Abstract：
Congenital complete heart block (CCHB) defines atrioventricular conduction abnormalities diagnosed in utero or within the first 27 days of life. Maternal autoimmune disease and congenital heart defects are most commonly responsible. Recent genetic discoveries have highlighted our understanding of the underlying mechanism. Hydroxychloroquine shows promise in preventing autoimmune CCHB. Patients may develop symptomatic bradycardia and cardiomyopathy. The presence of these and other specific findings warrants placement of a permanent pacemaker to relieve symptoms and prevent catastrophic events. The mechanisms, natural history, evaluation, and treatment of patients with or at risk for CCHB are reviewed.
摘要：
先天性完全性心脏传导阻滞（CCHB）定义了在子宫内或生命的前27天内诊断出的房室传导异常。母亲自身免疫性疾病和先天性心脏缺陷是最常见的原因。最近的遗传发现强调了我们对潜在机制的理解。羟氯喹在预防自身免疫性CCHB方面显示出希望。患者可能出现有症状的心动过缓和心肌病。这些和其他具体发现的存在保证放置永久性起搏器以缓解症状并防止灾难性事件。机制，自然史,评估，并对患有或有风险的患者的治疗进行审查。
