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short QT syndrome

Etiology: - genetic, idiopathic Pathology: - absence of structural heart disease Genetics: - associated with defects in KCNH2 (type 1) - associated with defects in KCNQ1 (type 2) - associated with defects in KCNJ2 (type 3) Clinical manifestations: - atrial fibrillation - ventricular tachydardia [3] - syncope Special laboratory: - electrocardiogram (ECG) a) persistently & uniformly short QT interval (QTc < 350 msec) b) type 3 has a unique ECG phenotype characterized by asymmetrical T waves Complications: - ventricular fibrillation, sudden death Management: - implantable cadioverter defibrillator [3]

Related

long QT syndrome QT interval

General

genetic disease of the heart syndrome

Database Correlations

OMIM correlations

References

  1. OMIM :accession 609621
  2. OMIM :accession 609622
  3. Medical Knowledge Self Assessment Program (MKSAP) 16, 17 American College of Physicians, Philadelphia 2012, 2015