Miklashevich I M, Shkolnikova M A
Research Institute of Clinical Pediatrics named after Acad. Yu.E. Veltischeva Pirogov Russian National Research Medical University,Moscow, Russia.
Kardiologiia. 2016 Oct;56(10):63-71. doi: 10.18565/cardio.2016.10.63-71.
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.
布加综合征是一种遗传性潜在致心律失常疾病，属于离子通道病范畴。其表现为无结构性心脏病的年轻人出现晕厥状态和猝死。该病的基础是心肌细胞离子通道（钠、钾、钙）功能的基因决定异常，表型上表现为持续性或短暂性ST段抬高以及多形性室性心动过速、颤动和猝死的高风险。预防致命性心律失常和猝死的唯一经证实的方法是植入心脏复律除颤器。
