Revista portuguesa de cardiologia : orgão oficial da Sociedade Portuguesa de Cardiologia = Portuguese journal of cardiology : an official journal of the Portuguese Society of Cardiology
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Congenital long QT syndrome (LQTS) is associated to an increased risk of ventricular arrhythmia, syncope and sudden cardiac death (SD). Four disease genes have been identified and different mutations described in each gene. This locus heterogenicity appears to have important functional and prognostic implications. ⋯ Other therapeutic options include left cervicothoracic sympathectomy, pacemakers, and the implantable cardioverter defibrillator. Risk stratification and efficacy of the subsequent treatment has significantly changed the clinical outcome of patients with LQTS. Recent molecular biology studies and data analysis from the International LQTS Registry may contribute to the definition of the best strategy for the future.