摘要目的:探讨新生儿先天性完全性房室传导阻滞产前、产时、产后诊治，以及转归和生长发育情况。方法:选择2017年12月至2021年6月清华大学第一附属医院新生儿科收治、胎儿期即发现完全性房室传导阻滞的新生儿进行回顾性研究，对其临床特点、治疗及转归情况进行分析。结果:研究期间共收治5例完全性房室传导阻滞患儿，男3例，女2例，均在胎儿期诊断并经剖宫产娩出，诊断胎龄（24.6±3.6）周，出生胎龄（36.7±1.5）周，出生体重（2 493±551）g，其中4例患儿母亲有自身免疫疾病，母亲抗核抗体、抗SSA及抗Ro52抗体均阳性，另1例原因不明。4例患儿生后予异丙肾上腺素治疗后效果差，住院期间最长1例静脉应用异丙肾上腺素18 d；3例住院期间安装永久起搏器，2例1岁内安装起搏器；1例生后26 d因起搏器囊袋感染合并脓毒症休克死亡，存活4例随访到安装起搏器后1岁，生长发育近正常。结论:先天性完全性房室传导阻滞患儿母亲多有自身免疫性疾病，常于孕中期发现胎儿心律失常，但多能近足月分娩，生后药物治疗一般效果差，需安装起搏器。

abstractsObjective:To study the diagnosis, treatment and outcome of congenital complete atrioventricular block (CCAVB) in neonates before, during and after delivery and to monitor their growth and development.Methods:From December 2017 to June 2021, clinical data of 5 neonates with CCAVB admitted to the neonatal department of our hospital were analyzed.Results:A total of 3 males and 2 females were included. The diagnosis was established at (24.6±3.6) weeks during the fetus period. The average birth weight was (2 493±551) g and the gestational age was (36.7±1.5) weeks. 4 mothers had autoimmune diseases, with positive antinuclear antibodies, SSA and Ro52. The cause of the remaining 1 neonate was undetermined. 4 neonates received isoproterenol treatment after birth, but the therapeutic effects were unsatisfying. The longest continuous isoproterenol infusion in 1 neonate lasted for 18 d. Permanent pacemakers were implanted during hospitalization in 3 neonates and 2 neonates were implanted within 1 year after birth. 1 neonate died of septic shock following pacemaker pocket infection at 26 d after birth. The other 4 neonates were followed up to 1-year-old after pacemaker implantation and their growth and development were otherwise normal.Conclusions:Most of the mothers of CCAVB neonates have autoimmune diseases. Fetal arrhythmias are often found in the second trimester during pregnancy, but most of the neonates can be delivered near full-term. The effects of drug therapy are poor and pacemakers are necessary.