两例自身抗体导致先天性心脏传导阻滞胎儿的治疗及随访。

Treatment and follow-up of fetuses that developed congenital heart block due to autoantibody in two cases.

机构信息

Department of Echocardiography, Beijing Anzhen Hospital, Capital Medical University, Beijing, China.

Department of Ultrasonography, Affiliated Hospital of Hebei University, Baoding, China.

出版信息

J Int Med Res. 2020 Jun;48(6):300060520925596. doi: 10.1177/0300060520925596.

DOI:10.1177/0300060520925596

PMID:32588691

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7436825/

Abstract

OBJECTIVE

Autoantibody-related congenital heart block (ACHB) is a passively acquired autoimmune disease. This study aimed to examine the pathogenesis, clinical manifestations, and treatment of ACHB.

METHOD

The clinical data of two fetuses with first-degree ACHB were retrospectively analyzed.

RESULTS

Two pregnant women were strongly positive for anti-Sjögren's syndrome-related antigen A (SSA) antibody. Among these two cases, one had a prolonged atrioventricular (AV) interval at 28 weeks , while the other had a prolonged AV interval at 24 weeks . After prenatal intervention, one patient recovered to normal, while one fetus continued to have ACHB after treatment with dexamethasone and intravenous immunoglobulin. Furthermore, the two neonates were positive for anti-SSA antibody and were diagnosed with ACHB.

CONCLUSION

The pathogenesis of ACHB is closely correlated with anti-SSA/Ro antibody and anti-SSB/La antibody from the mother, and is affected by fetal susceptibility. Early screening and early intervention for ACHB are important.

摘要

目的

自身抗体相关性先天性心脏传导阻滞（ACHB）是一种被动获得性自身免疫性疾病。本研究旨在探讨 ACHB 的发病机制、临床表现和治疗方法。

方法

回顾性分析两例一度 ACHB 胎儿的临床资料。

结果

两名孕妇抗干燥综合征相关抗原 A（SSA）抗体均强阳性。两例中一例于 28 周时出现房室（AV）间期延长，另一例于 24 周时出现 AV 间期延长。经产前干预后，一例患者恢复正常，另一例患者经地塞米松和静脉注射免疫球蛋白治疗后仍持续存在 ACHB。此外，两名新生儿均为抗 SSA 抗体阳性，被诊断为 ACHB。