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中国循证儿科杂志

中国循证儿科杂志 ›› 2016, Vol. 11 ›› Issue (4): 275-279.

• 论著 • 上一篇    下一篇

先天性血管环99例病例系列报告

张新1,2,吴晓云1,吕铁伟1,郑敏1,计晓娟1,冯川3   

  1. 1 重庆医科大学附属儿童医院心血管内科；2 儿童发育疾病研究教育部重点实验室、儿科学重庆市重点实验室、重庆市儿童发育重大疾病诊治与预防国际科技合作基地；3 重庆医科大学附属儿童医院放射科 重庆，400014
  • 收稿日期:2016-06-01 修回日期:2016-08-29 出版日期:2016-08-25 发布日期:2016-08-25
  • 通讯作者: 吴晓云

A systemic report of 99 cases with congenital vascular ring

ZHANG Xin1,2, WU Xiao-yun1, LV Tie-wei1, ZHENG Min1,JI Xiao-juan1,FENG Chuan3   

  1. 1 Department of Cardiology, Children′s Hospital，Chongqing Medical University; 2 Ministry of Education Key Laboratory of Child Development and Disorders,Key Laboratory of Pediatrics in Chongqing,Chongqing International Science and Technology Cooperation Center for Child Development and Disorders, Children's Hospital of Chongqing Medical University; 3 Department of Radiology,Children's Hospital, Chongqing Medical University,Chongqing 400014, China
  • Received:2016-06-01 Revised:2016-08-29 Online:2016-08-25 Published:2016-08-25
  • Contact: WU Xiao-yun

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摘要：

目的 探讨先天性血管环（CVR）的临床表现、诊断及预后的相关因素。方法 回顾性收集2006年1月至2015年12月重庆医科大学附属儿童医院确诊CVR病例，从病史中截取CVR分型、临床表现、影像学检查和治疗信息；根据出我院时的预后分为存活组和死亡或放弃治疗组，行预后不良的危险因素分析。结果 99例CVR患儿进入本文分析。男55例，女44例，中位年龄7.3月龄。肺动脉吊带（PAS）51例，双主动脉弓（DAA）32例，右主动脉弓+左主动脉导管/韧带15例，左主动脉弓+右迷走锁骨下动脉1例。入院时仅呼吸系统表现51例（51.5%），仅心血管系统表现8例（8.1%），仅消化系统表现2例（2.0%），呼吸+心血管系统表现33例（33.3%）， 5例无临床症状。CVR+心内畸形64例（64.6%），其中单纯房间隔缺损(ASD) 31例、室间隔缺损(VSD) 2例，ASD+VSD 9例，CVR+其他复杂心脏畸形22例。气管狭窄69例，食管狭窄14例，喉软骨发育不良10例，先天性支气管肺发育不良9例。超声心动图诊断CVR 54/99例（57.4%），CT血管造影诊断CVR 87/95例（91.6%），超声心动图或CT血管造影未诊断病例均通过CT血管造影或超声心动图诊断；26例行纤维支气管镜检查均发现气道狭窄；14/17例（82.3%）行钡剂食管造影检查示食管受压扭曲、狭窄。手术治疗57例，其中死亡4例，术后脱机困难放弃治疗6例；内科治疗42例，1例死亡，10例脱机困难放弃治疗。死亡或放弃治疗患儿的PAS比例(17/21 vs 34/78)、年龄(6.2 vs 15.5月龄)及合并肺发育不良（6/21 vs 3/78）显著高于存活组。结论 超声心动图联合CTA检查可提高CVR诊断率；PAS、年龄、合并肺发育不良是CVR患儿预后不良的危险因素。

Abstract:

Objective To evaluate the clinical characteristics, diagnosis and prognosis of congenital vascular ring(CVR) in children.Methods The CVR parting ,clinical data,imaging examinations and treatment of 99 cases with CVR in Children′s Hospital of Chongqing Medical University from January 2006 to December 2015 were retrospectively analyzed.According to the prognosis, patients were divided into survival group and death or giving up the treatment groups, the risk factors of poor prognosis were analyzed. Results A total of 99 cases diagnosed as CVR were enrolled,including 55 males and 44 females, with median age of 7.2 months.The diagnosis included pulmonary artery sling in 51 cases,double aorta arch in 32 cases,right aortic arch with left ductus arteriosus in 15 cases,left aoaic arch with vagus right subclavian artery in 1 case. Only 51 cases (51.5%) had respiratory system performance, 8 cases (8.1%) had cardiovascular system performance, 2 cases (2.0%) had digestive system performance, 3 cases (33.3%) had breathing combined cardiovascular system performance 3, 5 patients without clinical symptoms. Merge heart malformations were found in 64 cases (64.6%), of them 31 cases with ASD, 2 cases with VSD, 9 cases with ASD + VSD and 22 cases with complex cardiac anomalies. Tracheobronchial stenosis was found in 69 cases, esophageal stenosis in 14 cases, 10 cases with laryngeal cartilage dysplasia, 9 cases with congenital bronchial pulmonary hypoplasia.The diagnosis accuracy of echocardiographic was 57.4% and that of computed tomography angiography was 91.6%,26 cases checked with the fiber bronchoscope were found airway stenosis; 14/17 (82.3%) accepting line barium esophageal imaging examination were found esophageal pressure distortion and narrow. Fifty-seven cases were treated with surgical operation, of them 4 cases died, six cases with difficult postoperative offline gave up treatment. 42 cases with non-surgical treatment, 1 case died and 10 cases with difficult offline gave up treatment. Age and proportion of the pulmonary artery sling with pulmonary dysplasia of dead or abandoned children group was significantly higher than those of survival group.Conclusion Eehocardiography combined with CTA is the best way to diagnose CVR.Type of CVR,age and the combining pulmonary hypoplasia are related to prognosis.

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