MSCT及MRI诊断先天性右肺动脉缺如伴动脉导管未闭1例

2019-12-11 俞金娜 徐宏伟 邱勇钢 中国医学影像技术

患者女,20岁,因“咳嗽、咳痰伴走路气急1月余”入院。患者1岁时曾发现心脏扩大,诊断为“肥厚性心肌病”未经特殊治疗,此后反复肺部感染,运动后胸闷气促,近几年病情加重。体格检查:胸骨左缘第2、肋间可闻及收缩期杂音。

患者女,20岁,因“咳嗽、咳痰伴走路气急1月余”入院。患者1岁时曾发现心脏扩大,诊断为“肥厚性心肌病”未经特殊治疗,此后反复肺部感染,运动后胸闷气促,近几年病情加重。体格检查:胸骨左缘第2、肋间可闻及收缩期杂音。
 
X线胸片:左肺纹理增多,右肺纹理稀疏,肺动脉段突出。心脏超声:右心室增大、室壁增厚,中度肺动脉高压,右肺动脉显示不清;增强MSCT:左肺纹理增多,右下肺纹理稀疏,左肺膨胀且通过前纵隔向右延伸,右上肺可见压迫边缘;右心室明显增大,左肺动脉增宽,右肺动脉起始端呈盲端,断端光滑,右肺动脉未显示,纵隔及心脏右移(图1A)MRP示主动脉与肺动脉之间有一通道;肺动脉干增宽,最大直径约33mm(图1B);VR示主动弓下方与肺动脉干之间见动脉导管未闭,直径约18mm;右下肺动脉缺如,右侧胸前内侧多条侧支血管形成(图1C)。心脏动态MRI示主动脉与肺动脉间血流相通(动脉导管未闭)、右肺动脉缺如(图1D)。影像诊断:右肺动脉缺如伴动脉导管未闭,肺动脉高压,右侧体一肺侧支循环形成。




图1右肺动脉缺如伴动脉导管未闭A.增强MSCT轴位;B.MPR图像,箭示主动脉与肺动脉间异常通道;C.VR图像;D.MR冠状位图像
 
讨论
 
单侧肺动脉缺如是一种罕见的先天性肺血管畸形,常累及右侧,多合并其他心血管疾病,也可单发。在胚胎发育初期,肺动脉起源的第6对主动脉弓的腹侧由于各种原因发育缺陷或过早闭塞退化,导致其未能与各级肺内动脉分支相连,形成单侧肺动脉缺如,对本病的诊断和治疗均有一定难度。本例患者误诊近20年,既往诊断为“肥厚性心肌病”后经MSCT及MR等影像学检查综合诊断为右肺动脉缺如合并动脉导管未闭、肺动脉高压、右侧体一肺侧支循环形成。
 
单侧肺动脉缺如患者临床表现为反复肺部感染、活动后胸闷、气促或咯血等,常导致肺动脉高压,严重者可发展为充血性心力衰竭。X线胸片对诊断单侧肺动脉缺如有重要的提示作用。心脏超声检查可发现心血管畸形及肺动脉高压,但探查肺动脉病变有一定局限性。增强MSCT常表现为缺如侧的肺动脉起始部或近端呈盲端,断端光滑,远端未见显影,亦未见相延续的血管影;肺窗可显示肺动脉缺如侧不同程度的肺纹理稀疏,肺野透亮度增高,肺体积缩小。
 
本例患者MSCT的VR图像还清晰显示动脉导管未闭和右侧体-肺侧支循环形成,右肺供血动脉主要来源于右肋间动脉及锁骨下动脉,以此来保证肺灌注。心脏动态MRI可多平面、多序列显示主、肺动脉的结构及血流异常改变,且不需注射对比剂。心血管造影仍是目前诊断单侧肺动脉缺如的金标准,但由于其费用昂责、有创而应用受限。而MSCT、MRI具有无创、准确和快速的优点,对该类患者术前影像诊断有重要价值,并且随着仪器性能和扫描技术的发展,图像后处理也更趋完善,总体诊断评价将进_步提升。
 
原始出处:

俞金娜,徐宏伟,邱勇钢.MSCT及MRI诊断先天性右肺动脉缺如伴动脉导管未闭1例[J].中国医学影像技术,2018,34(01):157-158.

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    2020-02-18 zywlvao
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    2019-12-13 huirong
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    2019-12-13 huanbaofeng
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    2019-12-13 docwu2019

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