Short QT syndrome

Also known as: SQTS

Rare channelopathy with short QT interval, atrial and ventricular arrhythmias and sudden death.

Medical specialty: Cardiology
Subspecialty: Cardiology / Cardiac electrophysiology
Sex & age distribution: Male: affected, more common; Female: affected; Children: yes, including infants
Gene(s) involved: CACNA1C, CACNA2D1, CACNB2, KCNH2, KCNJ2, KCNQ1, SLC22A5, SLC4A3

Etiology & mechanism

Gain of function of potassium currents or loss of function of calcium channel currents shortens repolarization.

Gene(s) involved (8)

CACNA1CCACNA2D1CACNB2KCNH2KCNJ2KCNQ1SLC22A5SLC4A3

Symptoms & signs (7)

atrial fibrillationdizzinesspalpitationsshort qt intervalsudden cardiac deathsyncopeventricular fibrillation

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