Brugada-Syndrom

Abstract

In 1992, a new syndrome (Brugada syndrome, BS) was described consisting of syncopal episodes and/or sudden death in patients with a structurally normal heart and an electrocardiogram characteristic of right bundle branch block with ST segment elevation in leads V 1 -V 3 . The disease is genetically determined with an autosomal dominant pattern of transmission, and different mutations that affect the structure and function of the cardiac sodium channel gene SCN5A have been identified. BS can be diagnosed by ECG if the manifest form is present; at the present time, three different ECG types of BS are known. In patients with transient forms, the ECG can be modulated by the administration of antiarrhythmic drugs. Application of ajmaline (1 mg/kg i.v.), procainamide (10 mg/kg i.v.) or flecainide (2 mg/kg i.v.) accentuate ST segment elevation or are capable of unmasking concealed or intermittent forms of the disease. BS has been described as being associated with life-threatening ventricular tachyarrhythmias and a high incidence of sudden death. Antiarrhythmic drugs such as amiodarone or beta-blocking agents do not prevent sudden death in symptomatic or asymptomatic individuals. Implantation of an automatic cardioverter defibrillator is the only form of treatment currently proven to be effective. Gene therapy may offer a cure in the future.