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Short QT syndrome type 1

Summary

Short QT syndrome (SQT) is a cardiac channelopathy associated with a predisposition to atrial fibrillation and sudden cardiac death. Patients have a structurally normal heart, but electrocardiography (ECG) exhibits abbreviated QTc (Bazett's corrected QT) intervals of less than 360 ms (summary by Moreno et al., 2015). Genetic Heterogeneity of Short QT Syndrome Short QT syndrome-2 (SQT2; 609621) is caused by mutation in the KCNQ1 gene (607542). SQT3 (609622) is caused by mutation in the KCNJ2 gene (600681). SQT7 (620231) is caused by mutation in the SLC4A3 gene (106195). [from OMIM]

Available tests

76 tests are in the database for this condition.

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Clinical tests (76 available)

Molecular Genetics Tests

Genes See tests for all associated and related genes

  • Also known as: ERG-1, ERG1, H-ERG, HERG, HERG1, Kv11.1, LQT2, SQT1, KCNH2
    Summary: potassium voltage-gated channel subfamily H member 2

Clinical features

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Imported from Human Phenotype Ontology (HPO)

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Reviews

Suggested reading

  • HRS/EHRA/APHRS, 2013
    Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes.

Clinical resources

Molecular resources

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