Short QT Syndrome - Review of Diagnosis and Treatment

Short QT syndrome (SQTS) is an inherited cardiac channelopathy characterised by an abnormally short QT interval and increased risk for atrial and ventricular arrhythmias. Diagnosis is based on the evaluation of symptoms (syncope or cardiac arrest), family history and electrocardiogram (ECG) findings...

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Bibliographic Details
Published in:Arrhythmia & electrophysiology review 2014-08, Vol.3 (2), p.76-79
Main Authors: Rudic, Boris, Schimpf, Rainer, Borggrefe, Martin
Format: Article
Language:English
Subjects:
Arrhythmia Mechanisms
Asymptomatic
Cardiac arrhythmia
Electrocardiography
Family medical history
Genes
Genotype & phenotype
Heart rate
Mortality
Mutation
Population
Potassium
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Description
Summary:Short QT syndrome (SQTS) is an inherited cardiac channelopathy characterised by an abnormally short QT interval and increased risk for atrial and ventricular arrhythmias. Diagnosis is based on the evaluation of symptoms (syncope or cardiac arrest), family history and electrocardiogram (ECG) findings. Mutations of cardiac ion channels responsible for the repolarisation orchestrate electrical heterogeneity during the action potential and provide substrate for triggering and maintaining of tachyarrhythmias. Due to the malignant natural history of SQTS, implantable cardioverter defibrillator (ICD) is the first-line therapy in affected patients. This review summarises current data and addresses the genetic basis and clinical features of SQTS.
ISSN:2050-3369
2050-3377
2050-3377
DOI:10.15420/aer.2014.3.2.76