Brugada Syndrome: from syncope towards cardioverter-defibrillator implantation

• Chapurnykh A.V.
• Zaklyazminskaya E.V.

Abstract

Brugada syndrome (BrS) is a genetically determined syndrome that occurs in individuals with a structurally normal heart, manifested by ST-segment elevation >2 mV in V₁–V₃ leads, syncope, and a high risk of sudden cardiac death. This syndrome is accompanied by a wide range of arhythmias, including supraventricular tachycardias, artio-ventricular conduction block, ventricular tachycardia, and ventricular fibrillation. Mutations in the SCN5A gene are the most known cause of this disease, and account for 15–30% of BrS cases. Here we present a genotype-positive BrS patient with a transient spontaneous Brugada pattern, type I, on resting ECG, a history of VT, unexplained recurrent syncopes, and a positive programmed electrical stimulation (PES) test. The paper also discusses the role of endocardial electrophysiological testing (EPS) and DNA diagnostics results in assessing the immediate and long-term risk of ventricular events in BrS patients.

Keywords:Brugada syndrome; SCD risk stratification; endocardial electrophysiological study; SCN5A; ICD

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