先天性双主动脉弓的临床特点及预后分析  

Clinical characteristics and prognosis of congenital double aortic arch

作　　者：顾燕[1] 丁文虹[1] 王霄芳[1] 梁永梅[1] 焦萌[1] 苏俊武[1] 范祥明[1] 金梅[1] GU Yan;DING Wenhong;WANG Xiaofang;LIANG Yongmei;JIAO Meng;SU Junwu;FAN Xiangming;JIN Mei(Department of Pediatric Cardiology,Beijing Anzhen Hospital,Capital Medical University,Beijing Institute of Heart,Lung and Blood Vessel Diseases,Beijing,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脏中心,100029

出　　处：《心肺血管病杂志》2022年第5期518-522,536,共6页Journal of Cardiovascular and Pulmonary Diseases

摘　　要：目的:分析儿童单纯型和合并心脏畸形的先天性双主动脉弓(DAA)的临床特点,总结诊断及治疗经验。方法:收集2002年1月至2020年5月于我中心诊断DAA并手术治疗的患儿31例。按照是否合并心脏畸形分组并分析临床特点及预后情况。结果:共纳入31例DAA患儿,其中17例为单纯型,14例合并心脏畸形。两组的首诊症状差异有统计学意义(P<0.05),围术期合并心脏畸形组的术前及术后住院时间、手术时间以及监护室时间、有创呼吸机时间均长于单纯组,术后并发症发生率较高(P<0.05)。围术期采取个体化的呼吸道管理:5例术前并急性呼吸道感染者经控制感染后再实施手术;11例术前诊断气管狭窄,均于术中行DAA矫治解除血管环压迫,1例因仍存在气道狭窄同期行矫治术;6例患儿术后经CPAP过渡后撤离呼吸机,有术后并发症者较无并发症者应用CPAP过渡的风险增加(57.1%vs.8.3%,P<0.05)。结论:单纯型和合并心脏畸形的DAA患儿的临床表现有差异。超声心动图、心脏CTA及心血管造影在DAA的诊断和术后随访中各具优势。采取个体化的围术期呼吸道管理有助于术后康复,中远期预后良好。Objective:To analyze clinical characteristics and perioperative management in patients with isolated congenital double aortic arch(DAA)and DAA with concomitant cardiac malformations in a single center.Methods:Data of patients with DAA in Beijing Anzhen Hospital was collected from January 2002 to May 2020.The patients were divided into isolated DAA and DAA with concomitant cardiac anomalies subgroups.Clinical characteristics as well as the postoperative prognosis were collected and analyzed between the two subgroups.Results:A total of 31 patients were included in this study.Among them,17 cases belonged to the isolated subgroup,while 14 cases with cardiac malformations.There was statistical difference in the initial symptoms between the two subgroups(P<0.05).Patients of isolated subgroup were initially reterred to the clinic maily due to abnormal fetal echocardiography screening(64.7%)or respiratory symptoms(35.3%),while patients of the group with cardiac malformation were mainly initially treated for cardiac murmur or cyanosis(35.7%each).Echocardiography was the main method of diagnosis for both subgroups.In addition,4 cases of isolated DAA were missed by echocardiography and further confirmed by cardiac CTA;while 4 cases in the subgroup of DAA with cardiac malformation were confirmed by cardiac CTA and 1 case was confirmed by cardiac angiography.Analysis showed that the preoperative and postoperative hospital stay,operation time,intensive care unit time,as well as invasive ventilator time of the concomitant subgroup were longer than those of the isolated group,and complications were more likely to occur in the concomitant subgroup.Individualized respiratory tract management was adopted in perioperative period.Five patients with preoperative acute respiratory tract infection were operated only after infection control.While eleven patients with preoperative tracheal stenosis firstly underwent DAA to relieve vascular ring compression during operation,one patient underwent tracheal stenosis correction at the same tim

关 键 词：先天性心脏病 双主动脉弓 血管环 临床特点 预后 

分 类 号：R54[医药卫生—心血管疾病]