ST段抬高、右束支阻滞与猝死:Brugada综合征。

E Asensio, B Alvarez, E Lozano, A Farías, R Brugada, P Brugada, J Brugada
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引用次数: 0

摘要

Brugada综合征是无结构性心脏病的年轻人猝死的主要原因之一。这是一种继发于3号染色体SCN5A基因突变的电性心脏病,具有显性常染色体传播模式。这种突变暗示了钠通道的功能障碍,增加了Ito,失去了心外膜动作电位第二阶段的穹顶。“要么全极化,要么全极化”的复极化模式随之而来,并导致第二阶段再入。这种再入是这些患者恶性室性心律失常的开始和延续的原因。临床表现为右束支阻滞,ST段由V1导联抬高至V3导联,并发猝死或晕厥。在一些患者中,必须用ajmaline或procainamide进行药理学试验以揭示心电图变化。目前,唯一有效的治疗方法是植入式心律转复除颤器(ICD)。这个装置有能力将死亡率从每年40%降低到十年后的0%。药物治疗无效。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[ST segment elevation, right bundle branch block and sudden death: Brugada's syndrome].

Brugada's syndrome is one of the main causes of sudden death in young adults without a structural heart disease. This is an electrical cardiac illness secondary to a mutation of SCN5A gene of chromosome 3 that has a dominant autosomic transmission pattern. This mutation implies the dysfunction of the sodium channel that increases the Ito, loosing the dome of the epicardiac action potential phase two. An "all or none" repolarization pattern ensues and gives rise to a phase two reentry. This kind of reentry is responsible for the initiation and perpetuation of malignant ventricular arrhythmias among these patients. The clinical characteristics of the syndrome are the right bundle branch block, ST segment elevation from V1 to V3 leads and sudden death or syncope. In some patients, a pharmacological test must be done with ajmaline or procainamide to unmask the electrocardiographic changes. At present, the only effective treatment is the implantable cardioverter defibrillator (ICD). This device has the capability to reduce mortality from 40% annually to 0% at ten years. Pharmacological treatment is not useful.

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