Pascoli I, Cester M, Nanhorngue K, Paternoster D M
Department of Gynecological Sciences and Human Reproduction, University of Padua, Padua, Italy.
Minerva Ginecol. 2008 Apr;60(2):189-91.
Congenital complete heart block (CCHB) is an uncommon disorder with an incidence of about 1/20,000 in liveborn infants. It can occur in the setting of structurally normal heart or with structural disease; it is associated with high mortality and morbidity and requires a high index of suspicion for early diagnosis and therapy. Isolated CCHB in a fetus is usually associated with the presence of autoantibodies to SSA (Ro) and SSB (La) antigens in the maternal circulation. Such antibodies cross into the fetal circulation and cause inflammation of the conduction tissues; the causal mechanism is not known. Although the prognosis for the majority of fetuses is good, it is less favourable in fetuses with a ventricular rate <55 bpm in early pregnancy or with a decrease in the ventricular rate by >5 bpm during pregnancy. It is not known if the same prognostic criteria apply for fetuses with isolated non-autoimmune CCHB. This article reports authors' experience in managing a pregnancy with an extremely low fetal heart rate (47 bpm) in a single fetus with an isolated non-autoimmune CCHB in which the outcome was favorable.
先天性完全性心脏传导阻滞（CCHB）是一种罕见疾病，活产婴儿中的发病率约为1/20000。它可发生于心脏结构正常的情况下，也可伴有结构疾病；其与高死亡率和高发病率相关，需要高度怀疑以进行早期诊断和治疗。胎儿孤立性CCHB通常与母体循环中存在抗SSA（Ro）和SSB（La）抗原的自身抗体有关。此类抗体进入胎儿循环并导致传导组织炎症；其因果机制尚不清楚。尽管大多数胎儿的预后良好，但妊娠早期心室率<55次/分或妊娠期间心室率下降>5次/分的胎儿预后较差。目前尚不清楚相同的预后标准是否适用于孤立性非自身免疫性CCHB胎儿。本文报告了作者对一例单胎孤立性非自身免疫性CCHB且胎儿心率极低（47次/分）的妊娠进行管理的经验，其结局良好。
