遗传性房室传导缺陷是儿童完全性心脏传导阻滞的少见原因。作者介绍父子二人均患此病并因完全性传导阻滞需安置永久性起搏器。病例报告 10岁男孩,入院前3个月诉述每天头痛和头晕,近一周加重。入院当日清晨有一次全身强直一阵挛性惊厥,送入当地 Hereditary atrioventricular conduction defects are rare causes of complete heart block in children. The author describes both father and son suffer from the disease and due to complete conduction block need to be placed permanent pacemaker. Case report 10-year-old boy, complained of headache and dizziness every day for 3 months before admission, exacerbating in the past week. On the morning of admission, there was an episode of tetanus and convulsions