Paparella G, Sarkozy A, Brugada P
Heart Rhythm Management Center, UZ Brussels, Brussels, Belgium.
Minerva Med. 2009 Aug;100(4):307-19.
Since its first description in 1992 as a new clinical entity, the Brugada syndrome has stimulated great interest among physicians and basic scientists. In 2002 and 2005, two consensus conferences have respectively defined the diagnostic criteria for the syndrome. Currently the diagnosis of Brugada syndrome is based on a combination of clinical events (syncope and/or sudden cardiac death due to malignant ventricular arrhythmias) and electrocardiographic features (pathognomonic ST-segment elevation morphology). In the last years, many advances have been done in the knowledge about the genetic basis, the cellular mechanisms responsible for the typical electrocardiography features, susceptibility to ventricular arrhythmias and risk stratification. The implantable cardioverter defibrillator remains the only therapeutic option of proven efficacy for primary and secondary prophylaxis of sudden cardiac death. Identification of high risk subjects is one of the major goals in clinical decision-making. Syncope is ubiquitously recognized as a bad prognostic marker in Brugada syndrome. However, young individuals with this disease may suffer from vaso-vagal instead of arrhythmic syncope. The prognostic significance of syncope in patients with Brugada syndrome is discussed in this review.
自1992年首次被描述为一种新的临床实体以来， Brugada综合征引起了医生和基础科学家的极大兴趣。2002年和2005年，两次共识会议分别确定了该综合征的诊断标准。目前，Brugada综合征的诊断基于临床事件（因恶性室性心律失常导致的晕厥和/或心源性猝死）和心电图特征（特征性ST段抬高形态）的结合。在过去几年中，在基因基础、导致典型心电图特征的细胞机制、室性心律失常易感性和危险分层等方面的认识取得了许多进展。植入式心脏复律除颤器仍然是预防心源性猝死一级和二级预防中唯一经证实有效的治疗选择。识别高危患者是临床决策的主要目标之一。晕厥在Brugada综合征中普遍被认为是一个不良预后指标。然而，患有这种疾病的年轻人可能会出现血管迷走性晕厥而非心律失常性晕厥。本文综述了晕厥在Brugada综合征患者中的预后意义。
