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
• syncope

Diagnostic procedures

• electrocardiogram (ECG)
  • persistently & uniformly short QT interval (QTc < 350 msec)
  • type 3 has a unique ECG phenotype characterized by asymmetrical T waves

Complications

• ventricular fibrillation, sudden death

Management

• implantable cadioverter defibrillator

More general terms

• genetic disease of the heart
• syndrome

Additional terms

• long QT syndrome
• QT interval

Database

• OMIM: https://mirror.omim.org/entry/609621
• OMIM: https://mirror.omim.org/entry/609622