From the Institute for Women's Health, University College London Hospitals; the Cardiac Unit, Great Ormond Street Hospital for Children NHS Trust; and the Division of Immunology, Hammersmith Hospitals NHS Trust, London, United Kingdom.
Obstet Gynecol. 2010 Aug;116 Suppl 2:543-547. doi: 10.1097/AOG.0b013e3181e75a4a.
Congenital heart block affects 2% of all mothers with anti-Ro/La antibodies, can cause heart failure in utero, and has a 20% mortality rate in the first 3 years of life. Maternal fluorinated steroids to prevent or reverse congenital heart block can cause pregnancy complications. Intravenous immunoglobulin (IVIG) has been given with maternal steroids to prevent the recurrence of congenital heart block, although its efficacy is unproven.
We report the use of IVIG to prevent progression of 2:1 congenital heart block with intermittent complete heart block. After two maternal infusions of IVIG (0.4 g/kg) at 31 weeks of gestation, the fetal heart rate reverted to long periods of sinus rhythm, which was sustained until postnatal life.
Our case supports investigating IVIG in the prevention or treatment of this life-threatening condition.
先天性心脏传导阻滞影响所有抗 Ro/La 抗体阳性的母亲的 2%，可导致宫内心力衰竭，并且在生命的头 3 年内死亡率为 20%。为了预防或逆转先天性心脏传导阻滞，母亲使用氟皮质类固醇会引起妊娠并发症。尽管静脉注射免疫球蛋白 (IVIG) 与皮质类固醇联合用于预防先天性心脏传导阻滞的复发，但疗效尚未得到证实。
我们报告了使用 IVIG 预防 2:1 先天性心脏传导阻滞伴间歇性完全性心脏传导阻滞的进展。在妊娠 31 周时接受了两次 IVIG（0.4 g/kg）的母亲输注后，胎儿心率恢复为窦性节律的长间期，这种节律一直持续到新生儿期。
我们的病例支持在预防或治疗这种危及生命的疾病中研究 IVIG 的应用。
