[Brugada syndrome].
Brugada syndrome is characterized by an ECG pattern of right bundle branch block and ST-segment elevation in right precordial leads, and sudden death caused by ventricular fibrillation(VF). The cellular basis for the syndrome is thought to be due to an outward shift in the ionic current active during phase 1 of the right ventricular epicardial action potential. Mutations of the cardiac sodium channel gene, SCN5A, have been identified as the genesis of the syndrome. This ECG pattern, which appears intermittently in most patients, is accentuated just before and after episodes of VF and is unmasked by class IA and IC antiarrhythmic agents. Development of VF is associated with an increase in vagal activity, and it occurs frequently during sleep. Implantable-cardioverter defibrillator is the effective therapy for prevention from sudden death.
Matsuo
Kiyotaka
K
Third Department of Internal Medicine, Nagasaki University School of Medicine.
Yano
Katsusuke
K
D002037:Bundle-Branch Block / Q000209:etiology / Q000628:therapy; D003645:Death, Sudden / Q000209:etiology / Q000517:prevention & control; D017147:Defibrillators, Implantable; D004562:Electrocardiography; D006801:Humans; D009154:Mutation; D062554:NAV1.5 Voltage-Gated Sodium Channel; D015222:Sodium Channels / Q000235:genetics; D013577:Syndrome; D014693:Ventricular Fibrillation / Q000628:therapy
D062554:NAV1.5 Voltage-Gated Sodium Channel; C568320:SCN5A protein, human; D015222:Sodium Channels