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中华医学超声杂志(电子版) ›› 2019, Vol. 16 ›› Issue (07) : 511 -520. doi: 10.3877/cma.j.issn.1672-6448.2019.07.005

所属专题: 文献

妇产科超声影像学

胎儿右位主动脉弓产前超声诊断、妊娠结局及漏误诊分析
苏虹1,(), 陈明1, 王鸿凤1, 张娜1, 吴新华1, 马琦1, 申庆欣1, 胡程程1, 金芝学1, 陈孟华1   
  1. 1. 150076 黑龙江省哈尔滨市红十字中心医院超声科
  • 收稿日期:2019-04-14 出版日期:2019-07-01
  • 通信作者: 苏虹
  • 基金资助:
    哈尔滨市科技创新人才研究专项资金项目(2015RAQYJ073)

Prenatal ultrasound diagnosis, pregnancy outcome, and reasons for misdiagnosis and missed diagnosis of fetal right aortic arch

Hong Su1,(), Ming Chen1, Hongfeng Wang1, Na Zhang1, Xinhua Wu1, Qi Ma1, Qingxin Shen1, Chengcheng Hu1, Zhixue Jin1, Menghua Chen1   

  1. 1. Department of Ultrasound, the Red Cross Center Hospital of Harbin, Harbin 150076, China
  • Received:2019-04-14 Published:2019-07-01
  • Corresponding author: Hong Su
  • About author:
    Corresponding author: Su Hong, Email:
引用本文:

苏虹, 陈明, 王鸿凤, 张娜, 吴新华, 马琦, 申庆欣, 胡程程, 金芝学, 陈孟华. 胎儿右位主动脉弓产前超声诊断、妊娠结局及漏误诊分析[J/OL]. 中华医学超声杂志(电子版), 2019, 16(07): 511-520.

Hong Su, Ming Chen, Hongfeng Wang, Na Zhang, Xinhua Wu, Qi Ma, Qingxin Shen, Chengcheng Hu, Zhixue Jin, Menghua Chen. Prenatal ultrasound diagnosis, pregnancy outcome, and reasons for misdiagnosis and missed diagnosis of fetal right aortic arch[J/OL]. Chinese Journal of Medical Ultrasound (Electronic Edition), 2019, 16(07): 511-520.

目的

探讨并总结胎儿右位主动脉弓(RAA)超声图像特征、鉴别诊断方法,分析其漏误诊原因,提高胎儿RAA产前超声诊断准确率。

方法

选取2014年1月至2017年12月黑龙江省哈尔滨市红十字中心医院31例经产后小儿超声心动图检查、手术或引产后尸体解剖病理证实的胎儿RAA病例,总结胎儿RAA及其合并圆锥动脉干畸形产前超声图像特征、鉴别诊断方法,分析其漏误诊原因并随访其妊娠结局。应用胎儿心脏标准超声切面的灰阶和彩色多普勒血流成像,进行胎儿心脏系统超声检查。当三血管气管切面(3VT)发现主动脉弓位于气管右侧时,再获取气管及其分支冠状切面进一步明确主动脉弓、动脉导管弓与气管的位置关系。

结果

产前超声诊断41例胎儿RAA,活产20例,引产15例,失访6例;产后证实31例,产前超声正确诊断25例(25/31,80.65%),漏误诊6例(6/31,19.35%)。2例左位主动脉弓产前超声均未获取胎儿气管及其分支冠状切面,在3VT切面将支气管横断面误认为气管横断面而误诊为RAA,2例RAA伴左锁骨下动脉迷走(ALSA)左位动脉导管(LDA),将左颈总动脉误认为发育不良的左弓而误诊为双主动脉弓(DAA),2例RAA合并心脏圆锥动脉干畸形产前超声漏诊RAA。31例胎儿RAA在3VT切面显示主动脉弓位于气管右侧,不同类型的胎儿RAA在3VT切面形成血管环或无血管环。胎儿RAA合并圆锥动脉干畸形在3VT切面和气管及其分支冠状切面有不同超声表现。20例活产胎儿中13例孤立性RAA、2例合并永存左上腔、1例合并2~3腰椎椎体融合、2例合并室间隔缺损手术治疗、最大年龄随访至3岁半,无呼吸道消化道压迫症状,小儿状态良好。1例IDD型矫正型大动脉转位并RAA,未经手术治疗,目前小儿11个月,状态良好。1例合并食道闭锁产后14 d新生儿死亡。6例行胎儿染色体核型检查,5例染色体核型正常,1例合并心内外严重多发畸形胎儿染色体核型检查为18-三体。

