自身抗体相关性先天性心脏传导阻滞（ACHB）是抗SSA/Ro抗体和抗SSB/La抗体阳性母亲在妊娠过程中通过胎盘将抗体输送给胎儿，干扰胎儿心脏传导所致的一类疾病，大多以Ⅲ度房室传导阻滞为最初表现。该病在抗SSA/Ro抗体阳性的患者初次妊娠的发生率为2%，而再次妊娠的发生率为12%～20%。房室传导是心脏电生理与机械活动的基础，房室传导阻滞是指冲动从心房传导至心室的过程中出现异常延迟或不能抵达心室，严重程度取决于心室率的快慢。ACHB的发病率低，而一旦发展为Ⅲ度房室传导阻滞则预后不佳。综述该病的发病机制、危险因素、诊断方法、产前干预及预后等，为该病早期诊疗及改善预后等方面提供临床依据。%The autoantibody-related congenital heart block (ACHB) is an autoimmune condition associated with transplacental maternal autoantibodies (anti-SSA/Ro antibodies and anti-SSB/La antibodies) which interfere in the developing heart conduction of the fetus, mostly manifesting as third degree atrioventricular block. The incidence of primary pregnancy in those patients with the positive anti-SSA/Ro antibody is 2%, and 12%-20% in secondary pregnancy. Atrioventricular conduction is the basis of cardiac electric activity and mechanical activity. Atrioventricular block is a disturbance in conduction of sinus or atrial impulse through the specialized conducting system and the severity depends on how fast the ventricular rate is. The incidence of ACHB is low, however, its poor prognosis should be concerned. This review was aimed to provide clinical evidence for screening, treatment and prognosis of this disease by describing the pathogenesis, risk factors, diagnostic methods, prognosis and prenatal interventions.
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