Departments of Pediatric cardiology, Pediatrician Kartal Kobuyolu Training and Research Heart Hospital, Itanbul, Turkey.
Indian Pediatr. 2013 May 8;50(5):483-8. doi: 10.1007/s13312-013-0156-3.
Congenital Heart Block (CHB) is the most serious complication of neonatal lupus erythematosus. Transplasental transfer of maternal anti SSA/Ro or antiSSB/La antibodies around 12th week of gestation is associated with development of CHB. This may lead to inflammation, fibrosis and scarring of fetal conduction system in the early second trimester. Different degrees of atrioventricular (AV) block may be seen in the affected fetus. First and second-degree AV blocks may change in severity; however, third degree AV block is irreversible. CHB is mostly diagnosed between 18 - 24th weeks of gestation. Even if most of the mothers carrying autoantibodies of several rheumatic diseases such as systemic lupus erythematosus or Sjogrens syndrome are not aware of their diseases until their children are born with CHB, it is recommended that antibody-positive mothers or the mothers having babies with neonatal lupus erythematosus should be referred for close fetal echocardiographic surveillance beginning from the early second trimester. Although their utility is still controversial, various therapeutic regimes such as sympathomimetic, plasmapheresis, steroids, intravenous immunoglobulin, digoxin, diuretic and in utero pacing have been used for intrauterine treatment of CHB. Aggressive medical treatment is coupled with pacing in infants who do not respond to medical therapy alone.
先天性心脏传导阻滞（CHB）是新生儿红斑狼疮最严重的并发症。母体抗 SSA/Ro 或抗 SSB/La 抗体在妊娠 12 周左右通过胎盘转移与 CHB 的发生有关。这可能导致胎儿传导系统在妊娠中期早期发生炎症、纤维化和瘢痕形成。受影响的胎儿可能会出现不同程度的房室（AV）传导阻滞。一度和二度 AV 阻滞可能会发生变化，但三度 AV 阻滞是不可逆的。CHB 大多在妊娠 18-24 周之间诊断。即使大多数患有系统性红斑狼疮或干燥综合征等几种风湿性疾病自身抗体的母亲直到她们的孩子出生患有 CHB 才意识到自己的疾病，但建议抗体阳性的母亲或患有新生儿红斑狼疮的母亲应从妊娠中期早期开始进行密切的胎儿超声心动图监测。尽管它们的应用仍存在争议，但已经使用了各种治疗方案，如拟交感神经药、血浆置换、类固醇、静脉注射免疫球蛋白、地高辛、利尿剂和宫内起搏，用于 CHB 的宫内治疗。对于单独药物治疗无反应的婴儿，积极的药物治疗与起搏相结合。
