Síndrome de QT largo en pediatría.

The long QT syndrome can be congenital or acquired; if it is associate to congenital deafness is the Jervell and Lange-Nielsen syndrome and if there is not deafness is known as Romano-Ward syndrome. This report is a revision of the molecular mechanisms implicated and its correlation with the clinic, the epidemiological risk factors associated: congenital deafness, feminine sex, early age of presentation, antecedent of syncope, duration of the QTc greater to 500 ms and torsades des pointes. Mortality has been diminished to less of 10% by the used β blocking, the implantation of pacemaker or using an automatic cardioverter-defibrillator. Finally an management algorithm is presented. [ABSTRACT FROM AUTHOR]