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J-wave syndromes: Brugada and early repolarization syndromes.

ABSTRACT: A prominent J wave is encountered in a number of life-threatening cardiac arrhythmia syndromes, including the Brugada syndrome and early repolarization syndromes. Brugada syndrome and early repolarization syndromes differ with respect to the magnitude and lead location of abnormal J waves and are thought to represent a continuous spectrum of phenotypic expression termed J-wave syndromes. Despite two decades of intensive research, risk stratification and the approach to therapy of these 2 inherited cardiac arrhythmia syndromes are still undergoing rapid evolution. Our objective in this review is to provide an integrated synopsis of the clinical characteristics, risk stratifiers, and molecular, ionic, cellular, and genetic mechanisms underlying these 2 fascinating syndromes that have captured the interest and attention of the cardiology community in recent years.

SUBMITTER: Antzelevitch C

PROVIDER: S-EPMC4737709 | biostudies-literature | 2015 Aug

REPOSITORIES: biostudies-literature

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J-wave syndromes: Brugada and early repolarization syndromes.

Antzelevitch Charles C Yan Gan-Xin GX

Heart rhythm 20150411 8

A prominent J wave is encountered in a number of life-threatening cardiac arrhythmia syndromes, including the Brugada syndrome and early repolarization syndromes. Brugada syndrome and early repolarization syndromes differ with respect to the magnitude and lead location of abnormal J waves and are thought to represent a continuous spectrum of phenotypic expression termed J-wave syndromes. Despite two decades of intensive research, risk stratification and the approach to therapy of these 2 inherit ...[more]

PMID: 25869754

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Distinct Features of Probands With Early Repolarization and Brugada Syndromes Carrying SCN5A Pathogenic Variants.

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Molecular genetic and functional association of Brugada and early repolarization syndromes with S422L missense mutation in KCNJ8.

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ABCC9 is a novel Brugada and early repolarization syndrome susceptibility gene.

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