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Clinical Cardiogenetics pp 165–187Cite as

The Brugada Syndrome

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Abstract

The syndrome of right bundle branch block, persistent ST-segment elevation, and sudden cardiac death, today better known as the Brugada syndrome, was described in 1992 as a new clinical entity characterized by a typical electrocardiographic pattern and a susceptibility to develop polymorphic ventricular arrhythmias in the absence of structural heart disease.1 The description of the first eight patients was followed by other case reports2,3 and subsequently numerous works appeared either focusing on clinical characteristics of larger populations of patients4–7 or defining the genetic, molecular, and cellular aspects of the disease.8–10 In recent years, major advances in clinical and mechanistic knowledge have provided very valuable information about the disease, but remaining questions still propel today a large research activity on the subject. This chapter reviews the current knowledge on clinical, genetic, and molecular features of the Brugada syndrome, and provides updated information supplied by recent clinical and basic studies.

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Authors and Affiliations

  1. Electrophysiology Research Program, Research Center, Montreal Heart Institute, 5000 Rue Belanger, Montreal, QC, H1T 1C8, Canada

    Begoña Benito

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  1. Begoña Benito
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  2. Josep Brugada
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  3. Ramon Brugada
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  4. Pedro Brugada
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Correspondence to Begoña Benito .

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  1. University Medical Centre Utrecht, Heidelberglaan 100, 3584 CX, Utrecht, 3508 GA, Netherlands

    H.F. Baars

  2. University Medical Centre Utrecht, Heidelberglaan 100, 3584 CX, Utrecht, 3508 GA, Netherlands

    P.A.F.M. Doevendans

  3. University Medical Centre Utrecht, Heidelberglaan 100, 3584 CX, Utrecht, 3508 GA, Netherlands

    J.J. van der Smagt

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Benito, B., Brugada, J., Brugada, R., Brugada, P. (2011). The Brugada Syndrome. In: Baars, H., Doevendans, P., van der Smagt, J. (eds) Clinical Cardiogenetics. Springer, London. https://doi.org/10.1007/978-1-84996-471-5_10

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