新生儿狼疮并房室传导阻滞患儿18例临床分析及中长期随访  

Clinical analysis and long-term follow-up of 18 infants with neonatal lupus complicated with atrioventric⁃ular block

作　　者：陈燕玲 刘玉梅 孙云霞 王一飞 邹瑞坤 谭玉玉 CHEN Yan-ling;LIU Yu-mei;SUN Yun-xia;WANG Yi-fei;ZOU Rui-kun;TAN Yu-yu(Department of Pediatrics,Guangdong Provincial People′s Hospital,Guangdong Academy of Medical Sciences,Guangzhou 510100,China)

机构地区：[1]广东省人民医院(广东省医学科学院)新生儿科,广州510100

出　　处：《岭南心血管病杂志》2022年第4期323-327,333,共6页South China Journal of Cardiovascular Diseases

基　　金：广东省登峰计划项目(项目编号:DFJH201802)。

摘　　要：目的总结新生儿狼疮(neonatal lupus,NL)并发房室传导阻滞(atrioventricular block,AVB)患儿的临床特点,治疗转归及中长期随访,以提高临床对其认识。方法回顾性收集广东省人民医院于2013年1月至2019年12月收治的诊断为NL并发AVB患儿的临床表现、治疗情况及随访资料,并复习相关文献。结果18例NL并发AVB患儿中男12例,女6例。患儿母亲孕前诊断系统性红斑狼疮9例,干燥综合征(Sjögren syn⁃drome,SS)4例,其他自身免疫性疾病2例,无症状(未检测抗体)3例。14例患儿抗SSA抗体阳性伴4例抗SSB抗体阳性,4例抗体阴性。所有患儿于婴儿期发病,存在不同程度的AVB,其中Ⅱ度AVB患儿4例,Ⅲ度AVB患儿14例。所有患儿随访6个月至7年,经综合治疗后,3例Ⅱ度AVB患儿较前无进展,1例Ⅱ度Ⅱ型AVB患儿偶发Ⅲ度AVB,14例Ⅲ度AVB患儿均无明显改善。Ⅲ度AVB患儿中,10例患儿于生后1个月至8年出现心脏扩大伴或不伴左心功能减退,2例患儿出现阿斯综合征(含1例左心扩大)。3例Ⅲ度AVB患儿放弃治疗出院后失访,Ⅲ度AVB患儿中共8例患儿进行永久式心脏起搏器安装;在10例合并心脏扩大的患儿中,5例患儿心脏大小于安装起搏器后1个月内恢复正常,3例于安装起搏器后左心扩大较前改善,2例放弃治疗出院的患儿左心大小无改善并发展至全心扩大。2例患儿存在活动耐力下降,所有患儿暂无明显体格发育落后。结论NL并发AVB多为不可逆性损害,严重患儿可出现阿斯综合征、合并心脏扩大和(或)左心功能减退。对高危孕妇尽早开启系列超声检查能提高胎儿诊断率,早期诊断NL并发先天性心脏传导阻滞,尤其是完全性AVB合并结构性心脏病的患儿,如有安装永久式心脏起搏器指征,应尽早安装以预防患儿猝死或发展为扩张型心肌病。Objectives To summarize the clinical features,treatment,outcomes and long-term follow-up of infants with neonatal lupus(NL)complicated with atrioventricular block(AVB),and improve the clinical understanding of NL.Methods The clinical manifestations,treatment,and follow-up data of infants diagnosed with NL complicated with AVB hospitalized in Guangdong Provincial People′s Hospital from January 2013 to December 2019 were retrospectively analyzed,and relevant literatures were reviewed.Results There were 18 infants involved,including 12 males and 6 females.Maternal diagnoses of 9 infants were systemic lupus erythematosus,4 infants were Sjogren′s syndrome(SS)and 2 infants were other autoimmune diseases,3 infants were asymptomatic(no antibody test was performed).There were 14 infants with anti SSA antibody positive and 4 infants with anti SSB antibody positive,4 infants with anti⁃body negative.All the infants developed in infancy and admitted to hospital with AVB,including 4 infants second-degree block and 14 infants third-degree block.After comprehensive treatment,3 infants with second-degree block improved without any progress,1 infant with second-degree typeⅡblock had occasional third-degree block,and 14 infants with second-degree block had no significant improvement.Among the infants with third-degree block,10 developed cardiac enlargement with or without left ventricular dysfunction from 1 month to 8 years after birth,and 2 infants had AdamStoke syndrome.The follow-up duration ranged from 6 months to 7 years.Three patients with third-degree block withdrew treatment and lost to follow-up after discharge.A total of 8 patients with third-degree block received permanent pacemaker installation.Left ventricular enlargement in 5 infants returned to normal heart size within 1 month after the installation of pacemakers,3 infants were improved after the installation of pacemakers,and the heart size of 2 infants who withdrew treatment did not improve with total cardiac enlargement.Activity endurance was decreased in

关 键 词：新生儿狼疮综合征 房室传导阻滞 永久式起搏器 

分 类 号：R541.7[医药卫生—心血管疾病]