先天性肺动脉吊带患儿的临床特点及诊治分析  

Clinical features,diagnosis and treatment of congenital pulmonary artery sling in children

作　　者：梁文雅 蔡勇[1] 黄君瑶[2] 王丹瑜 蒋惠 陈德晖[1] Liang Wenya;Cai Yong;Huang Junyao;Wang DanYu;Jiang Hui;Chen Dehui(Department of Pediatrics,First Affiliated Hospital of Guangzhou Medical University,Guangzhou 510120;Statistics Office,First Affiliated Hospital of Guangzhou Medical University,Guangzhou 510120,China)

机构地区：[1]广州医科大学附属第一医院儿科 [2]广州医科大学附属第一医院统计室,广东广州510120

出　　处：《广州医科大学学报》2020年第1期23-26,共4页Academic Journal of Guangzhou Medical University

摘　　要：目的:分析总结儿童先天性肺动脉吊带(PAS)的临床特点与诊治要点。方法:回顾性分析2013年5月到2019年5月广州医科大学附属第一医院收治的11例合并气管狭窄的先天性肺动脉吊带患儿的临床特点、诊断、治疗及预后。结果:11例PAS患儿,其中男9例,女2例,年龄6个月~6岁,中位年龄9个月。11例患儿生后不久即有不同程度的呼吸道症状,包括典型的咳嗽、喘息、喉鸣、气促等,曾均误诊为喘息型支气管肺炎、先天性喉喘鸣、气管狭窄等。11例行超声心动图检查,1例确诊,10例疑诊,其中5例合并其他心脏畸形;10例行胸部CT平扫+气管支气管三围重建均提示气管狭窄,其中3例确诊,2例疑诊;9例患儿行肺动脉CTA均明确诊断,诊断符合率100%。其中7例患儿在本院体外循环下行左肺动脉矫治术,6例术后顺利脱离呼吸机,症状明显改善后出院,1例术后因呼吸衰竭死亡。结论:对生命早期,尤其是1岁内小儿出现不明原因反复咳嗽、喘鸣、喉鸣、气促,要考虑到肺动脉吊带的可能,建议常规行心脏彩超、气道CT三围重建、肺动脉CTA检查。未合并其他气道畸形的PAS患儿可选择单纯左肺动脉移植术治疗。Objective:To summarize the clinical features,diagnosis and treatment of congenital pulmonary artery sling(PAS)in children.Methods:A retrospective analysis was performed on the clinical features,diagnosis,treatment and prognosis in 11 children with congenital PAS who admitted to the First Affiliated Hospital of Guangzhou Medical University between May 2013 and May 2019.Results:The 11 PAS children comprised 9 boys and 2 girls,aged between 6 months and 6 years,with a median age of 9 months.All patients presented varied severity of respiratory symptoms,such as cough,wheezing,laryngeal stridor,and shortness of breath shortly after birth,and had been misdiagnosed with bionchial pneumonia,congenital laryngeal stridor,or tracheal stenosis.By echocardiography,one child was confirmed and 10 others were suspected to have PAS,with comorbidity of other cardiac anomalies identified with five of the cases.Ten children who underwent chest CT plain scan and 3D tracheal bronchus reconstruction were shown to have tracheal stenosis,with PAS confirmed in three and suspected in two of the cases.In nine children who underwent pulmonary artery CTA,all were diagnosed with PAS.Seven of the children diagnosed with PAS underwent left pulmonary artery correction,among whom,six experienced successful ventilatorweaning after the operation and were discharged with significantly improved symptoms,but one died of respiratory failure.Conclusion:Unexplained recurrent respiratory symptoms such as cough,wheezing,laryngeal stridor,and shortness of breath during early life,especially below the age of 1 year,should prompt for a probable PAS.Color Doppler echocardiography,3D CT reconstruction of the airway and pulmonary artery CTA are useful as routine diagnostic tools.Left pulmonary artery re-implantation is recommended for PAS patients without other airway malformations.

关 键 词：肺动脉吊带 儿童 气管狭窄 肺动脉CTA 左肺动脉移植术 

分 类 号：R725.4[医药卫生—儿科]