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Brugada 综合征:30 年的科学探索

Brugada Syndrome: 30 Years of Scientific Adventure.

机构信息

Division of Cardiovascular Diseases, Department of Cardiology, Brussel, Belgium.

出版信息

Turk Kardiyol Dern Ars. 2022 Sep;50(6):452-458. doi: 10.5543/tkda.2022.22444.

Abstract

Thirty years ago, a distinct new clinical-electrocardiographic syndrome was described, now known as Brugada syndrome (BrS). Typical for this syndrome is EKG with ST elevation in the right precordial leads. The clinical presentation of the disease is highly variable: Patients may remain completely asymptomatic, but may also develop episodes of syncope, atrial fibrillation (AF), sick sinus syndrome (SSS), conduction disturbances, asystole, and ventricular fibrillation (VF). The disease is caused by mutations in the genes responsible for the action potential of the heart cells. The most frequently involved gene is the SCN5A which controls the structure and function of the cardiac sodium channel. Describing this new syndrome has had very positive implications in all fields of medicine.

摘要

三十年前,一种独特的新临床心电图综合征被描述出来,现在被称为 Brugada 综合征(BrS)。该综合征的典型特征是心电图右胸前导联 ST 段抬高。该病的临床表现高度可变:患者可能完全无症状,但也可能出现晕厥、心房颤动(AF)、病态窦房结综合征(SSS)、传导障碍、停搏和心室颤动(VF)。该病是由负责心肌细胞动作电位的基因突变引起的。最常涉及的基因是 SCN5A,它控制着心脏钠离子通道的结构和功能。描述这种新综合征在医学的所有领域都产生了非常积极的影响。

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