Arrhythmia Section, Cardiology Department, Thorax Institute, Hospital Clinic, C/Villarroel 170, 6° Planta - Escalera 3, 08036, Barcelona, Spain,
Curr Cardiol Rep. 2014 Jul;16(7):508. doi: 10.1007/s11886-014-0508-1.
The Brugada syndrome (BS) is a hereditary disease characterized by typical electrocardiographic alterations (elevation of the ST segment in right precordial leads) that affects young individuals without structural heart disease, predisposing them to ventricular arrhythmias and sudden death (SD). Several genetic mutations of different subunits of the sodium, calcium and potassium channel have been involved. The majority of patients with BS remain asymptomatic; however, the most frequent symptoms are syncope and/or SD secondary to polymorphic ventricular tachycardia (PVT) or ventricular fibrillation (VF). Electrocardiographic manifestations of the BS are typically dynamic and occasionally only become apparent after the administration of a sodium channel blocker or with fever. Risk stratification is mainly based on symptoms and the surface electrocardiogram. However, in asymptomatic patients, risk evaluation is still controversial and requires further studies. This review provides an updated summary of the BS from the point of view of genetic, clinical manifestations, risk stratification and management.
Brugada 综合征（BS）是一种遗传性疾病，其特征为典型的心电图改变（右侧胸前导联 ST 段抬高），影响无结构性心脏病的年轻个体，使他们易发生室性心律失常和猝死（SD）。已涉及钠离子、钙离子和钾离子通道的不同亚基的几种基因突变。大多数 Brugada 综合征患者无症状；然而，最常见的症状是晕厥和/或多形性室性心动过速（PVT）或心室颤动（VF）继发的 SD。BS 的心电图表现通常是动态的，偶尔仅在给予钠通道阻滞剂或发热后才变得明显。危险分层主要基于症状和体表心电图。然而，在无症状患者中，风险评估仍存在争议，需要进一步研究。本综述从遗传、临床表现、危险分层和治疗的角度提供了 Brugada 综合征的最新总结。
