Resumen

ESCOBAR CERVANTES, Carlos et al. Síndrome de QT largo congénito: revisión de las diferentes variantes y tratamientos. Rev. costarric. cardiol [online]. 2005, vol.7, n.1, pp.23-29. ISSN 1409-4142.

Long-QT syndrome is a clinically and genetically heterogeneous syndrome characterized by lengthening of the QT interval and increased dispersion of the ventricular repolarization on the surface electrocardiogram and a propensity to malignant ventricular arrhythmias, torsade de pointes and ventricular fibrillation, which may lead to sudden cardiac death. Long-QT syndrome mostly affects adolescents and young adults with structurally and functionally normal hearts and is caused by aberrations in potassium and sodium ion channels. Different treatments have been used for long-QT syndrome. These treatments include correction of the underlying cause and the precipitating factors, antiadrenergic therapy (beta-adrenergic receptor blockers, left cervicothoracic sympathectomy), magnesium sulfate, isoproterenol, cardiac pacing, and implantable cardioverter defibrillator. In spite of these treatments the proportion of cardiac events is still high. Other potential treatments include sodium channel blockers, potassium channel activators, alpha-adrenergic receptor blockers, calcium channel blockers, atropine, and protein kinase inhibitors. The objective of this article is to go over the different variants of long-QT syndrome and the established therapies and to review the recent advances and potential future strategies in the treatment of this condition and torsade de pointes.

Palabras clave : Congenital long-QT syndrome; Torsade de pointes; Cardiac ion channels; Sudden cardiac death; Anti-arrhythmic drugs.