先天性血管环的产前产后诊治现状

doi:10.3969/j.issn.1674-8115.2022.11.015

摘要/Abstract

摘要：

先天性血管环（congenital vascular ring，CVR）是一种罕见的先天性血管畸形。主动脉弓发育的过程中，可能发生各种血管的先天性变异，部分变异形成血管环包绕气道和/或食管造成压迫，引起一系列呼吸道及消化道的症状。并且，CVR可能伴发多种染色体及基因异常和其他心内外结构畸形。由于CVR解剖结构及压迫程度的不同，各种类型CVR的治疗方法及预后也不尽相同。随着胎儿大畸形排查中心脏筛查的规范和普及、产后评估方法的多样化以及新术式的应用，CVR的产前检出率有所提高、产后评估更加精确、部分病种术后预后有所改善。但是临床诊疗中对于CVR的认知仍欠完善，缺少有关该病治疗及预后的专业咨询，造成孕妇及家庭的过度焦虑，甚至不必要的引产。同时，各类型血管环的手术指征、治疗方案及预后仍待形成共识。该文总结了CVR各个分型的解剖结构特点、诊断方法、临床表现、治疗及预后现状，以期完善并更新对于该病的认识，指导孕妇妊娠过程中做出更为合理的选择，并为制定诊疗及随访方案提供参考。

关键词: 先天性血管环, 胎儿心脏超声, 产前诊断, 治疗, 预后

Abstract:

Congenital vascular ring (CVR) is a rare congenital vascular malformation. During aortic arch development, congenital variations in vessels may occur, some of which form vascular rings that cause compression around the airway and/or esophagus, causing a range of respiratory and gastrointestinal symptoms. In addition, CVR may be associated with a variety of chromosomal and genetic abnormalities and other intracardiac and extracardiac structural malformations. The treatment and prognosis varies from type to type according to differences in anatomic structure and degree of compression. With the standardization and popularization of prenatal screening of fetal congenital diseases, the liversification of postnatal assessment methods and application of new operation methods, the prenatal detection rate of CVR has been increased, and the postnatal evaluation has been more accurate. The postoperative prognosis of some diseases has been improved. However, the clinical cognition of CVR still remains to be improved. The lack of professional consultation on treatment and prognosis may cause excessive anxiety of gravida and families, and even unnecessary induction. The surgical indication, treatment and prognosis of each type of CVR still remain to reach a consensus. This review summarizes the anatomical characteristics, diagnostic methods, clinical manifestations, and treatment and prognosis of CVR types in order to improve and update the understanding of this disease, guide gravidae to make more reasonable decisions during pregnancy and provide reference for diagnosis, treatment and follow-up plan.

Key words: congenital vascular ring, fetal echocardiography, prenatal diagnosis, treatment, prognosis

中图分类号:

R725.4

顾殊能, 吴鹏飞, 武育蓉. 先天性血管环的产前产后诊治现状[J]. 上海交通大学学报（医学版）, 2022, 42(11): 1620-1626.

GU Shuneng, WU Pengfei, WU Yurong. Diagnosis and treatment of congenital vascular ring: prenatal and postnatal[J]. Journal of Shanghai Jiao Tong University (Medical Science), 2022, 42(11): 1620-1626.

