胎儿动脉导管缺如的尸体解剖分析

目的对胎儿先天性动脉导管缺如进行尸体解剖分析,以提高该疾病诊断的精准性。方法回顾2011年1月至2019年9月北京安贞医院经超声心动图畸形筛查发现胎儿先天性心脏畸形引产而进行尸体解剖病例400例,检查胎儿心血管畸形及内脏的畸形情况,对其动脉导管缺如进行分析总结。结果400例胎儿先天性心脏病中,动脉导管缺如24例,检出率6%,男女各12例,左位主动脉弓19例,右位主动脉弓5例,21例具有肺动脉主干,3例无肺动脉主干及分支。具体情况如下:(1)肺动脉狭窄伴动脉导管缺如15例,伴发主要心血管畸形:单心室6例,房间隔缺损6例,单心房4例,右心房异构4例,右心室双出口4例,静肺脉异位引流4例,法洛四联症3例,永存左上腔静脉3例;(2)肺动脉闭锁伴动脉导管缺如7例,3例无肺动脉主干,4例肺动脉闭锁伴有狭窄的肺动脉主干,均可见体-肺动脉侧支;(3)法洛四联症伴肺动脉瓣缺如综合征及动脉导管缺如1例,肺动脉扩张;(4)主-肺动脉间隔缺损伴动脉导管缺如1例,肺动脉正常。动脉导管缺如伴发主要心外畸形:脾缺如7例,肺异常7例,内脏反位7例。结论胎儿动脉导管缺如常合并复杂先天性心血管畸形;动脉导管缺如合并肺动脉闭锁常伴体-肺动脉侧支;内脏畸形与伴随的先天性心血管畸形有关。

Absence of arterial duct in fetus: an autopsy analysis

Objective To analyze the absence of congenital arterial duct in fetus and to improve the diagnostic accuracy.Methods Four hundred cases of congenital heart disease diagnosed by echocardiography during pregnancy were examined the fetal cardiovascular malformation and visceral malformation,and the absence of arterial duct was analyzed.Results There were 24(6%)cases of absence of arterial duct,including 19 cases of left aortic arch and five cases of right aortic arch.There were 21 cases with main pulmonary arteries and 3 cases without main pulmonary arteries and branches.There were 15 cases of pulmonary artery stenosis with absence of arterial duct and the major cardiovascular malformations included six cases of single ventricle,six cases of atrial septal defect,four cases of single atrium,four cases of right atrium isomerism,four cases of double outlet right ventricle,four cases of anomalous pulmonary venous drainage,three cases of tetralogy of Fallot,and three cases of persistent left superior vena cava.There were seven cases of pulmonary atresia with absence of arterial duct and with systemic-pulmonary collateral circulation.There was one case of tetralogy of Fallot with absent pulmonary valve and absent arterial duct and the pulmonary artery was dilated.There was one case of aortopulmonary septal defect with absent arterial duct,with normal pulmonary artery.There were also seven cases of asplenia,seven cases of pulmonary abnormality and seven cases of visceral inversion.Conclusions The absence of arterial duct is often associated with congenital heart disease.Pulmonary atresia is often associated with systemic-pulmonary collateral circulation.The visceral malformations are related to the accompanying congenital cardiovascular malformations.