摘要
目的探讨儿童肺动脉吊带(prognosis of pulmonary artery sling,PAS)患者的临床特点、诊断经验及手术预后。方法对2015年3月至2019年2月广东省心血管病研究所收治的23例PAS患儿的临床表现、影像学特征、治疗方式及预后进行回顾性总结和分析。结果共纳入23例患儿,其中女14例,男9例,年龄6个月(6 d~7岁),体质量6.4(2.8~20.0)kg。临床表现主要为气促7例(30.43%,7/23),咳嗽5例(21.74%,5/23),喘息3例(13.04%,3/23),反复呼吸道感染3例(13.04%,3/23),心脏杂音3例(13.04%,3/23),喉鸣1例(4.35%,1/23),发绀1例(4.35%,1/23)。23例患儿均行心脏超声检查,诊断阳性22例(95.65%);所有患儿术前均行计算机断层扫描(computed tomography,CT)证实PAS,诊断准确率为100%。20例患儿(86.96%,20/23)合并其他先天性心血管畸形。19例患儿(82.60%,19/23)CT和(或)支气管镜检提示存在气管狭窄,其中8例(34.78%,8/23)合并气管畸形,先天性支气管桥(26.10%,6/23)最常见。22例(95.65%,22/23)患儿行PAS矫治术,其中17例(73.91%,17/23)同期行心内畸形矫治,5例(21.73%,5/23)单纯行左肺动脉重建;22例患儿均未进行气管干预,手术存活率为100%。1例(4.35%,1/23)术前因气管中下段重度环形锥样狭窄呼吸窘迫而死亡。随访时间4~36个月,失访2例(9.09%,2/22)。13例患儿仍存在少许咳嗽、气促等呼吸道症状;7例患儿出院后早期因肺部感染再次住院治疗,复查CT提示仍存在不同程度的气管狭窄。结论心脏超声结合CT及支气管镜检查更有利于PAS的综合诊断。采取左肺动脉矫治,避免气管成形,可改善多数患儿呼吸道症状。术后应定期复查评估气管狭窄程度,对于严重气管支气管狭窄的患儿,应进行气管干预手术。
Objectives To investigate the clinical characteristics,diagnostic experience and surgical prognosis of pulmonary artery sling(PAS)in children.Methods The clinical manifestations,imaging features,treatment methods and prognosis of 23 children with PAS admitted to Guangdong Institute of Cardiovascular from March 2015 to February 2019 were retrospectively summarized and analyzed.Results A total of 23 children were included,including 14 females and 9 males,aged 6 days to 7 years old,median 6 months,weight 2.8-20.0 kg,median 6.4 kg.The main clinical manifestations were shortness of breath in 7 cases(30.43%,7/23),cough in 5 cases(21.74%,5/23),wheeze in 3 cases(13.04%,3/23),recurrent respiratory tract infection in 3 cases(13.04%,3/23),cardiac murmur in 3 cases(13.04%,3/23),laryngitis in 1 case(4.35%,1/23),cyanosis in 1 case(4.35%,1/23).Echocardiography was performed in all the 23 cases,and 22 cases(95.65%)were diagnosed as positive.PAS was confirmed by computed tomography(CT)before operation in all the children,and the diagnostic accuracy was 100%.Twenty children(86.96%,20/23)were associated with other congenital cardiovascular malformations.CT and(or)bronchoscopy revealed tracheal stenosis in 19 cases(82.60%,19/23),among which 8 cases(34.78%,8/23)were associated with tracheal deformity and congenital bronchoscope bridge(26.10%,6/23)was the most common.Among the 22 patients(95.65%,22/23)who underwent PAS correction,17(73.91%,15/23)underwent intracardiac malformation correction at the same time,and 5(21.73%,5/23)underwent left pulmonary artery reconstruction alone.None of the 22 cases received trachea intervention,and the operative survival rate was 100%.One case(4.35%,1/23)died of respiratory distress due to severe annular conoid stenosis in the middle and lower trachea before operation.The follow-up duration was 4-36 months,and 2 cases(9.09%,2/22)were lost to follow-up.Thirteen children still had some respiratory symptoms such as cough and shortness of breath.After discharge,7 children were hospitalized again for pulmo
作者
黄欣
潘微
王树水
崔虎军
张智伟
李渝芬
HUANG Xin;PAN Wei;WANG Shu-shui;CUI Hu-jun;ZHANG Zhi-wei;LI Yu-fen(Pediatric Cardiac Ultrasound Room,Guangdong Cardiovascular Institute,Guangdong Provincial People′s Hospital,Guangdong Academy of Medical Sciences,Guangzhou 510100,China;Department of Pediatric Car-diology,Guangdong Cardiovascular Institute,Guangdong Provincial People′s Hospital,Guangdong Academy of Medical Sciences,Guangzhou 510100,China;Section of Pediatric Cardiac Surgery,Guangdong Cardiovascu-lar Institute,Guangdong Provincial People′s Hospital,Guangdong Academy of Medical Sciences,Guangzhou 510100,China)
出处
《岭南心血管病杂志》
CAS
2021年第3期292-297,365,共7页
South China Journal of Cardiovascular Diseases
基金
国家重点研发计划资助(项目编号:2018YFC1002602)。
关键词
肺动脉吊带
气管狭窄
肺动脉吊带矫治术
pulmonary artery sling
tracheal stenosis
correction of pulmonary artery sling
