2022 年第 1 期 第 17 卷

先天性血管环的临床判别与外科治疗经验

Clinical discrimination and surgical treatment experience of congenital vascular ring

作者：段蔚然苏俊武许耀强毛俊杨明王志君

英文作者：Duan Weiran Su Junwu Xu Yaoqiang Mao Jun Yang Ming Wang Zhijun

单位：首都医科大学附属北京安贞医院小儿心脏中心，北京100029

英文单位：Pediatric Cardiology Center Beijing Anzhen Hospital Capital Medical University Beijing 100029 China

关键词：先天性心脏病；血管环；诊断；治疗

英文关键词：Congenitalheartdisease;Vascularring;Diagnosis;Treatment

• 摘要：

• 目的 探讨先天性血管环的临床特征、诊断和外科治疗方法 及疗效。方法 回顾性分析2018年10月至2021年10月于首都医科大学附属北京安贞医院行手术治疗的43例先天性血管环患儿的临床资料，患儿年龄3个月～5.4岁，体质量5～20 kg。所有患儿术前均行X线胸片、心脏彩色多普勒超声检查，部分患儿行心脏大血管和气管树CT三维成像检查。对所有患儿近、中期结果进行随访分析。结果 完全性血管环37例，部分性血管环6例；完全性血管环中，先天性双主动脉弓30例，右位主动脉弓伴左位动脉导管未闭/动脉导管韧带7例；部分性血管环中，肺动脉吊带5例，左位主动脉弓伴迷走右锁骨下动脉1例。入院时17例患儿有明显症状。合并心内畸形12例，术前有明确气道狭窄19例。所有患儿均接受手术治疗。术后死亡1例，死于严重气道狭窄。围术期并发症4例，其中乳糜胸2例、心包积液合并肺不张1例、神经系统并发症1例。随访1～29个月，2例失访，3例患儿出院时仍有轻微呼吸道症状，分别于1、3和6个月后症状消失，其余患儿恢复良好，随访结果满意，术后1、2年生存率均为97.6%（40/41）。结论  婴幼儿出现原因不明的呼吸系统症状需警惕先天性血管环的可能性；心脏多普勒超声联合多排CT可明确诊断；早期正确诊断、及时手术是治疗和改善其预后的关键。

• Objective   To explore the clinical characteristics, diagnosis and surgical treatment of congenital vascular ring. Methods  The clinical data of 43 children with congenital vascular ring who underwent surgery in Beijing Anzhen Hospital, Capital Medical University from October 2018 to October 2021 were analyzed retrospectively. The age of the children ranged from 3 months to 5.4 years, and the body weight was 5-20 kg. All children underwent chest X-ray and cardiac color Doppler ultrasound before operation, and some children underwent CT three-dimensional imaging of cardiac great vessels and tracheal tree. The short-term and medium-term results of all children were followed-up. Results  There were 37 cases of complete vascular ring and 6 cases of partial vascular ring. For complete vascular ring, 30 cases had double aortic arch and 7 cases had right aortic arch and left patent ductus arteriosus/ductus arteriosus ligament. For partial vascular ring, 5 cases had pulmonary artery sling and 1 case had left aortic arch and aberrant right subclavian artery. Seventeen cases combined with obvious clinical symptoms, 12 cases combined with heart malformations and 19 cases combined with preoperative tracheal stenosis. All cases underwent surgery and 1 of them died for severe tracheal stenosis. Four cases had postoperative complications, including 2 cases of chylothorax, 1 case of pericardial effusion and pulmonary atelectasis, and 1 case of neurological complications. The follow-up periods ranged from 1 to 29 months and 2 cases lost. At discharge, 3 cases still had mild respiratory symptoms which respectively disappeared after 1, 3 and 6 months, other children recovered well and the follow-up results were satisfactory. Both 1- and 2-year survival rates were 97.6%(40/41). Conclusion  The children with unexplained respiratory symptoms should pay attention to the possible presence of congenital vascular ring. Cardiac Doppler ultrasound combined with multi-detector CT can show the definite diagnosis. Early correct diagnosis and timely surgical treatment are the key to survival.