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右束支传导阻滞、V_1~V_3导联ST段抬高和猝死综合征最早于1992年发现。其特点如下:(1)特征性的ECG表现,即右束支传导阻滞及V_1~V_3导联ST段抬高;(2)猝死或晕厥为快速的多形性室性心律失常所致;(3)心内结构正常;(4)猝死发作前无任何临床及ECG先兆。遗传资料已证实,该综合征是一遗传性疾病,呈常染色体显性遗传。其发病机制与心脏钠通道的病变有关。目前唯一有效的预防治疗方法是采用植入性心脏复律除颤器。
Right bundle branch block, V_1 ~ V_3 leads ST segment elevation and sudden death syndrome was first discovered in 1992. Its characteristics are as follows: (1) characteristic ECG manifestations, right bundle branch block and V_1 ~ V_3 lead ST segment elevation; (2) sudden death or syncope due to rapid pleomorphic ventricular arrhythmias; (3) intracardiac structure is normal; (4) no clinical and ECG aura before sudden onset of death. Genetic information has confirmed that the syndrome is a genetic disease, was autosomal dominant. Its pathogenesis and pathological changes of sodium channel. Currently the only effective preventive treatment is the use of implantable cardioverter-defibrillator.