Early repolarization syndrome

Also known as: idiopathic ventricular fibrillation with early repolarization; J-wave syndrome (with Brugada syndrome)

J-point elevation on ECG associated with idiopathic ventricular fibrillation in a minority of carriers.

Medical specialty: Cardiology
Subspecialty: Cardiology / Cardiac electrophysiology
Sex & age distribution: Male: affected, more common; Female: affected; Children: rare, young adults
Gene(s) involved: ABCC9, CACNA1C, CACNA2D1, CACNB2, DPP6, KCNJ8, SCN10A, SCN5A

Etiology & mechanism

Transmural voltage gradient from transient outward current heterogeneity; genetic and acquired contributors.

Gene(s) involved (8)

ABCC9CACNA1CCACNA2D1CACNB2DPP6KCNJ8SCN10ASCN5A

Symptoms & signs (5)

j-point elevationpalpitationssudden cardiac deathsyncopeventricular fibrillation

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