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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])