Bidirectional ventricular tachycardia and channelopathy.
Based on similarity of electrocardiographic features, bidirectional ventricular tachycardia has been considered a variant of long QT syndrome. Genes causing long QT syndrome were used as candidate genes in 4 patients with bidirectional ventricular tachycardia. In 2 patients, we identified a common low penetrance HERG allele (R1047L) with an intermediate biophysical phenotype.
Laohakunakorn
Preecha
P
Bumgrad Hospital, Bangkok, Thailand.
Benson
D Woodrow
DW
Yang
Ping
P
Yang
Tao
T
Roden
Dan M
DM
Kugler
John D
JD
D000293:Adolescent; D000483:Alleles; D027682:Cation Transport Proteins; D002675:Child, Preschool; D004268:DNA-Binding Proteins; D000072237:ERG1 Potassium Channel; D022062:Electrophysiologic Techniques, Cardiac; D051638:Ether-A-Go-Go Potassium Channels; D005260:Female; D006801:Humans; D008133:Long QT Syndrome / Q000235:genetics*; D008297:Male; D010641:Phenotype; D015221:Potassium Channels / Q000235:genetics*; D024642:Potassium Channels, Voltage-Gated; D015222:Sodium Channels / Q000235:genetics; D017180:Tachycardia, Ventricular / Q000235:genetics*; D015534:Trans-Activators; D000071230:Transcriptional Regulator ERG
D027682:Cation Transport Proteins; D004268:DNA-Binding Proteins; C072312:ERG protein, human; D000072237:ERG1 Potassium Channel; D051638:Ether-A-Go-Go Potassium Channels; C000606913:KCNH2 protein, human; C092509:KCNH6 protein, human; D015221:Potassium Channels; D024642:Potassium Channels, Voltage-Gated; D015222:Sodium Channels; D015534:Trans-Activators; D000071230:Transcriptional Regulator ERG