Brugada Syndrome: Clinical, Genetic, Molecular, Cellular, and Ionic Aspects.
Brugada Syndrome: Clinical, Genetic, Molecular, Cellular, and Ionic Aspects.
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DOI：
10.1016/j.cpcardiol.2015.06.002
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发表时间：
2016-01
影响因子：
4.2
通讯作者：
Patocskai B
中科院分区：
文献类型：
--
作者：
Antzelevitch C;Patocskai B
Brugada综合征（BrS）是一种遗传性心律失常综合征，于1992年首次作为一种新的临床实体被描述。心电图特征为右胸导联ST段明显弯曲型抬高，该综合征与年轻人心源性猝死的高风险相关，在婴儿和儿童中较少发生。BrS的ECG表现通常被隐藏，并且可能被钠通道阻滞剂、发热状态、迷走神经张力剂以及三环和四环抗抑郁药掩盖或加重。植入式心律转复除颤器（ICD）是最广泛接受的治疗方法。药物治疗旨在使右心室动作电位早期活动电流平衡向内偏移，可用于中止电风暴或在无法使用ICD时作为器械治疗的辅助或替代。异丙肾上腺素、西洛他唑和米力农增强钙通道电流，奎尼丁、苄普地尔和中药提取物稳心颗粒等药物抑制瞬时外向电流，从而减少动作电位（AP）切迹，从而抑制VT/VF的底物和触发。BrS患者的右心室流出道心外膜的放射性消融最近已被证明可以减少心肌易损性和疾病的ECG表现，可能是通过破坏具有更突出AP切迹的细胞。本文综述了BrS的临床、遗传、分子和细胞方面以及治疗方法。
The Brugada syndrome (BrS) is an inherited cardiac arrhythmia syndrome first described as a new clinical entity in 1992. Electrocardiographically characterized by distinct coved type ST segment elevation in the right precordial leads, the syndrome is associated with a high risk for sudden cardiac death in young adults, and less frequently in infants and children. The ECG manifestations of the BrS are often concealed and may be unmasked or aggravated by sodium channel blockers, a febrile state, vagotonic agents, as well as by tricyclic and tetracyclic antidepressants. An implantable cardioverter defibrillator (ICD) is the most widely accepted approach to therapy. Pharmacological therapy is designed to produce an inward shift in the balance of currents active during the early phases of the right ventricular action potential and can be used to abort electrical storms or as an adjunct or alternative to device therapy when use of an ICD is not possible. Isoproterenol, cilostazol and milrinone boost calcium channel current and drugs like quinidine, bepridil and the Chinese herb extract Wenxin Keli inhibit the transient outward current, acting to diminish the action potential (AP) notch and thus to suppress the substrate and trigger for VT/VF. Radiofrequency ablation of the right ventricular outflow tract epicardium of BrS patients has recently been shown to reduce arrhythmia-vulnerability and the ECG-manifestation of the disease, presumably by destroying the cells with more prominent AP notch. This review provides an overview of the clinical, genetic, molecular and cellular aspects of the BrS as well as the approach to therapy.