Table 5.

Long QT Syndrome: Mode of Inheritance

Gene 1LQTS PhenotypeMode of
Inheritance
CACNA1C LQTS w/o extracardiac features (See CACNA1C-Related Disorders.)AD
Timothy syndrome (See CACNA1C-Related Disorders.)
CALM1 LQTS w/o extracardiac features
CALM2
CALM3
CAV3 LQTS w/ or w/o extracardiac features
KCNE1 LQTS w/o extracardiac features
Susceptibility to acquired LQTS
Jervell and Lange-Nielsen syndrome AR
KCNE2 Susceptibility to LQTSAD
KCNH2 LQTS w/o extracardiac features
KCNJ2 Andersen-Tawil syndrome
KCNQ1 LQTS w/o extracardiac features
Jervell and Lange-Nielsen syndrome AR
SCN5A (GoF variants)LQTS w/o extracardiac featuresAD
TRDN CPVT-LQTS overlap syndromeAR

AD = autosomal dominant; AR = autosomal recessive; CPVT = catecholaminergic polymorphic ventricular tachycardia; GoF = gain-of-function; LQTS = long QT syndrome

1.

Genes are ordered alphabetically

From: Long QT Syndrome Overview

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