Department of Biomedical Science and Human Oncology, Neonatology and Neonatal Intensive Care Unit, University of Bari, "Aldo Moro", P,zza Giulio Cesare, 11, 70125 Bari, Italy.
BMC Pregnancy Childbirth. 2013 Nov 29;13:220. doi: 10.1186/1471-2393-13-220.
Autoantibody-related congenital heart block (CHB) is an autoimmune condition in which trans placental passage of maternal autoantibodies cause damage to the developing heart conduction system of the foetus.
We report a case of an Italian 31-year-old woman, in a good clinical status, referred to our Centre at 26 weeks of her first pregnancy, because of foetal bradycardia, found during routine foetal ultrasonography. Foetal echocardiography revealed a 3rd degree CHB, without any anatomical defects. Despite the mother was asymptomatic for autoimmune disease, anti-Ro/La were searched for, because of the hypothesis of autoantibody-related CHB. High title of maternal anti-Ro/SSA antibodies was found and diagnosis of an autoantibody-related CHB was made. A combination treatment protocol of the mother was started with oral betamethasone, plasmapheresis and IVIG. An emergency C-section was performed at 32 + 3 weeks of gestation because of a non-reassuring cardiotocography pattern. A male newborn (BW 1515 g, NGA, Apgar 8-10) was treated since birth with high-flow O2 for mild RDS. IVIG administration was started at one week, and then every two weeks, until complete disappearance of maternal antibodies from blood. Because of persistent low ventricular rate (<60/min), seven days following birth, pacemaker implantation was performed. The baby is now at 40th week with no signs of cardiac failure and free of any medications.
Up to date, no guidelines have been published for the treatment of "in utero-CHB" and only anecdotal reports are available. It has been stated that a combination therapy protocol is effective in reversing a 2nd degree CHB, but not for 3rd degree CHB. In cases of foetal bradycardia, weekly foetal echocardiographic monitoring needs to be performed and in cases of 2nd degree CHB and 3rd degree CHB maternal therapy could be suggested, as in our case, to avoid foetal heart failure. In cases of 3rd degree CHB often pacemaker implantation is needed.
自身抗体相关性先天性心脏传导阻滞（CHB）是一种自身免疫性疾病，母体自身抗体通过胎盘传递会导致胎儿心脏传导系统发育受损。
我们报告了一例意大利 31 岁女性病例，她在首次妊娠 26 周时因常规胎儿超声检查发现胎儿心动过缓而被转诊至我们中心。胎儿超声心动图显示 3 度 CHB，无任何解剖结构缺陷。尽管该母亲无症状自身免疫性疾病，但由于考虑到自身抗体相关性 CHB 的可能性，仍对其进行了抗 Ro/La 检查。发现母体抗 Ro/SSA 抗体滴度较高，诊断为自身抗体相关性 CHB。开始对母亲进行联合治疗方案，包括口服倍他米松、血浆置换和 IVIG。由于胎心监护图模式不佳，在妊娠 32+3 周时进行了紧急剖宫产。一名男婴（BW 1515g，NGA，Apgar 8-10）出生后即因轻度 RDS 接受高流量 O2 治疗。出生后一周开始给予 IVIG 治疗，然后每两周一次，直至母亲血液中的抗体完全消失。由于心室率持续较低（<60/min），出生后第 7 天进行了起搏器植入术。目前婴儿已 40 周，无心力衰竭迹象，且无需任何药物治疗。
目前尚无“宫内-CHB”治疗的指南，仅有一些个案报道。有研究表明，联合治疗方案可有效逆转 2 度 CHB，但对 3 度 CHB 无效。对于胎儿心动过缓，需要每周进行胎儿超声心动图监测，如果发生 2 度 CHB 和 3 度 CHB，可考虑对母体进行治疗，就像我们的病例一样，以避免胎儿心力衰竭。对于 3 度 CHB，通常需要植入起搏器。
