胎儿肺动脉起源异常8例尸体解剖分析

Fetal pulmonary artery with abnormal origin:a pathologic analysis of eight cases

目的 对胎儿先天性肺动脉起源异常进行分析,提高该疾病诊断的精准性。方法 回顾性分析8例胎儿先天性心脏畸形病例,均经尸体解剖证实,检查胎儿心血管畸形及内脏的畸形情况,对肺动脉异位起源进行分析总结。结果 肺动脉异位起源8例,其中男胎4例,女胎4例,孕期为孕21~28周。右肺动脉起自升主动脉1例,同时存在主-肺动脉间隔缺损、主动脉弓离断并室间隔完整,即Berry综合征。双侧肺动脉融合起自左头臂干1例,患者伴肺动脉闭锁等多种心血管畸形及心外脏器畸形。左肺动脉和右肺动脉分别起自不同主动脉或分支3例,均伴随肺动脉闭锁,1例左肺动脉起自左头臂干,右肺动脉起自降主动脉及右侧动脉导管;1例左肺动脉起自右动脉导管,右肺动脉起自右锁骨下动脉;1例左肺动脉起自左动脉导管,右肺动脉起自降主动脉。左肺动脉吊带3例,肺动脉主干发出右肺动脉,右肺动脉发出左肺动脉形成血管环。结论 胎儿肺动脉异位起源是一种少见血管畸形,其走行复杂多样,可伴随心内畸形及心外脏器畸形。

Purpose To analyze the abnormal origin of the pulmonary artery in fetus to improve the accuracy of the diagnosis of this disease.Methods A retrospective study was conducted on autopsy cases of fetal congenital heart malformation induced abortion in Beijing Anzhen Hospital in recent years,which were found through echocardiography malformation screening.The fetal cardiovascular and visceral malformations were examined,and the abnormal origin of the pulmonary artery in fetus was analyzed and summarized.Results 8 cases of abnormal origin of the pulmonary artery,4 males and 4 females.The pregnancy period was 21-28 weeks.One case had right pulmonary artery from ascending aorta.At the same time,there were aort-opulmonary septal defect,interruption of aortic arch and intact ventricular septum(Berry syndrome).One case of bilateral pulmonary artery fusion was originated from the left brachiocephalic trunk.The patient was accompanied by a variety of cardiovascular malformations,such as pulmonary atresia,and extracardiac organ malformations.Left and right pulmonary arteries originated from different aortas or branches in 3 cases.All patients were accompanied by pulmonary atresia.In one case,the left pulmonary artery originated from the left brachiocephalic trunk,and the right pulmonary artery originated from the descending aorta and the right ductus arteriosus;In one case,the left pulmonary artery originated from the right ductus arteriosus and the right pulmonary artery originated from the right subclavian artery;In one case,the left pulmonary artery originated from the left ductus arteriosus and the right pulmonary artery originated from the descending aorta.There were 3 cases of left pulmonary artery sling,with the main pulmonary artery originating from the right pulmonary artery and the right pulmonary artery originating from the left pulmonary artery to form a vascular ring.Conclusion Abnormal origin of the pulmonary artery in fetus is a rare vascular malformation with complex and diverse movements,which is typically accompanied by intracardiac and extracardiac organ malformations.