REVIEW – January 2013

Congenital long QT syndrome

Authors: Sander Pajusalu, Riina Žordania, Tiiu Jalas, Rain Paju, Katrin Õunap, Tiia Reimand

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Congenital long QT syndrome (LQTS) is a cardiac electrophysiologic disorder with a genetic aetiology, characterised by the prolongation of the QTc interval and T wave abnormalities on the ECG and paroxysms of the ventricular tachycardia torsades de pointes, which might lead to syncope. In some cases there occurs ventricular fibrillation and therefore LQTS is associated with sudden death which can be the first manifestation of the disorder.

LQTS is divided into 13 subtypes according to the genes where the diseasecausing mutation is located. Diagnosis of LQTS should be based on clinical findings (ECG), family history, and molecular diagnostic tests if indicated.

The aim of treatment in LQTS patients is to prevent syncope and cardiac arrest. The treatment options are life-style modifications, administration of beta blockers and implantation of an implantable cardioverterdefibrillator.