Brugada syndrome: a review of the literature

Abstract

Brugada syndrome is an example of a channelopathy caused by an alteration in the transmembrane ion currents that together constitute the cardiac action potential. Approximately 20% of the cases of Brugada syndrome have been shown to be associated with mutations in the gene coding for the sodium ion channel in the cell membranes of the muscle cells of the heart. Patients with Brugada syndrome are prone to develop ventricular tachyarrhythmias that may lead to syncope, cardiac arrest or sudden cardiac death. Many clinical situations have been reported to unmask or exacerbate the electrocardiography (ECG) pattern of Brugada syndrome. Genetic testing for Brugada syndrome is clinically available. Here we report two cases of Brugada syndrome followed by a comprehensive review of the literature.

Keywords: Brugada syndrome; atrial fibrillation; channelopathy; sudden cardiac death; syncope.

Fig 1.

(a) The 12-lead ECG on admission showing atrial fibrillation with fast ventricular rate. (b) The rhythm strip from the monitor showing ventricular fibrillation post flecainide loading. (c) The 12-lead ECG after the shock showing ST right bundle branch block with ST -elevation in V1–V2, suggestive of Brugada syndrome type 1. (d) Repeat 12-lead ECG 3 hours later showing no feature suggestive of Brugada syndrome. ECG = electrocardiography.

Fig 2.

The 12-lead ECG showing Brugada ECG pattern. ECG = electrocardiography.

Fig 3.

ECG patterns of -Brugada syndrome in leads V1–V2. Reproduced with permission from Bayés de Luna et al (2012).25 ECG = electrocardiography.

Fig 4.

Consensus recommendations for implantable cardiac defibrillators (ICDs) in patients diagnosed with Brugada syndrome. Reproduced with permission from Priori et al (2013).27 ECG = electrocardiography; EP = electrophysiology; ICD = implantable cardiac defibrillator; SCD = sudden cardiac death; VT = ventricular tachycardia.