结论

3VT切面是诊断胎儿RAA的主要切面,联合应用气管及其分支冠状切面可提高产前超声诊断准确率。单纯性RAA多数预后好、RAA合并严重心内外畸形预后与其合并畸形严重程度有关。

Objective

To investigate the ultrasonic imaging features of and differential diagnosis methods for the right aortic arch, to analyze the causes of missed diagnosis and misdiagnosis, and to further improve the accuracy of prenatal ultrasound diagnosis.

Methods

Cardiac screening examination of the fetus was carried out with views of gray-scale and color Doppler or power Doppler imaging (PDI). When the three-vessel tracheal (3VT) view found that the aortic arch was located in the right of the trachea, the coronary views of the trachea and its branches were obtained to further clarify the relationship among the aortic arch, arterial ductus, and trachea. The sonographic features of 31 cases of fetal right aortic arch were summarized, and the reasons for misdiagnosis were analyzed.

Results

Of the 31 cases of fetal right aortic arch diagnosed by prenatal ultrasound, 20 were alive, 15 were induced, and 6 were lost. As for the results of echocardiography for postpartum children, operation or autopsy were obtained in 31 cases, of which 6 had misdiagnosis or missed diagnosis by prenatal ultrasonography. The coronal views of the trachea and its branches were not obtained in two cases of fetal left aortic arch, and they were misdiagnosed as right aortic arch because the transversely sectioned bronchus were mistaken as the transversely sectioned trachea in the 3VT view. In two cases of right aortic arch with aberrant left subclavian artery, the left common carotid artery was misdiagnosed as a stunted left arch, which resulted in a misdiagnosis as a double aortic arch of the advantage type. In two cases of right aortic arch combined with cardiac defects, right aortic arch was missed in prenatal ultrasound diagnosis. Of 20 children who were followed, 13 were diagnosed with isolated right aortic arch, 2 with combined persistent left superior cavity, 1 with combined 2-3 lumbar vertebral body fusion, and 2 with combined right aortic arch inner or outer non-complex structure abnormality. Follow-up was conducted to the maximum age up to 3 years and 6 months, and 18 of these cases were in good condition and showed no symptoms of respiratory tract compression. One case suffered from complicated intracardiac malformation combined with IDD type congenitally corrected transposition of the great arteries. The child is currently in good condition 11 months after the delivery. One case suffered from combined esophageal atresia and died 14 days after delivery. Chromosome karyotype determination was performed in 6 cases, of whom 5 were normal and 1 was confirmed to have 18-trisomy with severe intracardiac multiple malformation.

Conclusions

The 3VT view is the most important view for diagnosis of right aortic arch of fetus. The accuracy of prenatal ultrasound diagnosis can be improved through the combination of the 3VT view and the coronal view of the trachea and its branches. Simple right aortic arch offers a good prognosis, and the prognosis of right aortic arch with severe ectopic and extracardial malformations depends on the severity of the combined malformation.

