进行性心脏传导疾病

Progressive Cardiac Conduction Disease

进行性心脏传导疾病以心内传导系统的进行性退行性变导致房室或室内传导阻滞,病因不明,体表心电图上出现PR间期及QRS间期的延长和/或左或右束支传导阻滞,病情严重时可导致晕厥或猝死。早期发病者预后差。患者常有钠通道基因异常的遗传性基础,诊断主要依赖心电图学的随访,因为发病率不明,临床应予以注意。治疗主要依赖永久性起搏治疗,适应证较其他传导系统疾病宽,抗心律失常药物治疗应谨慎。

Progressive cardiac conduct!on disease is caused by the progressive degeneration and fibrosis of the conductive tissue in the heart which causes atrioventricular and/or intracardiac block. Eletrocardiogram manifestations include the prolongation of the PR and QRS intervals and bundle branch block. It may cause syncope or sudden cardiac death, especially in those young patients. Sodium channelopa- thies exist in some patients with an unknown incidence or prevalence. Cardiac pacemaker implantation is the predominant therapeutic manner with wider indications than patients with bradyeardias. Antiarrhythmie drugs should be used with caution in patients with progressive cardiac conduction disease.