Kubo Kazuhiro, Nishikawa Koichi, Goto Fumio
Department of Anesthesiology, Gunma University, Graduate School of Medicine, Maebashi 371-8511, Japan.
Masui. 2005 Nov;54(11):1247-52.
In 1992, Brugada et al. first reported eight cases of ventricular fibrillation, in which ST-segment abnormalities in leads V1 through V3 along with T-wave inversion, and complete or incomplete right bundle branch block were observed on the standard 12-leads ECG. Since then, this syndrome has been widely recognized as one of important diseases that can produce sudden death in middle aged healthy males. The ECG morphology of Brugada syndrome is believed to be caused by either an accentuation of the notch in the early phase of the action potential or loss of the action potential dome in the epicardium. Mechanisms of ventricular fibrillation in this syndrome are still unclear, but thought to be phase II re-entry caused by dispersion of the action potentials. It has been shown that mutations of the human cardiac Na+ channel gene (SCN5A) underlie multiple cardiac diseases including Brugada syndrome. In fact, single amino acid substitution within the SCN5A coding region can evoke a cardiac rhythm behavior. In this review, we will focus on recent progress of basic and clinical research of Brugada syndrome and perioperative management of this syndrome.
1992年，布鲁加达等人首次报告了8例室颤病例，在标准12导联心电图上观察到V1至V3导联ST段异常伴T波倒置，以及完全或不完全性右束支传导阻滞。从那时起，该综合征已被广泛认为是可导致中年健康男性猝死的重要疾病之一。布鲁加达综合征的心电图形态被认为是由动作电位早期切迹的加重或心外膜动作电位圆顶的丧失引起的。该综合征室颤的机制仍不清楚，但被认为是由动作电位离散导致的II期折返。研究表明，人类心脏钠离子通道基因（SCN5A）突变是包括布鲁加达综合征在内的多种心脏疾病的基础。事实上，SCN5A编码区内的单个氨基酸替换可引发心律失常行为。在这篇综述中，我们将重点关注布鲁加达综合征基础和临床研究的最新进展以及该综合征的围手术期管理。
