title: Short QT Syndrome - Review of Diagnosis and Treatment.
abstract: 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.
authors.affiliation: Fellow in Cardiac Electrophysiology.
authors.forename: Boris
authors.initials: B
authors.lastname: Rudic
authors.affiliation: Associate Professor and Senior Electrophysiologist.
authors.forename: Rainer
authors.initials: R
authors.lastname: Schimpf
authors.affiliation: Director of the Department 1st Department of Medicine-Cardiology, University Medical Centre Mannheim, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Borggrefe
keywords: ECG; Sudden cardiac death; genetic; risk stratification; ventricular fibrillation
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