新生儿主动脉弓畸形超声心动图特征及预后分析  

Echocardiographic features and prognosis of severe aortic arch malformation in newborns

作　　者：石婉明[1] 丁文虹[1] 杨静[1] 莫莹[1] 刘晓然[1] SHI Wanming;DING Wenhong;YANG Jing;MO Ying;LIU Xiaoran(Department of Pediatric Cardiology,Beijing Anzhen Hospital Capital Medical University,Beijing Institute of Heart,Lung and Blood Vessel Diseases,Beijing 100029,China)

机构地区：[1]首都医科大学附属北京安贞医院-北京市心肺血管疾病研究所小儿心外科,100029

出　　处：《心肺血管病杂志》2022年第12期1276-1280,共5页Journal of Cardiovascular and Pulmonary Diseases

摘　　要：目的:探讨新生儿主动脉弓畸形超声心动图特征及临床预后。方法:回顾性分析2019年1月至2022年4月北京安贞医院小儿心脏中心收治的主动脉弓畸形(主动脉弓缩窄,主动脉弓离断)新生儿14例超声诊断特点,临床检查和转归。结果:超声诊断主动脉缩窄患儿9例(64.3%),主动脉弓离断患儿5例(35.7%)。9例主动脉缩窄患儿中:重度5例(55.6%),中度1例(11.1%),轻度3例(33.3%)。5例主动脉弓离断患儿中:A型3例(60.0%),B型2例(40.0%)。主动脉弓畸形合并心内畸形包括室间隔缺损9例(64.3%),动脉导管未闭11例(78.6%),房间隔缺损6例(42.9%),卵圆孔未闭5例(35.7%),头臂分支变异1例(7.1%),右锁骨下动脉异常起源1例(7.1%),主动脉瓣二叶畸形1例(7.1%),细小冠状动脉瘘1例(7.1%),永存左上腔静脉3例(21.4%)。主动脉弓畸形合并重度肺动脉高压12例(85.7%),心室收缩功能减低3例(21.4%),右心室心肌致密化不全1例(7.1%),心包积液3例(21.4%)。14例患儿中9例(64.3%)在全身麻醉体外循环下行一期手术矫治,术后6例(66.7%)存活,3例(33.3%)围术期死亡。余5例中1例经济原因放弃治疗;1例心力衰竭死亡;1例合并胆道闭锁转院治疗;2例病情平稳改为择期手术。结论:新生儿主动脉弓畸形需早期超声识别,产前生后畸形的及时准确评估有助改变预后。Objective:To investigate the echocardiographic features and clinical prognosis of neonatal severe aortic arch malformation.Methods:The ultrasonic diagnostic characteristics,clinical examination and prognosis of 14 newborns with severe aortic arch malformation(aortic arch narrowing and aortic arch disconnection)treated in the pediatric heart center of Beijing Anzhen Hospital from January 2019 to April 2022 were analyzed retrospectively.Results:Ultrasonic diagnosis of aortic coarctation in 9 children(64.3%),aortic arch disconnection in 5 children(35.7%).In 9 cases of aortic coarctation,5 cases were moderate and severe(55.6%),1 case was moderate(11.1%),and 3 cases were mild(33.3%).Among the 5 cases of aortic arch disconnection,3 cases were type A(60.0%)and 2 cases were type B(40.0%).Aortic arch malformation combined with intracardiac malformation included 9 cases of ventricular septal defect(64.3%),11 cases of patent ductus arteriosus(78.6%),6 cases of atrial septal defect(42.9%),5 cases of patent foramen ovale(35.7%),1 case of head arm branch variation(7.1%),1 case of abnormal origin of right subclavian artery(7.1%),1 case of aortic valve bilobar malformation(7.1%),1 case of small coronary artery fistula(7.1%),and 3 cases of persistent left superior vena cava(21.4%).Aortic arch malformation complicated with severe pulmonary hypertension in 12 cases(85.7%),decreased ventricular systolic function in 3 cases(21.4%),right ventricular myocardial insufficiency in 1 case(7.1%),and pericardial effusion in 3 cases(21.4%).Among the 14 children,9(64.3%)underwent one-stage surgical correction under general anesthesia and cardiopulmonary bypass.6(66.7%)survived and 3(33.3%)died during the perioperative period.Among the remaining 5 cases,1 case gave up treatment for economic reasons;one case died of heart failure;one case complicated with biliary atresia was transferred to hospital for treatment;two patients with stable condition were changed to elective operation.Conclusions:Neonatal severe aortic arch malformation needs early

关 键 词：主动弓畸形 新生儿 超声心动图 

分 类 号：R54[医药卫生—心血管疾病]