自身抗体相关性先天性房室传导阻滞发病机制的研究进展

Progress in pathogenesis of autoantibody-related congenital heart block

自身抗体相关性先天性房室传导阻滞是指母体抗Ro/干燥综合征抗原A抗体和/或抗La/干燥综合征抗原B抗体经胎盘传递到胎儿体内产生被动免疫,进而导致胎儿心脏传导系统损伤后发生的房室传导阻滞。但母体抗体阳性并不一定会导致胎儿出现房室传导阻滞,提示该疾病发病机制复杂。本文围绕钙通道学说、细胞凋亡学说、母体其他因素与外部环境变化,以及自然杀伤细胞与人类白细胞抗原在该疾病中的作用进行文献综述。

Autoantibody-related congenital heart block(ACHB)is a passively acquired autoimmune disease developing in fetuses after exposuring to maternal anti-Ro/Sjögren's syndrome type A(SSA)antibody and/or anti-La/SSB antibody transported across the placenta,which contributes to fetal heart conduction system damage and signal conduction block at the atrioventricular node.However,fetal atrioventricular block does not necessarily occur with the presence of maternal autoantibodies,indicating its complex pathogenesis.This review focuses on the theories of calcium channels and apoptosis,the influence of other maternal factors and environmental changes on ACHB and the roles of natural killer cells and human leukocyte antigen in ACHB,aiming to provide reference for further study on the pathogenesis.