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Brugada syndrome and reduced right ventricular outflow tract conduction reserve: a final common pathway?

Journal

EUROPEAN HEART JOURNAL
Volume 42, Issue 11, Pages -

Publisher

OXFORD UNIV PRESS
DOI: 10.1093/eurheartj/ehaa1051

Keywords

Brugada syndrome; Genetics; Depolarization; Right ventricular outflow tract

Funding

  1. Robert Lancaster Memorial Fund
  2. Netherlands CardioVascular Research Initiative
  3. Dutch Heart Foundation
  4. Dutch Federation of University Medical Centres
  5. Netherlands Organisation for Health Research and Development
  6. Royal Netherlands Academy of Sciences (PREDICT2)

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Brugada syndrome is a primary electrical disorder associated with an increased risk of sudden cardiac death, with controversy over whether it is caused by cardiomyopathy or channelopathy. The final common pathway for BrS may be viewed as a disease of reduced RVOT conduction reserve.
Brugada syndrome (BrS) was first described as a primary electrical disorder predisposing to the risk of sudden cardiac death and characterized by right precordial lead ST elevation. Early description of right ventricular structural abnormalities and of right ventricular outflow tract (RVOT) conduction delay in BrS patients set the stage for the current controversy over the pathophysiology underlying the syndrome: channelopathy or cardiomyopathy; repolarization or depolarization. This review examines the current understanding of the BrS substrate, its genetic and non-genetic basis, theories of pathophysiotogy, and the clinical implications thereof. We propose that the final common pathway for BrS could be viewed as a disease of 'reduced RVOT conduction reserve'. [GRAPHICS] .

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