参考文献 54

1	接连利. 胎儿心脏病理解剖与超声诊断学[M]. 北京: 人民卫生出版社, 2010: 260-264.
	JIE L L. Fetel echocardiography and pathoanatomy[M]. Beijing: People′s Medical Publishing House, 2010: 260-264.
2	BACKER C L, MONGÉ M C, POPESCU A R, et al. Vascular rings[J]. Semin Pediatr Surg, 2016, 25(3): 165-175.
3	LICARI A, MANCA E, RISPOLI G A, et al. Congenital vascular rings: a clinical challenge for the pediatrician[J]. Pediatr Pulmonol, 2015, 50(5): 511-524.
4	TROBO D, BRAVO C, ALVAREZ T, et al. Prenatal sonographic features of a double aortic arch: literature review and perinatal management[J]. J Ultrasound Med, 2015, 34(11): 1921-1927.
5	YOSHIMURA N, FUKAHARA K, YAMASHITA A, et al. Congenital vascular ring[J]. Surg Today, 2020, 50(10): 1151-1158.
6	STEPHENS E H, ELTAYEB O, KENNEDY C, et al. Influence of fetal diagnosis on management of vascular rings[J]. Ann Thorac Surg, 2022, 113(2): 630-636.
7	YOUNG A A, HORNBERGER L K, HABERER K, et al. Prenatal detection, comorbidities, and management of vascular rings[J]. Am J Cardiol, 2019, 123(10): 1703-1708.
8	ALVAREZ S G V, MCBRIEN A. Ductus arteriosus and fetal echocardiography: implications for practice[J]. Semin Fetal Neonatal Med, 2018, 23(4): 285-291.
9	AIUM practice parameter for the performance of fetal echocardiography[J]. J Ultrasound Med, 2020, 39(1): E5-E16.
10	EVANS W N, ACHERMAN R J, CICCOLO M L, et al. Vascular ring diagnosis and management: notable trends over 25 years[J]. World J Pediatr Congenit Heart Surg, 2016, 7(6): 717-720.
11	中国医师协会超声医师分会. 中国胎儿心脏超声检查指南[M]. 北京: 人民卫生出版社, 2018: 7.
	Ultrasound Branch of Chinese Medical Doctor Association. Guidelines for Chinese the clinical application of echocardiography[M]. Beijing: People′s Medical Publishing House, 2018: 7.
12	WANG Y, ZHANG Y. Fetal vascular rings and pulmonary slings: strategies for two-and three-dimensional echocardiographic diagnosis[J]. J Am Soc Echocardiogr, 2021, 34(4): 336-351.
13	许燕, 接连利, 姜志荣, 等. 三血管观多切面扫查对胎儿先天性血管环的超声诊断价值[J]. 中国超声医学杂志, 2015, 31(9): 807-809.
	XU Y, JIE L L, JIANG Z R, et al. Value of combination of three vessels views in ultrasound diagnosis of fetal congenital vascular ring[J]. Chin J Med Ultrasound, 2015, 31(9): 807-809.
14	WANG Y, FAN M, SIDDIQUI F A, et al. Strategies for accurate diagnosis of fetal aortic arch anomalies: benefits of three-dimensional sonography with spatiotemporal image correlation and a novel algorithm for volume analysis[J]. J Am Soc Echocardiogr, 2018, 31(11): 1238-1251.
15	SNARR B S, DYER A, THANKAVEL P P. Is there a role for echocardiography in vascular ring diagnosis?[J]. J Am Soc Echocardiogr, 2018, 31(8): 965-966.
16	WORHUNSKY D J, LEVY B E, STEPHENS E H, et al. Vascular rings[J]. Semin Pediatr Surg, 2021, 30(6): 151128. .
17	PRIYA S, THOMAS R, NAGPAL P, et al. Congenital anomalies of the aortic arch[J]. Cardiovasc Diagn Ther, 2018, 8(Suppl 1): S26-S44.
18	涂娟娟, 张丙宏. 螺旋CT和心脏彩超对新生儿先天性血管环诊断价值分析[J]. 中国CT和MRI杂志, 2017, 15(1): 45-48.
	TU J J, ZHANG B H. Spiral CT and cardiac ultrasound diagnostic value of congenital vascular ring analysis[J]. Chin J CT MRI, 2017, 15(1): 45-48.
19	BRET-ZURITA M, CUESTA E, CARTÓN A, et al. Usefulness of 64-detector computed tomography in the diagnosis and management of patients with congenital heart disease[J]. Rev Esp Cardiol, 2014, 67(11): 898-905.
