Modern tactics in management of young patients with long Qt syndrome: early diagnosis, implantation of cardioverter-defibrillator, and monitoring of sudden cardiac death risk markers

The study of primary electrical diseases of the heart in children and young people is of high demand because these diseases are the main cause of malignant ventricular tachycardias and sudden cardiac death (SCD). This group of diseases includes long QT syndrome with estimated prevalence rate of 1 in 2500 to 3000 in new-borns according to recent data. Clinical manifestation of the disease and efficacy of treatment depend on particular molecular-genetic variant of the syndrome. Sudden cardiac death risk stratification in patients with long QT syndrome is based on clinical, electrocardiography, and molecular-genetic factors and markers. Common approach to treatment of long QT syndrome consists in administration of beta-adrenoblockers. Efficacious method of SCD prevention in patients with long QT syndrome is implantation of cardioverter-defibrillator.