Lenegre病的临床诊治  

Lenegre's disease:an inherited conductive system disease

作　　者：董蕾[1] 

机构地区：[1]北京市第六医院心内科,北京100007

出　　处：《中国实用内科杂志》2013年第1期26-29,共4页Chinese Journal of Practical Internal Medicine

摘　　要：Lenegre病是引起先天性传导阻滞最常见的病因之一。它是一种SCN5A基因突变所致的常染色体显性遗传性疾病,男性多于女性,发病年龄较低(多数<40岁),有明显的家族聚集性。该病的本质是传导系统发生组织纤维变性,病变广泛,常累及传导系统的多个部位,最早、最多累及右束支,其次是左前分支,严重时则会发生高度或完全性房室传导阻滞。在单束支和双束支阻滞时,患者常无症状,不需治疗。而一旦发展到高度或三度房室阻滞,患者即可能出现明显的心悸、黑朦、晕厥或阿-斯综合征,需积极给予起搏器治疗,当患者因心率缓慢继发严重的室性心律失常时,需置入植入式心律转复除颤器(ICD)。Lenegre＇s disease is the most common cause of inherited conductive blockade disorder caused by SCN5A gene mutation leading to an autosomal dominant hereditary disease that is characterized of a higher incidence rate in males than in females,a lower age of onset （mostly 〈40 years） and a significant famihal aggregation. This has been pathophysiologi- cally linked to the conductive system fibrosis with extensive lesions that often involved multiple sites of the conductive sys- tem. The right bundle branch may be initially and mostly involved,followed by the left anterior fascicle and the highly or complete atrioventricular block found in severe cases. Patients with single or double bundle branch block frequently appear asymptomatic and do not require treatment, yet may experience considerable palpitation, amaurosis, syncope or Adams- Stokes syndrome in case of highly or grade-three atrioventricular block. This urgently indicated the administration of pace- maker therapy, and if warranted, the addition of implantable cardioverter defibrillator （ICD） when brachycardia developed.

关 键 词：LENEGRE病 传导阻滞 SCN5A基因 纤维变性 

分 类 号：R541.7[医药卫生—心血管疾病]