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.

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