摘要目的：对胎儿肺静脉畸形伴心脏畸形进行尸体解剖分析，以提高对该疾病诊断的精准性。方法回顾北京安贞医院2010年5月至2015年8月因怀孕期间经超声心动图畸形筛查，发现先天性心脏畸形引产而进行尸体解剖的病例，检查胎儿肺静脉畸形、心脏畸形、体静脉畸形以及内脏的畸形情况。结果胎儿先天性心脏病尸体解剖234例，肺静脉畸形19例，其中肺静脉闭锁或发育不良2例、部分型肺静脉异位引流4例、完全性肺静脉异位引流7例、完全性肺静脉共同腔闭锁3例、部分性肺静脉共同腔闭锁2例、肺静脉闭锁伴完全性肺静脉异位引流1例；19例中6例具有3支右肺静脉。伴发心内畸形：单心室11例、右位主动脉弓8例、单心房7例、肺动脉狭窄6例，伴发心外畸形：肺发育异常11例、脾脏异常9例、无脾症8例、多脾1例。结论肺静脉畸形变异较多，常合并严重而复杂心内畸形及心外畸形，解剖时应仔细寻找每一个分支，做到诊断精确。

abstractsObjective To improve the diagnostic accuracy of fetal pulmonary venous abnormalities through the analysis of the fetal pulmonary vein anatomy.Methods 234 cases of congenital cardiac abnormalities were detected by echocardiography during pregnancy in An Zhen Hospital, Capital Medical University from May 2010 to August 2015.Autopsy was then performed.The type of fetal pulmonary venous malformation, cardiac abnormalities, systemic venous malformations, and other internal organs deformities were documented.Results There were ninteen cases of pulmonary venous malformations among the 234 cases of fetal congenital heart disease.These included two cases of congenital pulmonary venous hypoplasia ( CPVH) or atresia, four cases of partial anomalous pulmonary venous drainage ( PAPVD) , seven cases of total anomalous pulmonary venous drainage ( TAPVD ) , five cases of atresia of common pulmonary vein ( CPV ) , one case of congenital pulmonary venous hypoplasia with total anomalous pulmonary venous drainage.There were eleven cases with single ventricle, eight cases with right aortic arch, seven cases with single atrium and six cases with pulmonary valve stenosis.Eleven cases had pulmonary hypoplasia and nine cases had abnormal spleen.Conclusions There are many variations in pulmonary venous abnormalities associated with severe and complex cardiac abnormalities and internal organs malformation.Care should be exercised during autopsy examination to look for all branches of the pulmonary vein.