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Short QT syndrome type 1

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Description

Short QT syndrome 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).

OMIM

  • Mode of Inheritance

  • Autosomal dominant inheritance

VARIANTS

27

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Genes

External Links

  • OMIM

    609620

  • Orphanet
  • HPO
  • Medgen

    C1865020

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