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中华医学超声杂志（电子版）

中华医学超声杂志（电子版） ›› 2018, Vol. 15 ›› Issue (12) : 925 -930. doi: 10.3877/cma.j.issn.1672-6448.2018.12.008

所属专题： 妇产科超声影像学； 文献； 妇产科超声；

妇产科超声影像学

胎儿超声心动图在诊断主-肺动脉间隔缺损中的应用
潘琦1, 邓学东1,†(), 王珍琦1, 徐秋琴2, 马建芳1, 张俊1   
  1. 1. 215002　南京医科大学附属苏州医院　苏州市立医院超声中心
    2. 215000　苏州大学附属儿童医院心超室
  • 收稿日期:2018-06-15 出版日期:2018-12-01
  • 通信作者: 邓学东

The use of fetal echocardiography in the prenatal diagnosis of aortopulmonary septal defect

Qi Pan1, Xuedong Deng1,†(), Zhenqi Wang1, Qiuqin Xu2, Jianfang Ma1, Jun Zhang1   

  1. 1. Center for Medical Ultrasound, The Affiliated Suzhou Hospital of Nanjing Medical University, Suzhou 215002, China
    2. Department of Cardiology, Children′s Hospital of Soochow University, Suzhou 215000, China
  • Received:2018-06-15 Published:2018-12-01
  • Corresponding author: Xuedong Deng
  • About author:
    Corresponding author: Deng Xuedong, Email:
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潘琦, 邓学东, 王珍琦, 徐秋琴, 马建芳, 张俊. 胎儿超声心动图在诊断主-肺动脉间隔缺损中的应用[J]. 中华医学超声杂志（电子版）, 2018, 15(12): 925-930.

Qi Pan, Xuedong Deng, Zhenqi Wang, Qiuqin Xu, Jianfang Ma, Jun Zhang. The use of fetal echocardiography in the prenatal diagnosis of aortopulmonary septal defect[J]. Chinese Journal of Medical Ultrasound (Electronic Edition), 2018, 15(12): 925-930.

目的

探讨主-肺动脉间隔缺损（APSD）的胎儿超声心动图特征。

方法

回顾2008年1月至2017年12月在南京医科大学附属苏州医院苏州市立医院超声中心产前诊断APSD的4例胎儿超声心动图图像，总结胎儿APSD超声心动图特征及产前诊断APSD的临床意义。

结果

4例胎儿APSD最直接的表现是升主动脉和主肺动脉之间的间隔出现回声连续中断，产前超声诊断APSD最关键的切面是三血管观、三血管气管观和大动脉短轴观，彩色多普勒血流成像（CDFI）于缺损处可探及双向、右向左或左向右的低速分流信号。超声提示可能存在APSD的间接征象主要是升主动脉内径增宽。4例胎儿APSD均合并其他心内畸形，其中1例为Berry综合征，1例还合并心外畸形。

结论

APSD有特征性胎儿超声心动图表现。发现远端型APSD时要注意是否为Berry综合征，Berry综合征合并遗传学异常风险较高。产前诊断APSD可行，且对患儿出生后及时接受手术治疗和改善预后意义重大。

关键词: 超声检查，产前, 主-肺动脉间隔缺损, 先天性心脏病
Objective

To investigate the echocardiographic features and clinical significance of prenatal diagnosis of aortopulmonary septal defect (APSD).

Methods

Images of 4 cases of APSD diagnosed by fetal echocardiography during January 2008 to December 2017 in the affiliated Suzhou hospital of Nanjing medical university were reviewed. Echocardiographic features and clinical significance of prenatal diagnosis of APSD were summarized.

Results

The key feature of prenatal diagnosis of APSD was the demonstration of a connection between the ascending aorta and main pulmonary artery. The defect could be best identified on the three-vessel view, three-vessel and trachea view and short axis view of great arteries. Flow across the defect could be bidirectional, left-to-right or right-to-left. Dilated ascending aorta may be seen as an indirect sign of APSD. All the 4 cases were associated with other cardiac anomalies, including 1 case of Berry syndrome. One case was associated with extracardiac abnormalities.

Conclusions

There are fetal echocardiographic features of APSD. Berry syndrome, which was commonly associated with genetic anomaly, should be suspected when distal type APSD was found. Prenatal diagnosis of APSD is feasible and essential as a surgical correction early after birth can improve the prognosis.

Key words: Ultrasonography, prenatal, Aortopulmonary septal defect, Congenital heart disease
图1~4 Berry综合征胎儿产前超声声像图。图1三血管观显示主肺动脉远端近分叉处与主动脉间回声中断（Ⅱ型APSD）；图2 CDFI显示缺损处自主动脉向肺动脉的红色分流；图3显示右肺动脉起自升主动脉；图4主动脉弓长轴观显示主动脉弓连续性中断，中断处位于第二支头臂血管远端（B型主动脉弓离断）
图5~10 胎儿APSD产前超声声像图。图5三血管气管观示肺动脉与升主动脉远端之间的间隔回声中断（Ⅱ型APSD）；图6 CDFI显示缺损处自肺动脉向主动脉的蓝色分流；图7大动脉短轴观显示APSD；图8新生儿超声心动图大动脉短轴显示APSD；图9新生儿CTA显示APSD；图10新生儿CTA测量APSD间距约8.1 mm
表1 4例APSD胎儿的超声心动图表现、遗传学检查及随访结果
胎儿孕周 主要心内畸形 主要诊断切面 分流方向 心外畸形或其他畸形 遗传学检查 随访结果
21＋4 Ⅱ型主-肺动脉间隔缺损（3.8 mm），右位主动脉弓，右位动脉导管，左上腔静脉 三血管观，三血管气管观 左向右分流 单脐动脉，股骨、肱骨偏短 NA 外院引产，未尸检
22＋3 Berry综合征：Ⅱ型主-肺动脉间隔缺损（4.7 mm)，主动脉弓离断（B型），右肺动脉发自主动脉；升主动脉增宽 三血管观，三血管气管观 左向右分流 无 NA 外院引产，未尸检
24＋4 Ⅱ型主-肺动脉间隔缺损（3.9 mm），主动脉弓离断 三血管观，三血管气管观 左向右分流 无 46, XX，22q11del NA 出生后手术证实，存活
25 Ⅱ型主-肺动脉间隔缺损（4.3 mm），左上腔静脉，升主动脉增宽 三血管观，大动脉短轴 右向左分流 无 46, XY，22q11del NA 出生后手术证实，存活
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