关键词: Lenegre病, 传导阻滞, SCN5A基因, 纤维变性

Abstract:

Abstract：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 familial aggregation.This has been pathophysiologically linked to the conductive system fibrosis with extensive lesions that often involved multiple sites of the conductive system.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 pacemaker therapy，and if warranted，the addition of implantable cardioverter defibrillator (ICD) when brachycardia developed.

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Key words: Lenegre&rsquo, s disease, conduction block, SCN5A gene, cystic fibrosis