Ruffatti Amelia, Cerutti Alessia, Favaro Maria, Del Ross Teresa, Calligaro Antonia, Hoxha Ariela, Marson Piero, Leoni Loira, Milanesi Ornella
Rheumatology Unit, Department of Medicine, University of Padua, Italy.
Paediatric Cardiology Unit, Department of Women's and Children's Health, University of Padua, Italy.
Clin Exp Rheumatol. 2016 Jul-Aug;34(4):706-13. Epub 2016 Jun 22.
At the moment there are no standard guidelines for the treatment of autoimmune congenital heart block (CHB). We set out to carry out a prospective cohort study to evaluate the benefits, limits, and safety of a combined therapy protocol to treat antibody-related CHB.
Twelve consecutive pregnant patients positive to anti-SSA/Ro ± anti-SSB/La antibodies in whom CHB was detected were prospectively evaluated from 2009 to 2014. The treatment protocol consisted of: weekly plasmapheresis, fortnightly intravenous immunoglobulins (IVIG), and daily 4 mg betamethasone from CHB detection until delivery; IVIG was administered to the neonates soon after birth.
At the time CHB was detected, six of the foetuses presented atrioventricular blocks of 2(nd) degree type and six of 3(rd) degree type. Two of the foetuses with a 2(nd) degree block reverted to a 1st degree block and one to a normal atrioventricular conduction. The condition was stable throughout the pregnancy in the other three cases of 2(nd) degree block. All six 3(rd) degree blocks were stable during pregnancy and confirmed at birth. After a mean of 37.6 months ± 19.6 SD post-birth, the infants with 1st, normal sinus rhythm, and 2(nd) degree blocks at birth were all found to be stable. During the follow-up (29 months ± 19.8 SD), pacemakers were implanted in three of the six infants with 3(rd) degree blocks.
This combined therapy seems to be effective and safe in treating 2(nd) degree CHB, while its efficacy in treating 3rd degree CHB remains to be established.
目前尚无自身免疫性先天性心脏传导阻滞（CHB）的标准治疗指南。我们开展了一项前瞻性队列研究，以评估联合治疗方案治疗抗体相关CHB的益处、局限性及安全性。
对2009年至2014年间连续纳入的12例抗SSA/Ro±抗SSB/La抗体阳性且检测出CHB的孕妇进行前瞻性评估。治疗方案包括：自检测出CHB至分娩，每周进行血浆置换，每两周静脉注射免疫球蛋白（IVIG），每日服用4mg倍他米松；新生儿出生后不久即给予IVIG。
检测出CHB时，6例胎儿为二度房室传导阻滞，6例为三度房室传导阻滞。2例二度传导阻滞胎儿恢复为一度传导阻滞，1例恢复为正常房室传导。另外3例二度传导阻滞胎儿在整个孕期病情稳定。所有6例三度传导阻滞在孕期均稳定，出生时得以确认。出生后平均37.6个月±19.6标准差，出生时为一度、正常窦性心律及二度传导阻滞的婴儿均病情稳定。在随访期间（29个月±19.8标准差），6例三度传导阻滞婴儿中有3例植入了起搏器。
这种联合治疗似乎在治疗二度CHB方面有效且安全，而其在治疗三度CHB方面的疗效仍有待确定。
