Abstract
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.
Publication types
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Research Support, U.S. Gov't, P.H.S.
MeSH terms
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Adolescent
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Alleles
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Cation Transport Proteins*
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Child, Preschool
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DNA-Binding Proteins*
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ERG1 Potassium Channel
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Electrophysiologic Techniques, Cardiac
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Ether-A-Go-Go Potassium Channels
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Female
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Humans
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Long QT Syndrome / genetics*
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Male
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Phenotype
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Potassium Channels / genetics*
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Potassium Channels, Voltage-Gated*
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Sodium Channels / genetics
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Tachycardia, Ventricular / genetics*
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Trans-Activators*
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Transcriptional Regulator ERG
Substances
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Cation Transport Proteins
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DNA-Binding Proteins
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ERG protein, human
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ERG1 Potassium Channel
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Ether-A-Go-Go Potassium Channels
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KCNH2 protein, human
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KCNH6 protein, human
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Potassium Channels
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Potassium Channels, Voltage-Gated
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Sodium Channels
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Trans-Activators
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Transcriptional Regulator ERG