Brugada症候群とその取扱い

In 1992, Brugada brothers reported 8 patients with aborted sudden death withoutorganic heart disease and exhibiting a characteristic ECG pattern of right bundle branch block(RBBB) and ST-segment elevation in right precordial leads (V1-V3). The syndrome is afamilial disease and most frequently diagnosed in middle-aged men of Asian origin. Thearrhythmic events such as polymorphic ventricular tachycardia (VT) or ventricular fibrillation(VF) are frequently generated during sleep. The Brugada syndrome has been linked tomutations in SCN5A, the gene encording for the -subunit of the sodium channel. Sodiumchannel blockers (class IA and IC) identify the risk of sudden death in patients with thesyndrome. Implantation of ICD is the only effective treatment of the VF for Brugadasyndrome. The Brugada-type ECG in annual health examinations for adult citizens is not avery rare condition in Japan. Although it is reported that the mortality of subjects with theBrugada-type ECG in a community-based population is low compared with the mortalityseen in a hospital-based study, there is also a report of the example of death in asymptomaticcases and the further study about a prognosis of Brugada syndrome is required.