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Familial short QT syndrome

Disease definition

Familial short QT syndrome is a newly described cardiologic entity that associates a short QT interval (QT and QTc 300 ms) on the surface electrocardiogram (ECG) with a high risk of syncope or sudden death due to malignant ventricular arrhythmia.

ORPHA:51083

  • Synonym(s):
    • SQTS
  • Prevalence: Unknown
  • Inheritance: Autosomal dominant 
  • Age of onset: All ages
  • ICD-10: I49.8
  • OMIM: 609620  609621  609622
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -

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