[Perioperative management of patients with Brugada syndrome].

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.