目的 探讨自身抗体相关性先天性心脏传导阻滞的发病机制,临床表现及诊断和治疗。方法 回顾性分析1例自身抗体相关性先天性心脏传导阻滞患儿的临床资料。结果 患儿于24周胎龄时即在超声检查中发现胎儿心动过缓,出生后检测患儿及其母亲的抗SSA/Ro抗体和抗SSB/La抗体均为阳性,确诊为自身抗体相关性先天性心脏传导阻滞,予静脉注射免疫球蛋白1 g/kg治疗。6月龄随访提示仍为Ⅱ度Ⅰ型房室传导阻滞。结论 当发现胎儿或新生儿心动过缓且排除心脏结构异常时,应完善自身免疫抗体检查并尽早干预。
Objective To explore the pathogenesis, clinical manifestations, diagnosis, and treatment of autoantibody-associated congenital heart block. Methods The clinical data of one child with autoantibody-associated congenital heart block was retrospectively analyzed. Results In 24 week gestation, fetal bradycardia had been found by routine fetal echocardiography. After birth, the anti-SSA/Ro antibodies and anti-SSB/La antibodies were positive in both infant and her mother. The diagnosis of autoantibody-associated congenital heart block was confirmed. Intravenous immunoglobulin at 1 g/kg was administrated. At 6 months follow-up, the electrocardiogram suggested type I second degree atrioventricular block. Conclusion In the fetus or neonates found to have bradycardia and excluded the cardiac structural abnormalities, the autoimmune antibody should been tested and early intervention should been promoted.