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. 2018 Aug;7(3):199–209. doi: 10.15420/aer.2018.29.2

Table 5: Genes Associated with Congenital Long QT Syndrome.

Chromosome Gene Ion Channel
LQT1 11 KCNQ1, KvLQT1 ↓IKs 30–35 %
LQT2 7 KCNH2, HERG ↓IKr 20–25 %
LQT3 3 SCN5A, Nav1.5 ↑Late INa 5–10 %
LQT4 4 Ankyrin-B, ANK2 ↑Cai, ↑Late INa ? 1–2 %
LQT5 21 KCNE1, MinK ↓IKs 1 %
LQT6 21 KCNE2, MiRP1 ↓IKr Rare
LQT7 * 17 KCNJ2, Kir 2.1 ↓IK1 Rare
LQT8 † 6 CACNA1C, Cav1.2 ↑ICa Rare
LQT9 3 CAV3, Caveolin-3 ↑Late INa Rare
LQT10 11 SCN4B, NavB4 ↑Late INa Rare
LQT11 7 AKAP9, Yatiao ↓IKs Rare
LQT12 20 SNTA1, a1 Syntrophin ↑Late INa Rare
LQT13 11 KCNJ5, Kir 3.4 ↓IK-ACh Rare
LQT14 14 CALM1, Calmodulin ↑ICa, ↑Late INa Rare
LQT15 2 CALM2, Calmodulin ↑ICa, ↑Late INa Rare
LQT16 19 CALM3, Calmodulin ↑ICa, ↑Late INa Rare
LQT17 19 TRPM4, Transient receptor potential cation channel ↓Inon-selctive cation channel Rare

*Andersen-Tawill Syndrome, † Timothy Syndrome. LQT = Long QT. Source: Modified from Obeyesekere, et al., 2015.[155]