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产前超声诊断主-肺动脉间隔缺损1例

2019-12-19 李继光 张丰明 吴江 临床超声医学杂志

孕妇,37岁,孕2产0,行体外-胚胎移植术,孕19周,否认药物接触史、家族遗传史及近亲结婚史。产科常规检查(-)。胎儿超声心动图检查示:胎儿心脏大小正常,心尖指向左侧,心轴约45°,心房正位,心室右襻,大动脉交叉存在。主动脉弓“拐棍征”不典型,形态欠圆滑,动脉导管探查不清,于主动脉升部至肺动脉分叉前(距肺动脉瓣上约6mm处)见主肺动脉间隔回声失落,宽约5mm,CDFI示大动脉水平见双向分流信号(图

孕妇,37岁,孕2产0,行体外-胚胎移植术,孕19周,否认药物接触史、家族遗传史及近亲结婚史。产科常规检查(-)。胎儿超声心动图检查示:胎儿心脏大小正常,心尖指向左侧,心轴约45°,心房正位,心室右襻,大动脉交叉存在。主动脉弓“拐棍征”不典型,形态欠圆滑,动脉导管探查不清,于主动脉升部至肺动脉分叉前(距肺动脉瓣上约6mm处)见主肺动脉间隔回声失落,宽约5mm,CDFI示大动脉水平见双向分流信号(图1),余未见异常。


图1 孕19周胎儿超声心动图
 
超声提示:胎儿主-肺动脉间隔缺损(Ⅰ型)可能性大,建议随诊。孕妇和家属咨询产科和心外科医师后决定继续妊娠。期间随访,均诊断为胎儿主-肺动脉间隔缺损畸形。孕39+2周于我院行剖宫产生产,8h后行新生儿超声心动图检查:左室略大,余未见异常。大动脉关系正常,主动脉弓降部未见异常,于主动脉升部至肺动脉分叉前见主肺动脉间隔回声失落,约6.8mm;CDFI示大动脉水平可见双向分流(图2)。超声心动图提示:主-肺动脉间隔缺损(Ⅰ型)。


图2 产后8h患儿超声心动图
 
患儿产后28d于外院行主-肺动脉间隔缺损修补术,术后3个月于我院行心脏彩色多普勒超声检查示:大动脉关系正常,主动脉弓降部未见异常,主动脉与肺动脉主干之间回声略增强,CDFI:主、肺动脉间(-)。见图3。


图3 术后3个月患儿超声心动图
 
讨论:
 
胚胎发育第5~8周,由于左、右锥干嵴分隔不全,主-肺间隔发育停滞,致升主动脉和肺动脉间存在残缺,形成主-肺动脉间隔缺损。其多位于升主动脉左后外壁或其后壁,与相邻的肺动脉右前外壁或右肺动脉前壁相通,缺损大小从数毫米至数厘米不等,形状多呈圆形或卵圆形。主-肺动脉间隔缺损分为3型:Ⅰ型(近端缺损),缺损位于主动脉瓣上方约1.0~1.5 cm;Ⅱ型(远端缺损),在升主动脉远端与肺动脉交通;Ⅲ型,完全缺损。胎儿期缺损处显示右向左为主的双向分流信号,由此判断缺损区血流分流接近于动脉导管,故对胎儿心脏发育及功能影响较小。
 
出生后,新生儿左室流出道压力大于右室流出道,缺损处左向右分流造成肺血流量的增多,进而引起肺小动脉痉挛,内膜增厚,中层纤维增生,管腔变小,阻力增加,形成肺动脉高压,若继续发展成阻力型肺动脉高压,则预后较差。故产前胎儿心脏超声筛查时不可局限于固定心脏标准切面的扫查,应加强胎儿心脏大动脉的序贯检查,以期提高此病诊出率。
 
原始出处:

李继光,张丰明,吴江,张帅.产前超声诊断主-肺动脉间隔缺损1例[J].临床超声医学杂志,2018(02):136+138.

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