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Cardiology · Cardiology / Cardiac electrophysiology

Long QT syndrome

also: LQTS; Romano–Ward syndrome (autosomal dominant form)
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Inherited channelopathy with prolonged ventricular repolarization (long QT interval) predisposing to torsades de pointes.

Specialty
Cardiology
Subspecialty
Cardiology / Cardiac electrophysiology
Sex & age distribution
Male: affected, higher cardiac event risk in childhood; Female: affected, higher risk from adolescence and postpartum; Children: yes, events often in childhood or adolescence
Gene(s) involved
AKAP9, ANK2, CACNA1C, CALM1, CALM2, CALM3, CAV3, KCNE1, KCNE2, KCNE3, KCNH2, KCNJ2, KCNJ5, KCNQ1, SCN4B, SCN5A, SNTA1, TRDN
Alternative names & eponyms
LQTS; Romano–Ward syndrome (autosomal dominant form)

Etiology & mechanism

Loss of function of repolarizing potassium currents or gain of function of depolarizing sodium or calcium currents prolongs the action potential.

Gene(s) involved (18)

AKAP9 ANK2 CACNA1C CALM1 CALM2 CALM3 CAV3 KCNE1 KCNE2 KCNE3 KCNH2 KCNJ2 KCNJ5 KCNQ1 SCN4B SCN5A SNTA1 TRDN

Symptoms & signs (10)

In plain terms, someone with Long QT syndrome may experience the following — click any to explore related syndromes:

asymptomatic carriers events during sleep or rest (lqt3) events with exercise or swimming (lqt1) events with sudden noise (lqt2) palpitations prolonged qt interval seizure-like episodes sudden cardiac death syncope torsades de pointes

Same gene(s)

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Overlapping symptoms

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