Table 1.
Rhythm | Inheritance | Locus | Ion channel | Gene | ||
---|---|---|---|---|---|---|
Long QT syndrome | (RW) | TdP | AD | |||
LQT1 | 11p15 | IKs | KCNQ1, KvLQT1 | |||
LQT2 | 7q35 | IKr | KCNH2, HERG | |||
LQT3 | 3p21 | INa | SCN5A, Nav1.5 | |||
LQT4 | 4q25 | ANKB, ANK2 | ||||
LQT5 | 21q22 | IKs | KCNE1, minK | |||
LQT6 | 21q22 | IKr | KCNE2, MiRP1 | |||
LQT7 | (Andersen-Tawil syndrome) | 17q23 | IK1 | KCNJ2, Kir 2.1 | ||
LQT8 | (Timothy syndrome) | 6q8A | ICa | CACNA1C, Cav1.2 | ||
LQT9 | 3p25 | INa | CAV3, Caveolin-3 | |||
LQT10 | 11q23.3 | INa | SCN4B. Navb4 | |||
LQT syndrome (JLN) | TdP | AR | 11p15 | IKs | KCNQ1, KvLQT1 | |
21q22 | IKs | KCNE1, minK | ||||
Brugada syndrome | BrS1 | PVT | AD | 3p21 | INa | SCN5A, Nav1.5 |
BrS2 | PVT | AD | 3p24 | INa | GPD1L | |
BrS3 | PVT | AD | 12p13.3 | ICa | CACNA1C, CaV1.2 | |
BrS4 | PVT | AD | 10p12.33 | ICa | CACNB2β, Cavβ2b |
AD — autosomal dominant; AR — autosomal recessive; JLN — Jervell and Lange-Nielsen; LQT — long QT; RW — Romano-Ward; TdP — torsade de pointes; VF — ventricular fibrillation; VT — ventricular tachycardia; PVT — polymorphic VT