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中华医学超声杂志(电子版) ›› 2017, Vol. 14 ›› Issue (03) : 161 -166. doi: 10.3877/cma.j.issn.1672-6448.2017.03.001

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圆锥动脉干相关心血管畸形的再认识:从解剖学到胚胎学、基因学
任卫东1,(), 胡金玲1   
  1. 1. 110004 沈阳,中国医科大学附属盛京医院超声科
  • 收稿日期:2017-01-13 出版日期:2017-03-01
  • 通信作者: 任卫东

Conotruncal malformations: cardiovascular recognition from anatomy to embryology and genetics

Weidong Ren1(), Jinling Hu1   

  • Received:2017-01-13 Published:2017-03-01
  • Corresponding author: Weidong Ren
引用本文:

任卫东, 胡金玲. 圆锥动脉干相关心血管畸形的再认识:从解剖学到胚胎学、基因学[J]. 中华医学超声杂志(电子版), 2017, 14(03): 161-166.

Weidong Ren, Jinling Hu. Conotruncal malformations: cardiovascular recognition from anatomy to embryology and genetics[J]. Chinese Journal of Medical Ultrasound (Electronic Edition), 2017, 14(03): 161-166.

图1 螺旋状走行的圆锥动脉干间隔将圆锥动脉干分为主肺动脉干和升主动脉。螺旋状走行的圆锥动脉干间隔由动脉干嵴和球嵴融合形成。正常形态的圆锥动脉干间隔应具有四大特征:(1)完整无缺损;(2)分隔均匀;(3)螺旋状走行;(4)向下延伸与室间隔对接良好封闭室间孔。这四大特征使得肺动脉干和升主动脉分隔均匀且相互缠绕走行,肺动脉干向下连接右心室,升主动脉向下连接左心室
图2~8 圆锥动脉干间隔发育异常。圆锥动脉干间隔任一特征的丧失都将表现出某种圆锥动脉干发育异常。(1)间隔缺损将表现为主肺动脉间隔缺损(图2),如果间隔缺损同时合并半月瓣的发育异常将表现为共同动脉干(图3);(2)分隔不均将表现为动脉干的狭窄(图4);(3)螺旋不良将表现为大动脉转位(图5);(4)与室间隔对接不良将表现为室间隔缺损(图6),如果与室间隔对接不良同时合并主动脉骑跨比率大于50%将表现为右心室双出口(图7),如果同时合并对接不良、分隔不均和圆锥移位将表现为法洛四联症(图8)
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