Brugada syndrome

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Brugada syndrome is a rare inherited cause of sudden cardiac death (SCD)

The characteristic ECG findings are:

  • right bundle branch block
  • persistent ST elevation in leads V1 to V3
  • normal QT intervals

Mutation of the cardiac sodium channel gene SCN5A has been identified in some clinical cases, but most of the reported cases have been adult male patients despite the condition's autosomal dominant inheritance.

  • the autonomic nervous system is considered to play an important role in modulation of this ECG abnormality
  • a study has found that subjects with the Brugada-type ECG have slender builds (2). The authors suggest that this may provide a clue to the basic mechanisms of the Brugada-type ECG with a focus on this association

Brugada syndrome is a diagnosis of exclusion: the differential diagnoses should be excluded first.

Presentation

  • often asymptomatic.Typically presents 3rd - 4th decade with syncope or SCD (3)

Click here for an example ECG and further information

Reference:

  1. Brugada P, Brugada J.Right bundle branch block, persistent ST elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. A multicenter report. J Am Coll Cardiol 1992;20:1391?1396
  2. Matsuo et al. Clinical Characteristics of Subjects with the Brugada-Type Electrocardiogram. Journal of Cardiovascular Electrophysiology 2004;15 (6): 653-657
  3. British Heart Foundation (2009).Factfile 5/09, Inherited cardiovascular disease.

Last reviewed 06/2019

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