Department of Pediatric Cardiology, Key Laboratory of Obstetric and Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, Key Laboratory of Obstetric and Gynecologic and Pediatric Diseases of Sichuan Province, West China Second University Hospital, Sichuan University, Chengdu, Sichuan 610041, China.
Chin Med J (Engl). 2017 Dec 5;130(23):2863-2871. doi: 10.4103/0366-6999.219160.
Congenital heart block (CHB) is a rare but life-threatening disorder. More than half of CHB cases are associated with maternal autoimmune, which are termed as autoimmune-associated CHB. This review summarized the recent research findings in understanding autoimmune-associated CHB, discussed the current diagnostic approaches and management strategies, and summarized the problems and future directions for this disorder.
We retrieved the articles published in English from the PubMed database up to January 2017, using the keywords including "Autoimmune-associated", "Autoimmune-mediated", and "Congenital heart block".
Articles about autoimmune-associated CHB were obtained and reviewed.
Observational studies consistently reported that transplacental maternal antibodies might recognize fetal or neonatal antigens in various tissues and result in immunological damages, but the molecular mechanisms underlying CHB pathogenesis still need illuminated. Multiple factors were involved in the process of atrioventricular block development and progression. While several susceptibility genes had been successfully defined, how these genes and their protein interact and impact each other remains to be explored. With currently available diagnostic tools, fetal ultrasound cardiography, and fetal magnetocardiography, most of CHB could be successfully diagnosed and comprehensively evaluated prenatally. The efficacy of current approaches for preventing the progression and recurrence of CHB and other autoimmune-mediated damages was still controversial.
This review highlighted the relationships between autoimmune injuries and CHB and strengthened the importance of perinatal management and therapy for autoimmune-associated CHB.
先天性心脏传导阻滞（CHB）是一种罕见但危及生命的疾病。超过一半的 CHB 病例与母体自身免疫有关，被称为自身免疫相关 CHB。本综述总结了目前对自身免疫相关 CHB 的理解的研究结果，讨论了目前的诊断方法和管理策略，并总结了该疾病存在的问题和未来方向。
我们从 PubMed 数据库中检索了截至 2017 年 1 月以“Autoimmune-associated”、“Autoimmune-mediated”和“Congenital heart block”为关键词发表的英文文章，并进行了筛选。
获得了关于自身免疫相关 CHB 的文章并进行了综述。
观察性研究一致表明，胎盘母体抗体可能识别胎儿或新生儿的各种组织中的抗原，并导致免疫损伤，但 CHB 发病机制的分子机制仍需要阐明。多个因素参与了房室传导阻滞的发展和进展过程。虽然已经成功定义了多个易感基因，但这些基因及其蛋白如何相互作用和影响仍有待探索。目前的诊断工具如胎儿超声心动图和胎儿心磁图可成功诊断大多数 CHB 并进行全面的产前评估。目前预防 CHB 及其他自身免疫介导损伤进展和复发的方法的疗效仍存在争议。
本综述强调了自身免疫损伤与 CHB 之间的关系，加强了围产期管理和治疗自身免疫相关 CHB 的重要性。
