[The Brugada Syndrome as a Cause of Sudden Death. Diagnostics and Clinical Manifestations in Children].

The Brugada syndrome is a hereditary potentially arrhythmogenic disease related to the category of channelopathies. It is manifested as syncopal states and sudden death in young people in the absence of structural cardiac disease. The basis of the disease is genetically determined abnormality of function of ionic channels of cardiomyocytes (sodium, potassium, calcium) phenotypically manifesting as sustained or transitory segment-ST elevation and high risk of polymorphic ventricular tachycardia, fibrillation, and sudden death. The only proven method of prevention of fatal arrhythmia and sudden death is cardioverter defibrillator implantation.