СИНДРОМ БРУГАДА В КЛИНИЧЕСКОЙ ПРАКТИКЕ

Abstract

Brugada syndrome is a congenital channelopathy with an autosomal dominant type of inheritance and low penetrance, characterized by the presence of a typical clinical and electrocardiographic symptom complex, including a special form of blockade of the right bundle branch block with ST segment elevation in one or more right chest leads on the electrocardiogram, the absence of organic pathology of the heart and various life-threatening ventricular arrhythmias, which can lead to the development of sudden cardiac death. The article defines the concept of "Brugada's syndrome", the geography and frequency of occurrence of the disease. It is indicated that more than 450 pathogenic variants in 24 genes are involved in the development of Brugada syndrome. The article presents two main hypotheses of pathogenesis, describes the electrocardiographic picture, three types of ECG changes, clinical manifestations and classification of the Brugada syndrome. Listed diseases with which it is necessary to carry out differential diagnosis of Brugada syndrome. Various aspects of the treatment of Brugada syndrome are presented: non-drug methods, pharmacotherapy, installation of an implantable automatic cardioverter-defibrillator, epicardial catheter ablation of abnormal areas of the anterior part of the outflow tract of the right ventricle.