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2012, Number 2

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Rev Cubana Invest Bioméd 2012; 31 (2)

Update on the congenital long QT syndrome

Zayas MR

Language: Spanish
References: 60
Page: 129-144
PDF size: 243.25 Kb.


ABSTRACT

The congenital long QT syndrome is characterized by a prolongation of the QT interval and T wave abnormalities on the ECG, associated to a predisposition for the appearance of malignant ventricular arrhythmias (torsade de pointes), syncope, ventricular fibrillation and sudden cardiac death (SCD). Training in taking the measurements, penetrance and mutation variability, overlaps, mixed forms and overlap syndrome may all hamper diagnostic and therapeutic management. The present review intends to update essential aspects related to the diagnosis, genetic bases, physiopathological and molecular mechanisms, and therapeutic management of this complex channelopathy.


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