2020 年第 8 期 第 15 卷

孤立性主动脉血管环的产前诊断与预后分析

Prenatal diagnosis and prognosis assessment of isolated aortic vascular ring 

作者：包照亮1李文秀2张军1苏俊武2张琪3

英文作者：Bao Zhaoliang1 Li Wenxiu2 Zhang Jun1 Su Junwu2 Zhang Qi3 

单位：1首都医科大学附属北京安贞医院妇产科100029；2首都医科大学附属北京安贞医院小儿心脏中心100029；3首都医科大学附属北京安贞医院超声诊断科100029

英文单位：1Department of Obstetrics and Gynecology Beijing Anzhen Hospital Capital Medical University Beijing 100029 China; 2Pediatric Cardiovascular Center Beijing Anzhen Hospital Capital Medical University Beijing 100029 China; 3Department of Echocardiography Beijing Anzhen Hospital Capital Medical University Beijing 100029 China 

关键词：血管环；双主动脉弓；右位主动脉弓；产前诊断 

英文关键词：Vascularring;Doubleaorticarch;Rightaorticarch;Prenataldiagnosis 

• 摘要：

• 目的 探讨各种类型孤立性主动脉血管环的产前诊断与预后情况。方法 选取2015年 1月至2020年1月于首都医科大学附属北京安贞医院行产前超声心动图检查发现的胎儿孤立性主动脉血管环患者174例，分析产前诊断不同类型血管环胎儿的出生后诊断、临床表现、手术治疗情况以及生长发育情况。结果 174例产前诊断孤立性主动脉血管环胎儿中，出生后失访18例，随访156例。21例（12.1%）胎儿产前诊断为双主动脉弓（DAA）并“O”型完整性血管环形成，出生后经超声心动图、心脏CT确诊为DAA 20例，其中7例气管树CT三维成像显示存在气管受压表现，16例行外科手术治疗痊愈出院；另1例外科手术后确诊为右位主动脉弓（RAA）+左位动脉导管(LDA)+迷走左锁骨下动脉。131例（75.3%）胎儿产前诊断为RAA+LDA并“U”型完整型血管环形成，出生后失访16例,出生后1~3个月行超声心动图检查诊断为RAA+LDA+迷走左锁骨下动脉90例，镜像右位主动脉弓（MRAA）+LDA 25例。5例（2.9%）胎儿产前诊断高度怀疑MRAA+LDA，不除外DAA，其中4例出生后经超声心动图检查诊断为MRAA+LDA， 1例经心脏CT检查后确诊为右侧优势型DAA，左侧主动脉弓远端闭锁，患儿出现相关的呼吸系统症状行DAA外科矫治手术，术后7 d痊愈出院。未手术者随访期间均无相关的临床症状，生长发育正常。结论 产前诊断各种类型孤立性主动脉血管环胎儿预后良好，分娩后重点观察新生儿有无呼吸系统及食管受压的临床症状，及时进行相关的检查，出现相关症状应积极就医，避免延误治疗。

• Objective To investigate prenatal diagnosis and prognosis of various types  of isolated aortic vascular ring. Methods  From January 2015 to January 2020, the medical records of 174 patients with fetal isolated aortic ring found by prenatal echocardiography admitted to Beijing Anzhen Hospital, Capital Medical University were collected. The postnatal diagnosis, clinical manifestations, surgical treatment, growth and development of fetuses with different types of vascular rings were analyzed prospective.Results Among 174 cases of prenatal diagnosis of isolated aortic rings, 18 cases were lost after birth and 156 cases were followed up. Twenty-one fetuses (12.1%) were diagnosed as double aortic arch (DAA) with "O" type intact vascular ring formation. After birth, 20 cases was diagnosed as DAA by echocardiography and cardiac CT, CT three-dimensional imaging of the tracheal tree showed that 7 patients had tracheal compression, 16 cases were cured by surgical treatment, and 1 case was diagnosed as right aortic arch (RAA)+left ductus arteriosus (LDA)+vagal left subclavian artery（ALSA） after surgery. One hundred and thirty-one cases (75.3%) were diagnosed as RAA+LDA with "U" type intact vascular ring formation. Sixteen cases were lost to follow-up after birth. Ninety cases were diagnosed as RAA+LDA+ALSA by echocardiography 1-3 months after birth, and 25 cases were MRAA+LDA. Five cases (2.9%) were highly suspected of MRAA+LDA in prenatal diagnosis, not excluding DAA, including 4 cases diagnosed as MRAA+LDA by echocardiography after birth, 1 case diagnosed as right dominant DAA after cardiac CT examination, with left aortic arch distal atresia. The children with related respiratory symptoms were treated with DAA surgery and they were cured and discharged 7 days after operation. During the follow-up period, the unoperated children had no related clinical symptoms, their growth and development were normal. Conclusion Prenatal diagnosis of various types of isolated aortic rings the fetus has a good prognosis. After delivery, the clinical symptoms of respiratory system and esophageal compression should be observed, and relevant examinations should be carried out in time. If there are relevant symptoms, active medical treatment should be taken to avoid delayed treatment.