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Table 1 Long QT syndrome (LQTS) subtypes, disease-associated genes and prevalence

From: Congenital long QT syndrome

LQTS subtypes

Gene

Prevalence (% of all

genotyped cases)

LQT1 and JLN1 (AR)

KCNQ1

>50

LQT2

KCNH2

35–40

LQT3

SCN5A

10–15

LQT4

ANK2

 

LQT5 (RWS) and JLN2

KCNE1

 

LQT6

KCNE2

 

LQT7 (Andersen-Tawil syndrome)*

KCNJ2

 

LQT8 (Timothy syndrome)

CACNA1c

 

LQT9

CAV3

 

LQT10

SCN4B

 
  1. Abbreviations: LQTS = Long QT syndrome; JLN = Jervell and Lange-Nielsen syndrome; RWS = Romano-Ward syndrome; AR = autosomal recessive
  2. * We consider the Andersen-Tawil syndrome to be a disorder different from LQTS (see ref. [78])