Honarbakhsh Shohreh, Providencia Rui, Lambiase Pier D
Barts Heart Centre, St Bartholomew's Hospital London, UK.
Arrhythm Electrophysiol Rev. 2018 Jun;7(2):79-83. doi: 10.15420/aer.2018.2.2.
Brugada syndrome (BrS) is one of the most common inherited channelopathies associated with an increased risk of sudden cardiac death. Appropriate use of an ICD in high-risk patients is life-saving. However, there remains a lack of consensus on risk stratification, and even on the diagnosis of BrS itself. Some argue that people with a type 1 Brugada ECG pattern but no symptoms should not be diagnosed with BrS, and guidelines recommend observation without therapy in these patients. Others argue that the presence of a spontaneous (rather than drug-induced) type 1 ECG pattern alone is enough to label them as high-risk for arrhythmic events, particularly if syncope is also present. Syncope and a spontaneous type 1 ECG pattern are the only factors that have consistently been shown to predict ventricular arrhythmic events and sudden cardiac death. Other markers have yielded conflicting data. However, in combination they may have roles in risk scoring models. Epicardial catheter ablation in the right ventricular outflow tract has shown promise in studies as an alternative management option to an ICD, but longer follow-up is required to ensure that the ablation effect is permanent.
布加综合征（BrS）是最常见的遗传性离子通道病之一，与心脏性猝死风险增加相关。在高危患者中合理使用植入式心律转复除颤器（ICD）可挽救生命。然而，在风险分层甚至布加综合征本身的诊断方面仍缺乏共识。一些人认为，有1型布加综合征心电图模式但无症状的人不应被诊断为布加综合征，指南建议对这些患者进行观察而不进行治疗。另一些人则认为，仅存在自发（而非药物诱发）的1型心电图模式就足以将他们标记为心律失常事件的高危人群，特别是如果同时存在晕厥的话。晕厥和自发的1型心电图模式是唯一一直被证明可预测室性心律失常事件和心脏性猝死的因素。其他标志物产生了相互矛盾的数据。然而，它们联合起来可能在风险评分模型中发挥作用。右心室流出道的心外膜导管消融术在研究中已显示出作为ICD替代治疗选择的前景，但需要更长时间的随访以确保消融效果是永久性的。
