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J wave syndromes: What's new?

Charles Antzelevitch1, Jose M Di Diego2

  • 1Distinguished Professor Emeritus and Executive Director, Cardiovascular Research, Lankenau Institute for Medical Research, Wynnewood, PA 19096, USA; Lankenau Institute for Medical Research, Wynnwoddm PA USA; Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia PA, USA.

Trends in Cardiovascular Medicine
|July 13, 2021
PubMed

Insights

J wave syndromes, including Brugada syndrome (BrS) and early repolarization syndrome (ERS), are inherited heart conditions causing sudden cardiac death. Research explores their mechanisms and a new whole-heart model of BrS.

Area of Science:

  • Cardiology
  • Molecular Biology
  • Genetics

Background:

  • Inherited ion channelopathies, specifically J wave syndromes like Brugada syndrome (BrS) and early repolarization syndrome (ERS), are linked to life-threatening ventricular arrhythmias in structurally normal hearts.
  • These conditions are a significant cause of sudden cardiac death (SCD), particularly in young adults.

Purpose of the Study:

  • To review the clinical features, molecular, ionic, cellular, and genetic mechanisms of J wave syndromes.
  • To present a novel whole-heart experimental model of BrS and discuss its implications.

Main Methods:

  • Literature review of clinical characteristics and underlying mechanisms.
  • Development and application of a whole-heart experimental model for Brugada syndrome.

Main Results:

  • The whole-heart model provides evidence supporting the repolarization hypothesis for BrS.
  • Novel findings reveal interactions between voltage-gated sodium and transient outward current channels.

Conclusions:

  • Understanding the interplay between ion channel function, trafficking, and gating is crucial for elucidating the genetics of cardiac and neuronal syndromes.
  • The developed model offers new insights into the pathophysiology of BrS.

Keywords:
Brugada syndromeCardiac arrhythmiasEarly repolarization syndromeInherited cardiac arrhythmia syndromesLangendorff-perfused heartsSudden cardiac death

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