Autoimmune congenital heart block (CHB) may develop in foetuses of women carrying anti‐Ro/SSA and La/SSB autoantibodies and is characterized by disruption of signal conduction at the atrioventricular (AV) node, resulting in partial or complete AV block. If not fatal in utero, complete CHB typically requires lifelong cardiac pacing. No treatment has so far been unequivocally demonstrated to prevent or treat autoimmune CHB, and the relatively low incidence (1%‐5%) and recurrence (12%‐16%) rates of second/third‐degree AV block add to the complexity of managing pregnancies in women with anti‐Ro/La antibodies. Altogether, a better understanding of events leading to development of autoimmune CHB is needed to improve surveillance and treatment strategies. In the past decade, studies have started to look beyond the role of maternal autoantibodies in disease pathogenesis to assess other contributing factors such as foetal genetics and, more recently, immune responses in foetuses and neonates of anti‐Ro/La antibody‐positive women. In this review, we provide an update on the epidemiology, clinical presentation and current treatment approaches of autoimmune CHB, summarize the previously proposed pathogenic mechanisms implicating maternal autoantibodies, and discuss the recent findings of type I interferon (IFN) and innate immune activation in foetuses with autoimmune CHB and in neonates of anti‐Ro/La antibody‐positive mothers, and how these may contribute to autoimmune CHB pathogenesis.

中文翻译：

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自身免疫性先天性心脏传导阻滞中的干扰素和先天性免疫激活

自身免疫性先天性心脏传导阻滞（CHB）可能在携带抗Ro / SSA和La / SSB自身抗体的妇女的胎儿中发展，其特征是房室（AV）节点的信号传导受阻，导致部分或完全的AV阻滞。如果在子宫内不致命，完整的CHB通常需要终身心脏起搏。迄今为止，尚无明确证明可预防或治疗自身免疫性CHB的治疗方法，二级/三级房室传导阻滞的相对较低的发生率（1％-5％）和复发率（12％-16％）增添了复杂性。用抗Ro / La抗体处理孕妇的妊娠。总之，需要更好地了解导致自身免疫性CHB发生的事件，以改善监测和治疗策略。在过去的十年中，研究已经开始超越孕产妇自身抗体在疾病发病机理中的作用，以评估其他促成因素，例如胎儿遗传学以及最近的抗Ro / La抗体阳性女性的胎儿和新生儿的免疫反应。在这篇评论中，我们提供了流行病学的最新信息，