母体自身抗体介导的儿童完全性左束支传导阻滞9例临床分析  

Clinical analysis of maternal autoantibody-mediated complete left bundle branch block in 9 children

作　　者：王晓莉 杨芳[1] 韩玲[2] 丁文虹[2] 郭馨馨 林欣雨 陈圣威 Wang Xiaoli;Yang Fang;Han Ling;Ding Wenhong;Guo Xinxin;Lin Xinyu;Chen Shengwei(Department of Pediatrics,Provincial Clinical Medical College of Fujian Medical University,Fuzhou University Affiliated Provincial Hospital,Fujian Provincial Hospital,Fuzhou 350001,China;Department of Pediatric Cardiology,Beijing Anzhen Hospital,Capital Medical University,Beijing 100029,China)

机构地区：[1]福建医科大学省立临床医学院,福州大学附属省立医院,福建省立医院儿科,福州350001 [2]首都医科大学附属北京安贞医院小儿心内科,北京100029

出　　处：《中华儿科杂志》2024年第9期872-876,共5页Chinese Journal of Pediatrics

摘　　要：目的分析母体自身抗体介导的儿童完全性左束支传导阻滞(CLBBB)的临床特点、治疗方法及效果。方法回顾性分析2015年3月至2023年8月北京安贞医院和福建省立医院收治的9例母体自身抗体介导的CLBBB患儿治疗前后的临床特征、心电图、超声心动图变化情况,组间比较采用配对样本t检验。结果母亲阳性自身抗体中抗核抗体6例、抗干燥综合征抗原A抗体5例、抗Ro-52抗体3例。9例患儿女1例、男8例,诊断CLBBB时年龄为1(2,13)月龄;有3例患儿出生3个月内测的阳性自身抗体和母亲一致。治疗上予以抗心力衰竭、营养心肌、静脉注射免疫球蛋白(IVIG)、糖皮质激素(GC)。治疗前肌钙蛋白I、N末端B型脑利钠肽前体(NT-proBNP)均有增高,分别为0.175(0.060,10.270)μg/L、420(327,12865)ng/L,治疗后大多数降至正常。治疗后心电图QRS时限较治疗前明显缩短[(137±15)比(169±25)ms,t=3.76,P<0.001],QTc间期较治疗前明显缩短[(433±41)比(514±27)ms,t=4.95,P=0.001]。治疗前超声心动图存在不同程度二尖瓣反流、三尖瓣反流,室间隔运动明显不协调,治疗后瓣膜反流及室间隔运动协调性明显改善,且治疗后LVEF较治疗前明显升高[(51±13)%比(27±6)%,t=-6.66,P<0.001]。结论母体自身抗体介导的儿童CLBBB可在婴幼儿期出现慢性心力衰竭,及早予以抗心力衰竭、IVIG、GC治疗可改善临床表现。Objective To analyze the clinical characteristics,treatment,and outcomes of children with complete left bundle branch block(CLBBB)mediated by maternal autoantibodies.Methods A retrospective analysis was conducted on nine children diagnosed with maternal autoantibody-mediated CLBBB,treated at Beijing Anzhen Hospital and Fujian Provincial Hospital from March 2015 to August 2023.Their clinical characteristics,electrocardiographic and echocardiographic findings before and after treatment were reviewed.Paired sample t-test was used for inter-group comparison.Results Among the mothers,6 had positive antinuclear antibodies(ANA),5 had anti-Sjogren syndrome antigen A antibodies,and 3 had anti-Ro-52 antibodies.The cohort included one female and eight male children,diagnosed with CLBBB at the age of 1(2,13)months.The positive autoantibodies in the infants,consisted with maternal antibodies,were detected within the first 3 months of life among 3 cases.Treatments included anti-heart failure therapy,myocardial nutritional support,intravenous immunoglobulin(IVIG)and glucocorticoids.Before treatment,the levels of troponin I(0.175(0.060,10.270)μg/L)and N-terminal pro-B-type natriuretic peptide(420(327,12865)ng/L)were elevated,which normalized in most cases after treatment.Post-treatment,the QRS duration significantly shortened compared to pre-treatment((137±15)vs.(169±25)ms,t=3.76,P<0.001),and the QTc interval significantly decreased((433±41)vs.(514±27)ms,t=4.95,P=0.001).Before treatment,varying degrees of mitral and tricuspid regurgitation and marked interventricular septal dyskinesia were observed in echocardiography.After treatment,valve regurgitation and ventricular septum motion significantly improved,with a marked increase in left ventricular ejection fraction((51±13)%vs.(27±6)%,t=-6.66,P<0.001).Conclusions Maternal autoantibody-mediated CLBBB in children presents with chronic heart failure in infancy.Early treatment with anti-heart failure medications,IVIG and glucocorticoids can improve clinical symptoms.

关 键 词：自身抗体 束支传导阻滞 免疫球蛋白类 糖皮质激素类 

分 类 号：R725.4[医药卫生—儿科]