20	李庚武, 王昶, 胡俊, 等. 64层螺旋计算机断层摄影术及1.5T磁共振成像对婴幼儿先天性动脉血管环的诊断价值[J]. 中国循环杂志, 2016, 31(2): 156-160.
	LI G W, WANG C, HU J, et al. Diagnostic value of 64-MSCT and 1.5T MRI in nurseling with congenital vascular rings[J]. Chin Circ J, 2016, 31(2): 156-160.
21	WOODS R K, SHARP R J, HOLCOMB G W 3rd, et al. Vascular anomalies and tracheoesophageal compression: a single institution′s 25-year experience[J]. Ann Thorac Surg, 2001, 72(2): 434-438;discussion 438-439.
22	ALSENAIDI K, GUROFSKY R, KARAMLOU T, et al. Management and outcomes of double aortic arch in 81 patients[J]. Pediatrics, 2006, 118(5): e1336-e1341.
23	BRAVO C, GÁMEZ F, PÉREZ R, et al. Fetal aortic arch anomalies: key sonographic views for their differential diagnosis and clinical implications using the cardiovascular system sonographic evaluation protocol[J]. J Ultrasound Med, 2016, 35(2): 237-251.
24	GUO Q, KONG Y F, ZENG S, et al. Fetal double aortic arch: prenatal sonographic and postnatal computed tomography angiography features, associated abnormalities and clinical outcomes[J]. BMC Pregnancy Childbirth, 2020, 20(1): 614.
25	鲁力, 杨小红, 赵胜, 等. 胎儿单纯双主动脉弓的产前诊断与临床随访分析[J]. 中华超声影像学杂志, 2018(12): 1069-1074.
	LU L, YANG X H, ZHAO S, et al. Prenatal ultrasound diagnosis and clinical follow-up analysis of fetal isolated double aortic arch[J]. Chin J Ultrasonogr, 2018, 27(12): 1069-1074.
26	胡原, 刘倩君, 肖丽苗, 等. 婴幼儿先天性双主动脉弓的临床特征和超声诊断[J]. 临床小儿外科杂志, 2020, 19(9): 842-846.
	HU Y, LIU Q J, XIAO L M, et al. Clinical features and ultrasonic diagnosis of congenital double arch in infants and toddlers[J]. J Clin Pediatr Surg, 2020, 19(9): 842-846.
27	CHARBONNEAU P, FABRE D, LE BRET E, et al. A ten-year single-center surgical experience with symptomatic complete vascular rings[J]. Ann Vasc Surg, 2022, 78: 70-76.
28	YANG Y T, JIN X, PAN Z X, et al. Diagnosis and surgical repair of congenital double aortic arch in infants[J]. J Cardiothorac Surg, 2019, 14(1): 160.
29	ARCIERI L, PROVOST B, CHARBONNEAU P, et al. Three-dimensional printing for surgical planning of a double aortic arch case[J]. J Card Surg, 2020, 35(4): 912-915.
30	BEEMAN A, RAMASWAMY M, SRINIVASAN Y, et al. Importance of acute anterior angulation in double aortic arch needing attention at primary surgery[J]. Front Cardiovasc Med, 2022, 8: 760523.
31	BACKER C L, BHARADWAJ S N, ELTAYEB O M, et al. Double aortic arch with kommerell diverticulum[J]. Ann Thorac Surg, 2019, 108(1): 161-166.
32	SCHMIDT A M S, LARSEN S H, HJORTDAL V E. Vascular ring: early and long-term mortality and morbidity after surgical repair[J]. J Pediatr Surg, 2018, 53(10): 1976-1979.
33	SARAN N, DEARANI J, SAID S, et al. Vascular rings in adults: outcome of surgical management[J]. Ann Thorac Surg, 2019, 108(4): 1217-1227.
34	FLECK R J, PACHARN P, FRICKE B L, et al. Imaging findings in pediatric patients with persistent airway symptoms after surgery for double aortic arch[J]. AJR Am J Roentgenol, 2002, 178(5): 1275-1279.
35	KUSSMAN B D, GEVA T, MCGOWAN F X. Cardiovascular causes of airway compression[J]. Paediatr Anaesth, 2004, 14(1): 60-74.
