布鲁加达综合征:2017年更新。(RCD代码:V-1A.1)

Paweł Rubiś
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引用次数: 1

摘要

Brugada综合征(BrS)传统上被认为是原发性通道病,最常见的原因是内钠电流减少,晕厥和心源性猝死(SCD)的风险增加。根据最近的指南,右心前导联V1和V2中形态为1型的ST段抬高患者可诊断为BrS,可能是自发发生的,也可能是在静脉注射I类抗心律失常药物的刺激药物试验后发生的。此外,当静脉注射I类抗心律失常药物引起I型心电图形态时,右侧心前导联ST段抬高≥1导联的2型或3型患者可诊断为BrS。SCD的风险分层是对这些患者进行简明管理的最重要方面。重要的是,曾经被认为是纯粹的心律失常综合征,现在,越来越多的人认识到BrS可能存在一些结构异常。因此,成像在这一领域得到了积极的研究。Brugada综合征可被视为一种罕见疾病,患病率为千分之一至万分之一,在东南亚比在西方国家更为常见。因此,这一主题已经在《罕见心血管疾病杂志》上进行了探讨(见参考文献)。本评论的目的是向读者介绍本实体的主要发展情况。
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Brugada syndrome: 2017 update. (RCD code: V-1A.1)
Brugada Syndrome (BrS) is traditionally considered a primary channelopathy, most commonly due to reduced inward sodium current with an increased risk of syncope and sudden cardiac death (SCD). According to the recent guidelines, BrS is diag‐ nosed in patients with ST‐segment elevation with type 1 morphol‐ ogy among the right precordial leads V1 and V2, occurring either spontaneously or after provocative drug test with intravenous administration of Class I antiarrhythmic drugs. Moreover, BrS is diagnosed in patients with type 2 or type 3 ST‐segment elevation in ≥1 lead among the right precordial leads when a provocative drug test with intravenous administration of Class I antiarrhyth‐ mic drugs induces a type I ECG morphology. Risk stratification of SCD is the most important aspect of the concise management of those patients. Importantly, once considered pure arrhyth‐ mic syndrome, nowadays, there is growing understanding that some structural abnormalities may be present in BrS. Therefore, imaging is actively investigated in this field. Brugada syndrome can be considered as a rare disease, with the prevalence of 1 in 1000 to 1 in 10 000, being more frequent in south‐east Asia than in the western countries. Thus, this topic has been already explored in the Journal of Rare Cardiovascular Disease (see references be‐ low). The purpose of this Review is to update the Readers with the main developments in this entity.
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来源期刊
Journal of Rare Cardiovascular Diseases
Journal of Rare Cardiovascular Diseases Medicine-Cardiology and Cardiovascular Medicine
自引率
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审稿时长
23 weeks
期刊介绍: Journal of Rare Cardiovascular Diseases (JRCD) is an international, quarterly issued, peer-reviewed, open access, online journal that keeps cardiologists and non-cardiologists up-to-date with rare disorders of the heart and vessels. The Journal publishes fine quality review articles, original, basic and clinical sciences research papers, either positive or negative, case reports and articles on public health issues in the field of rare cardiovascular diseases and orphan cardiovascular drugs. Topics of interest include, but are not limited to the following areas: (1) rare diseases of systemic circulation (2) rare diseases of pulmonary circulation (3) rare diseases of the heart (cardiomyopathies) (4) rare congenital cardiovascular diseases (5) rare arrhythmogenic disorders (6) cardiac tumors and cardiovascular diseases in malignancy (7) cardiovascular diseases in pregnancy (8) basic science (9) quality of life
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