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Brugada-Syndrom

Definition, Diagnostik, Therapie, Prognose

Brugada syndrome

Definition, diagnostics, therapy, prognosis

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Zusammenfassung

Im Jahr 1992 wurde ein neues Syndrom („Brugada-Syndrom“, BS) beschrieben, das bei Herzgesunden mit synkopalen Ereignissen und/oder plötzlichen Todesfällen einhergeht und im Oberflächen-EKG einen Rechtsschenkelblock und ST-Strecken-Hebungen in V1–V3 zeigt. Die Erkrankung ist genetisch bedingt und mit einem autosomal-dominanten Erbgang verbunden. Mutationen im Bereich des SCN5A-Gens, das den kardialen Natriumstrom beeinflusst, sind nachgewiesen worden. Die Diagnose des BS ist bei manifester Form durch das 12-Kanal-Oberflächen-EKG möglich, wobei mittlerweile 3 EKG-Typen eines BS beschrieben sind. Das BS kann im EKG in einer manifesten, einer verborgenen oder einer intermittierenden Form vorliegen. Die Applikation von Ajmalin (1 mg/kg i.v.), Procainamid (10 mg/kg i.v.) oder Flecainid (2 mg/kg i.v.) kann zur Demaskierung eines BS oder deutlicheren Ausprägung von ST-Strecken-Hebungen in V1–V3 führen. Es ist unbestritten, dass Patienten mit Brugada-Syndrom durch lebensbedrohliche ventrikuläre Tachyarrhythmien und/oder einen plötzlichen Tod gefährdet sind. Antiarrhythmika wie Amiodaron oder β-Blocker sind hinsichtlich der Prävention eines plötzlichen Todes bei symptomatischen oder asymptomatischen Patienten mit Brugada-Syndrom nicht effektiv, sodass der implantierbare automatische Defibrillator zum jetzigen Zeitpunkt die einzig wirksame therapeutische Alternative ist. Möglicherweise wird die Gentherapie in der Zukunft zu einer Heilung von Patienten mit Brugada-Syndrom führen.

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 V1–V3. 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.

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  1. Medizinische Universitätsklinik II (Schwerpunkte Kardiologie und Angiologie), Ruhr-Universität Bochum, Hölkeskampring 40, 44625, Herne, Deutschland

    H.-J. Trappe

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Trappe, HJ. Brugada-Syndrom. Kardiologe 3, 309–318 (2009). https://doi.org/10.1007/s12181-009-0203-4

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