表1 31例胎儿右位主动脉弓分型与超声表现
图2 胎儿镜像右位主动脉弓左位动脉导管3VT切面二维与彩色多普勒血流成像图。主动脉弓位于气管右侧,动脉导管位于气管左侧,左位动脉导管与气管前方血管连接不形成血管环
图3 胎儿右位主动脉弓并法洛四联症,动脉导管缺如产前超声图像与引产后病理解剖标本图。图a为3VT切面仅显示1条大动脉位于气管右侧;图b为气管及其分支冠状切面显示主动脉弓位于气管右侧,左肺动脉位于气管左侧;图c为引产儿尸体病理解剖证实:镜像右位主动脉弓并法洛氏四联症、动脉导管缺如
图4 胎儿右位主动脉弓并完全型大动脉转位产前超声图像与引产后病理解剖大体标本图。图a为三血管气管切面仅显示一条大动脉位于气管右侧;图b为气管及其分支冠状切面显示主动脉弓位于气管右侧,动脉导管弓位于气管左侧;图c为引产儿尸体病理解剖证实镜像右位主动脉弓并完全型大动脉转位,左位动脉导管连接左锁骨下动脉
图5 胎儿右位主动脉弓并永存动脉干Ⅰ型产前超声图像与引产后病理解剖大体标本图。图a,b为三血管气管切面二维与彩色多普勒血流成像显示动脉干位于气管右侧其左后壁发出短小的主肺动脉;图c为病理解剖证实镜像右位主动脉弓并永存动脉干Ⅰ型
图7 胎儿右位主动脉弓并肺动脉瓣缺如产前超声图像。气管及其分支冠状切面,主动脉弓位于气管右侧,气管左侧可见瘤样扩张的肺动脉
表2 8例右位主动脉弓合并圆锥动脉干畸形产前超声表现
图8 胎儿左位主动脉弓误诊为右位主动脉弓三血管气管切面二维与彩色多普勒血流成像图。主动脉与肺动脉间距增宽,将主动脉与肺动脉间支气管横断面误认为气管横断面,而将左位主动脉弓误诊为右位主动脉弓
图9 胎儿右位主动脉弓伴迷走的左锁骨下动脉产前误诊为双主动脉弓病例1三血管气管切面二维、彩色多普勒、频谱多普勒图像。升主动脉发出两支,一支粗大的右弓,另一支为细小左颈总动脉,产前误认为发育不良的左弓,左颈总动脉向胎儿左肩部走行与左位动脉导管弓呈上下交叉走行,未绕过气管与食管后方,未与动脉导管弓、降主动脉连接汇合
图10 胎儿右位主动脉弓伴迷走的左锁骨下动脉产前误诊为双主动脉弓病例1引产儿尸体解剖图证实右位主动脉弓伴迷走的左锁骨下动脉并双侧唇腭裂。图a升主动脉发出两支,一支粗大的右弓,另一支为细小左颈总动脉;图b降主动脉发出ALSA,RARCH由左至右发出分支依次为LCCA、RCCA、RSA、ALSA
图11 胎儿右位主动脉弓伴左锁骨下迷走产前误诊为双主动脉弓病例2。图a,b为三血管气管切面二维、频谱多普勒超声图像:升主动脉发出两支,一支粗大的右弓,另一支为细小左颈总动脉,产前误认为发育不良的左弓,左颈总动脉向胎儿左肩部走行与左位动脉导管弓呈平行走行,未绕过气管与食管后方,未与动脉导管弓、降主动脉连接汇合;图c为引产儿尸体解剖证实右位主动脉弓伴左锁骨下动脉迷走,RARCH、MPA、LDA、ALSA形成以气管为中心的"U"形血管环
动态图1 胎儿右位主动脉弓伴左锁骨下动脉迷走产前超声误诊为双主动脉弓左右心室流出道切面、三血管切面二维超声心动图像
动态图2 胎儿右位主动脉弓伴左锁骨下动脉迷走产前超声误诊为双主动脉弓左右心室流出道切面、三血管切面彩色多普勒血流成像
动态图3 胎儿右位主动脉弓伴左锁骨下动脉迷走产前超声误诊为双主动脉弓三血管气管切面彩色多普勒血流成像
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