Diagnosis and treatment of congenital vascular ring: prenatal and postnatal

doi:10.3969/j.issn.1674-8115.2022.11.015

Abstract

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

CLC Number:

R725.4

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.

TrendMD

References 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]	CHEN Siyuan, SHI Qing, FU Di, WANG Li, CHENG Shu, XU Pengpeng, ZHAO Weili. Clinicopathologic characteristics, gene mutation profile, and prognostic analysis of diffuse large B-cell lymphoma with lung involvement [J]. Journal of Shanghai Jiao Tong University (Medical Science), 2025, 45(9): 1214-1220.
[2]	YAN Zhi, WU Xingyue, YAO Weiqin, YAN Lingzhi, JIN Song, SHANG Jingjing, SHI Xiaolan, WU Depei, FU Chengcheng. Dynamic changes and prognostic significance of immunoparesis in newly diagnosed multiple myeloma patients [J]. Journal of Shanghai Jiao Tong University (Medical Science), 2025, 45(7): 807-814.
[3]	XU Tianyun, SHEN Yiming, JIANG Meng. Clinical management of heart failure with improved ejection fraction: treatment and maintenance [J]. Journal of Shanghai Jiao Tong University (Medical Science), 2025, 45(4): 493-499.
[4]	HUANG Zhouxuan, SHAO Jingbo. Research progress in the treatment of chronic primary immune thrombocytopenia [J]. Journal of Shanghai Jiao Tong University (Medical Science), 2025, 45(4): 508-516.
[5]	ZHOU Zhiyi, ZHAO Hao, MIAO Yifeng, ZHU Chihao, YANG Xi, WANG Siyuan, FENG Junfeng, QIU Yongming. Application of full-neuroendoscopic technique in surgical treatment of posterior cranial fossa lesions [J]. Journal of Shanghai Jiao Tong University (Medical Science), 2025, 45(3): 365-372.
[6]	SONG Yijie, CHEN Tianzhen, ZHONG Na, ZHAO Min. Applications and challenges of generative artificial intelligence in psychiatry [J]. Journal of Shanghai Jiao Tong University (Medical Science), 2025, 45(10): 1271-1278.
[7]	DU Fang, ZHOU Lingyun, CHEN Jiao, LIU Danbo, XIANG Hongxian, CHEN Haifei. A case report of relapsed and refractory multiple myeloma with multifocal extramedullary infiltration and pulmonary adenocarcinoma [J]. Journal of Shanghai Jiao Tong University (Medical Science), 2025, 45(1): 122-128.
[8]	WANG Boen, CHEN Siyuan, SHI Qing, ZHANG Muchen, YI Hongmei, DONG Lei, WANG Li, CHENG Shu, XU Pengpeng, ZHAO Weili. Clinicopathologic characteristics of patients with kidney-involved diffuse large B-cell lymphoma [J]. Journal of Shanghai Jiao Tong University (Medical Science), 2024, 44(9): 1162-1168.
[9]	CHEN Shentse, CHEN Yiming, WANG Fan, ZHANG Mengke, YANG Weichieh, LÜ Dongbin, HONG Wu. Advances in dietary interventions for the treatment of depression-related symptoms [J]. Journal of Shanghai Jiao Tong University (Medical Science), 2024, 44(8): 1050-1055.
[10]	LÜ Qinyi, GAO Ziqi, FENG Qingchen, MEI Hongxiang, LI Juan. Optimizing arch expansion with clear aligners in the mixed dentition based on finite element analysis [J]. Journal of Shanghai Jiao Tong University (Medical Science), 2024, 44(8): 935-943.
[11]	SONG Chenlu, XIANG Jun, YANG Huizhong. Early alarming effect of serum heparin-binding protein on prognosis and occurrence of sepsis in severely burned patients [J]. Journal of Shanghai Jiao Tong University (Medical Science), 2024, 44(4): 474-481.
[12]	WANG Guijie, DU Chuanchong, LU Ye, ZHAO Jian, SHEN Xie, JIN Donglin, GENG Jiacai. Changes of serum high mobility group box 1 and soluble triggering receptor expressed on myeloid cells-1 in patients with multiple injuries and their prognostic significance [J]. Journal of Shanghai Jiao Tong University (Medical Science), 2024, 44(3): 350-357.
[13]	GUO Yonglin, CHEN Moxin, LIU Zheyuan, LI Yifei, WANG Ziqi, SHU Qin, LI Lin. Progress in diagnosis and treatment of strabismus based on artificial intelligence technology [J]. Journal of Shanghai Jiao Tong University (Medical Science), 2024, 44(3): 393-398.
[14]	LIU Meiling, ZHOU Yabing, WANG Xiaoqiang. Advances in the treatment of intracranial neoplastic lesions in children with neurofibromatosis 1 [J]. Journal of Shanghai Jiao Tong University (Medical Science), 2024, 44(3): 399-406.
[15]	CHEN Zixuan, LIU Min. Progress in mechanisms and treatment of sunitinib resistance in renal cell carcinoma [J]. Journal of Shanghai Jiao Tong University (Medical Science), 2024, 44(10): 1307-1315.