Short QT syndromes

Fiorenzo Gaita, Carla Giustetto, Andrea Mazzanti

Research output: Contribution to journalArticle

Abstract

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
Volume2
Issue number4
DOIs
Publication statusPublished - Dec 2010

Fingerprint

L-Type Calcium Channels
Mutation
Inborn Genetic Diseases
Implantable Defibrillators
Potassium Channels
Sudden Cardiac Death
Sudden Death
Atrial Fibrillation
Genes
Cardiac Arrhythmias
Incidence
Therapeutics
SHORT syndrome
hydroquinidine

Keywords

  • Hydroquinidine
  • ICD
  • Short QT syndrome
  • Sudden death

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

Short QT syndromes. / Gaita, Fiorenzo; Giustetto, Carla; Mazzanti, Andrea.

In: Cardiac Electrophysiology Clinics, Vol. 2, No. 4, 12.2010, p. 551-558.

Research output: Contribution to journalArticle

Gaita, Fiorenzo ; Giustetto, Carla ; Mazzanti, Andrea. / Short QT syndromes. In: Cardiac Electrophysiology Clinics. 2010 ; Vol. 2, No. 4. pp. 551-558.
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