Johansen A S, Herlin T
Arhus Universitetshospital, Skejby Sygehus, paediatrisk afdeling.
Ugeskr Laeger. 1998 Apr 20;160(17):2521-5.
Neonatal lupus erythematosus (NLE) is characterized by persistent congenital complete heart block, often without any other structural heart defects. Lupus-like dermatitis is seen transiently, more rarely hepatitis and thrombocytopenia occurs. Recent investigations have shown a close relation between NLE and maternal anti-Ro/La antibodies. These antibodies seem responsible for the destruction of the bundle of His and the AV node in the foetus. Total AV block is seen in 1:15.-22,000 of liveborn children, 70-90% of them are caused by NLE. It is difficult to identify the pregnancies at risk since at delivery most of the mothers (up to 66%) are without symptoms. If the mother has anti-Ro/La antibodies the risk for having a child with NLE is probably less than 5%. However, new investigations have shown that mothers who in addition have anti-DNA antibodies have significantly lower risk of bearing a child with NLE. In most cases foetal complete AV block is found accidentally during pregnancy. Slow foetal heart rate with the demonstration of AV dissociation should not, unless the foetus shows sign of incompensation, lead to acute delivery, but pregnancy should be monitored carefully by serial echocardiography. More than half of the children with congenital heart block need pacemaker therapy shortly after birth. The other children should be followed closely for signs of incompensation and may need pacemaker therapy later on.
新生儿红斑狼疮（NLE）的特征是持续性先天性完全性心脏传导阻滞，通常无任何其他心脏结构缺陷。可见短暂性狼疮样皮炎，较少见肝炎和血小板减少症。最近的研究表明NLE与母体抗Ro/La抗体之间存在密切关系。这些抗体似乎是导致胎儿希氏束和房室结破坏的原因。在每15000 - 22000例活产儿中可见1例完全性房室传导阻滞，其中70 - 90%由NLE引起。由于分娩时大多数母亲（高达66%）无症状，因此很难识别有风险的妊娠。如果母亲有抗Ro/La抗体，生育患NLE孩子的风险可能低于5%。然而，新的研究表明，此外还具有抗DNA抗体的母亲生育患NLE孩子的风险显著降低。在大多数情况下，胎儿完全性房室传导阻滞是在孕期偶然发现的。除非胎儿出现失代偿迹象，否则胎儿心率缓慢伴房室分离现象不应导致紧急分娩，但应通过系列超声心动图对妊娠进行密切监测。超过一半的先天性心脏传导阻滞患儿在出生后不久需要起搏器治疗。其他患儿应密切随访有无失代偿迹象，可能随后需要起搏器治疗。
