Short QT syndromes

Fiorenzo Gaita, Carla Giustetto, Andrea Mazzanti

Research output: Contribution to journalArticle


The short QT syndrome is a recently described genetic arrhythmogenic disorder, characterized by abnormally short QT intervals and a high incidence of sudden death and atrial fibrillation. Clinical manifestations may also be present in infants; a family history of cardiac sudden death is often present. Gain-of-function mutations in 3 genes encoding potassium channels and loss-of-function mutations in 2 genes encoding the cardiac L-type calcium channel have been identified. Today, the first choice therapy is implantable cardioverter-defibrillator implantation; however, pharmacologic treatment with hydroquinidine, which prolongs QT and reduces the inducibility of ventricular arrhythmias, may be proposed for children and probably for elderly asymptomatic patients.

Original languageEnglish
Pages (from-to)551-558
Number of pages8
JournalCardiac Electrophysiology Clinics
Issue number4
Publication statusPublished - Dec 2010



  • Hydroquinidine
  • ICD
  • Short QT syndrome
  • Sudden death

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

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