PDF(Pubmed)
Abstract：
Complete congenital heart block (CHB), a rare and fatal bradyarrhythmia observed in children, carries significant mortality and morbidity. When congenital heart block occurs in isolation with a structurally normal heart, it prompts suspicion of an autoimmune etiology, wherein maternal antibodies are transmitted transplacentally, impacting the fetal conducting system. The manifestation of congenital complete atrioventricular block (CCAVB) can lead to complications such as dilated cardiomyopathies, arrhythmias, and fibroelastosis in certain cases. Notably, dilated cardiomyopathy is a significant prognostic factor in children diagnosed with congenital heart block. Pathological investigations have revealed the presence of antibodies, complements, and indicators of inflammation or fibrosis across the myocardium, emphasizing the shared molecular mechanisms between CCAVB and the development of dilated cardiomyopathy (DCM). This article presents the case of a one-year-old female child who presented with signs of dilated cardiomyopathy, later identified through retrospective evaluation as having autoimmune congenital heart block. The mother of the child was diagnosed with Sjogren\'s syndrome, characterized by positive anti-RO titers. Remarkably, the child remained asymptomatic for a year without the need for pacing intervention. The child\'s condition was successfully stabilized with appropriate treatment, and plans for pacemaker insertion will be considered once specific criteria are met. The onset of cardiomyopathy in a known case of CCAVB should serve as a crucial alert for prognostic considerations and the potential necessity for early-pacing intervention.
摘要：
完全先天性心脏传导阻滞（CHB），在儿童中观察到的罕见和致命的缓慢性心律失常，具有显著的死亡率和发病率。当先天性心脏传导阻滞与结构正常的心脏隔离发生时，它提示怀疑自身免疫性病因，其中母体抗体经胎盘传播，影响胎儿传导系统.先天性完全性房室传导阻滞（CCAVB）的表现可导致扩张型心肌病等并发症，心律失常,在某些情况下和纤维弹性增生症。值得注意的是,扩张型心肌病是诊断为先天性心脏传导阻滞的儿童的重要预后因素。病理调查显示存在抗体，补语,以及心肌炎症或纤维化的指标，强调CCAVB与扩张型心肌病（DCM）发展之间的共同分子机制。本文介绍了一名一岁的女性儿童的情况，该儿童表现出扩张型心肌病的体征，后来通过回顾性评估确定患有自身免疫性先天性心脏传导阻滞.孩子的母亲被诊断出患有干燥综合征,以阳性抗RO滴度为特征。值得注意的是,患儿在一年内无症状,无需起搏干预.通过适当的治疗，孩子的病情成功稳定，一旦达到特定标准，将考虑起搏器插入计划。在已知的CCAVB病例中，心肌病的发作应作为预后考虑和早期起搏干预的潜在必要性的关键警报。
