Finkelstein Y, Adler Y, Harel L, Nussinovitch M, Youinou P
Department of Pediatrics C Schneider Children's Medical Center of Israel, Petah Tikvah, Israel.
Ann Med Interne (Paris). 1997;148(3):205-8.
Complete congenital heart block (CCHB) is the most severe manifestation of neonatal lupus syndrome, associated with a mortality rate of up to 31%. It is caused by irreversible damage to the cardiac conduction system due to the transplacental passage of maternal antibodies to the fetus. Anti-Ro (SSA) and anti-La (SSB) antibodies are usually detected in sera of mothers of children with prenatally diagnosed CCHB and in cardiac tissues of affected newborns. Their pathogeneic role in the development of CCHB has been established in several studies. When CCHB is detected during pregnancy, careful monitoring and delivery are needed before heart failure is developed. Treatment strategies are debatable and may include prophylactic therapy for high-risk pregnant women and a combination of intrauterine plasmapheresis with plasma exchange or with corticosteroids. Pacemaker insertion is required in most infants during the first three months of life. Assays for anti-Ro (SSA) and anti-La (SSB) antibodies should be performed on sera of pregnant women with SLE and newborns with CCHB.
完全性先天性心脏传导阻滞（CCHB）是新生儿狼疮综合征最严重的表现形式，死亡率高达31%。它是由于母体抗体经胎盘传递给胎儿，导致心脏传导系统发生不可逆损伤所致。产前诊断为CCHB的患儿母亲血清以及受影响新生儿的心脏组织中通常可检测到抗Ro（SSA）和抗La（SSB）抗体。多项研究已证实它们在CCHB发病过程中的致病作用。孕期检测到CCHB时，需要在发生心力衰竭前进行密切监测并分娩。治疗策略存在争议，可能包括对高危孕妇进行预防性治疗以及将宫内血浆置换与血浆交换或与皮质类固醇联合使用。大多数婴儿在出生后的头三个月需要植入起搏器。应对患有系统性红斑狼疮的孕妇血清以及患有CCHB的新生儿进行抗Ro（SSA）和抗La（SSB）抗体检测。
