疗中的治疗策略的作用方面的重要性。强调其潜在的好处和进一步调查的必要性。
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