36	D′ANTONIO F, KHALIL A, ZIDERE V, et al. Fetuses with right aortic arch: a multicenter cohort study and meta-analysis[J]. Ultrasound Obstet Gynecol, 2016, 47(4): 423-432.
37	HAN J C, ZHANG Y, GU X Y, et al. The differential diagnosis of double aortic arch and right aortic arch with mirror-image branches in the fetus: a potential novel method[J]. Pediatr Cardiol, 2021, 42(6): 1405-1409.
38	CAMPANALE C M, PASQUINI L, SANTANGELO T P, et al. Prenatal echocardiographic assessment of right aortic arch[J]. Ultrasound Obstet Gynecol, 2019, 54(1): 96-102.
39	YU D, GUO Z K, YOU X, et al. Long-term outcomes in children undergoing vascular ring division: a multi-institution experience[J]. Eur J Cardiothorac Surg, 2022, 61(3): 605-613.
40	FAN W J, TAN J Y, SHI W H, et al. A systematic review of total thoracic endovascular aortic repair in treatment of kommerell diverticulum combined with right-sided aortic arch[J]. Med Sci Monit, 2021, 27: e930031.
41	OTA T, OKADA K, TAKANASHI S, et al. Surgical treatment for kommerell′s diverticulum[J]. J Thorac Cardiovasc Surg, 2006, 131(3): 574-578.
42	IDREES J, KESHAVAMURTHY S, SUBRAMANIAN S, et al. Hybrid repair of kommerell diverticulum[J]. J Thorac Cardiovasc Surg, 2014, 147(3): 973-976.
43	OSHIMA Y, YAMAGUCHI M, YOSHIMURA N, et al. Management of pulmonary artery sling associated with tracheal stenosis[J]. Ann Thorac Surg, 2008, 86(4): 1334-1338.
44	QI Y H, HUO H H, MA M Y, et al. Prenatal diagnosis of pulmonary artery sling associated with tracheal agenesis: a case report[J]. Echocardiography, 2020, 37(12): 2148-2151.
45	ATKINS M D, FULLER S. Thoracic surgery considerations in the child and young adult[J]. Thorac Surg Clin, 2018, 28(1): 43-52.
46	HIRSIG L E, SHARMA P G, VERMA N, et al. Congenital pulmonary artery anomalies: a review and approach to classification[J]. J Clin Imaging Sci, 2018, 8: 29.
47	WADA Y, MATSUHISA H, MORITA K, et al. Surgical outcomes of pulmonary artery sling and congenital tracheal stenosis with right lung anomaly[J]. Eur J Cardiothorac Surg, 2022, 61(6): 1290-1297.
48	YONG M S, ZHU M Z L, BELL D, et al. Long-term outcomes of surgery for pulmonary artery sling in children[J]. Eur J Cardiothorac Surg, 2019. DOI: 10.1093/ejcts/ezz012.
49	HONG X Y, ZHOU G X, LIU Y H, et al. Management of pulmonary artery sling with tracheal stenosis: LPA re-implantation without tracheoplasty[J]. Int J Clin Exp Med, 2015, 8(2): 2741-2747.
50	HARADA A, SHIMOJIMA N, SHIMOTAKAHARA A, et al. Surgical indication for congenital tracheal stenosis complicated by pulmonary artery sling[J]. J Thorac Dis, 2019, 11(12): 5474-5479.
51	YONG M S, D′UDEKEM Y, BRIZARD C P, et al. Surgical management of pulmonary artery sling in children[J]. J Thorac Cardiovasc Surg, 2013, 145(4): 1033-1039.
52	LES A S, OHYE R G, FILBRUN A G, et al. 3D-printed, externally-implanted, bioresorbable airway splints for severe tracheobronchomalacia[J]. Laryngoscope, 2019, 129(8): 1763-1771.
53	李海燕, 陈博, 孙王明, 等. 儿童无名动脉压迫综合征二例[J]. 中华儿科杂志, 2020, 58(8): 679-681.
	LI H Y, CHEN B, SUN W M. Two cases of innominate artery compression syndrome in children[J]. Chin J Pediatr, 2020, 58(8): 679-681.
54	BACKER C L. The vascular ring decision tree[J]. Eur J Cardiothorac Surg, 2022, 62(1): ezab525.

