Behan W M, Behan P O, Reid J M, Doig W, Gairns J
Department of Pathology, Glasgow University.
Br Heart J. 1989 Oct;62(4):320-4. doi: 10.1136/hrt.62.4.320.
Complete congenital heart block is associated with the presence of maternal autoantibodies to small ribosomal nucleoproteins (such as anti-Ro) which cross the placenta and may be deposited at the site of cardiac damage. Ten such cases of congenital heart block, their mothers, and their siblings were studied. The seropositive mother of one case had a similar conduction defect (bifascicular block) to that in her affected child. None of the siblings examined had cardiac lesions. Six mothers had Ro or La antibody five to 17 years after the birth of the affected child. Four mothers examined 11-32 years after the birth of an affected child were seronegative. Three of these mothers had evidence of a connective tissue disorder. This evidence is consistent with a hypothesis that a maternal viral infection, associated with autoantibody production, leads to virus crossing the placenta, damaging the fetal heart, and eliciting local deposition of maternal antibody.
完全性先天性心脏传导阻滞与母体存在针对小核糖体核蛋白的自身抗体（如抗Ro抗体）有关，这些抗体可穿过胎盘并可能沉积在心脏损伤部位。对10例此类先天性心脏传导阻滞病例及其母亲和兄弟姐妹进行了研究。其中1例病例的血清学阳性母亲存在与受影响孩子类似的传导缺陷（双束支传导阻滞）。所检查的兄弟姐妹均无心脏病变。6名母亲在受影响孩子出生5至17年后检测出Ro或La抗体。4名在受影响孩子出生11至32年后接受检查的母亲血清学呈阴性。其中3名母亲有结缔组织病的证据。这一证据与以下假说相符：与自身抗体产生相关的母体病毒感染导致病毒穿过胎盘，损害胎儿心脏，并引发母体抗体的局部沉积。
