Prolonged QT interval syndrome in pediatric practice: literature data and clinical case description

The article provides basic information about the syndrome of prolonged QT interval, which is a rare pathology of the cardiovascular system and can cause sudden death. The mutations in the genes responsible for dysfunction of ion channels are the key in the pathogenesis of this disease. Jerwell and Lange—Nielsen, Romano—Ward, Andersen—Tavil and Timothy syndromes are described as the main hereditary variants of this syndrome. A own case of the disease in a patient was given. The peculiarity of this case was on the acute, atypical onset of the disease, which started with convulsions and sudden cardiac death. The clinical picture of this case, features of a current are described, indicators of the basic methods of research are resulted. Information on the treatment of this clinical case at the local level and in the department of surgical treatment of arrhythmias, where the patient underwent implantation of endocardial bicameral cardioverter-defibrillator, іs presented. Data on further observation and treatment of the child at the place of residence are given. The research was carried out in accordance with the principles of the Helsinki declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: syndrome, QT interval, etiology, pathogenesis, clinical picture, diagnosis, examination, treatment.