[1]	李倩玉, 钱逸斐, 李松玲, 朱子俊, 覃雯莉, 刘艳丰, 邱必军. Zeste 12抑制基因在肝细胞癌中的功能及机制[J]. 上海交通大学学报（医学版）, 2025, 45(9): 1138-1148.
[2]	王静怡, 邓佳丽, 朱仪, 丁心怡, 郭嘉婧, 王中领. 新型pH响应性锰基纳米探针用于乳腺癌铁死亡及磁共振成像实验研究[J]. 上海交通大学学报（医学版）, 2025, 45(9): 1183-1193.
[3]	何嘉音, 陈思远, 施晴, 张慕晨, 易红梅, 董磊, 钱樱, 王黎, 程澍, 许彭鹏, 赵维莅. 肾上腺累及的弥漫性大B细胞淋巴瘤患者临床病理特征、基因突变谱及预后分析[J]. 上海交通大学学报（医学版）, 2025, 45(9): 1194-1201.
[4]	陈思远, 施晴, 付迪, 王黎, 程澍, 许彭鹏, 赵维莅. 肺受累弥漫大B细胞淋巴瘤临床病理特征、基因突变谱及预后分析[J]. 上海交通大学学报（医学版）, 2025, 45(9): 1214-1220.
[5]	江怡, 黄晨浩, 李祉良, 吴珺玮, 赵任, 张弢. 1例KRAS突变的结直肠癌患者术前接受化疗联合免疫治疗的效果报道[J]. 上海交通大学学报（医学版）, 2025, 45(9): 1256-1260.
[6]	何苏荟, 赵银龙, 张家毓. 端粒酶基因治疗对压力超负荷心力衰竭小鼠的影响[J]. 上海交通大学学报（医学版）, 2025, 45(8): 949-956.
[7]	严治, 吴星玥, 姚卫芹, 颜灵芝, 金松, 商京晶, 施晓兰, 吴德沛, 傅琤琤. 免疫不全麻痹在新诊断多发性骨髓瘤患者中的动态变化及预后意义[J]. 上海交通大学学报（医学版）, 2025, 45(7): 807-814.
[8]	林桐, 陶怡, 金诗炜, 孙淼, 糜坚青. 血浆置换联合经典化学治疗对多发性骨髓瘤患者肾功能的影响[J]. 上海交通大学学报（医学版）, 2025, 45(7): 823-828.
[9]	孟靖, 谢玉婷, 左佳鑫, 熊屏. 纳米工程化T细胞体系的构建及其对口腔鳞状细胞癌的体外治疗研究[J]. 上海交通大学学报（医学版）, 2025, 45(7): 866-873.
[10]	陈子旋, 刘敏. 肾细胞癌免疫细胞治疗的研究进展[J]. 上海交通大学学报（医学版）, 2025, 45(7): 916-925.
[11]	李卓杭, 于新迪, 任婧雅, 沈佳, 董素贞, 王伟. 主动脉缩窄端侧吻合纠治术后的神经系统预后分析[J]. 上海交通大学学报（医学版）, 2025, 45(6): 753-759.
[12]	高欣洁, 刘艳, 王大威. 地中海贫血基因治疗研究进展及思考[J]. 上海交通大学学报（医学版）, 2025, 45(5): 540-548.
[13]	汤开然, 冯成领, 韩邦旻. 基于单细胞测序与转录组测序构建M2巨噬细胞基因相关的前列腺癌预后模型[J]. 上海交通大学学报（医学版）, 2025, 45(5): 549-561.
[14]	张钲佳, 李小敏, 周鑫, 马海荣, 艾松涛. 高阶磁共振功能成像评估骨与软组织肿瘤价值初探[J]. 上海交通大学学报（医学版）, 2025, 45(5): 585-596.
[15]	李文妙, 邢莉, 潘英瑜, 黄滢, 杨国访, 刘德达. 下颌第一磨牙根管直径和锥度的锥形束CT测量分析[J]. 上海交通大学学报（医学版）, 2025, 45(5): 597-604.