400例胎儿先天性心脏病的主动脉弓解剖分析

The pathologic analyses of abnormal aortic arch of congenital heart disease of fetus

目的:对胎儿先天性心脏病主动脉弓异常进行分析,提高该疾病诊断的精准性。方法:回顾近几年我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,对其主动脉弓走行进行分析总结,主动脉弓畸形分为6类:主动脉弓分支变异、双主动脉弓、左位主动脉弓、右位主动脉弓、主动脉弓其他异常及肺动脉异常起源。结果:胎儿先天性心脏病尸体解剖400例,主动脉弓异常144例,其中主动脉弓分支变异5例,双主动脉弓3例,左位主动脉弓畸形34例,右位主动脉弓畸形44例,其他畸形55例,肺动脉异常起源5例。左位主动脉弓畸形中动脉导管缺如11例,动脉导管闭锁4例,右锁骨下动脉迷走18例,孤立右锁骨下动脉1例。右位主动脉弓畸形镜面右位主动脉弓33例,左锁骨下动脉迷走7例,孤立左锁骨下动脉1例,孤立无名动脉2例,4支动脉分支对称发出1例。其他畸形包括主动脉弓缩窄45例,主动脉弓离断9例,颈部主动脉弓1例。肺动脉起源异常包括肺动脉吊带3例,Berry综合征1例,肺动脉起源于降主动脉1例。结论:主动脉弓畸形复杂多样,以主动脉弓缩窄最多见,左位主动脉弓畸形以右锁骨下动脉迷走多见,右位主动脉弓畸形主要是镜面右位主动脉弓。

Objective:To analyze the abnormal aortic arch of fetal congenital heart disease and improve the accuracy diagnosis.Methods:We review 400 cases who were found congenital heart disease by echocardiography during pregnancy,and check the course of aortic arch.The aortic arch malformation was divided into six categories:variation of the main branch of the aortic arch,double aortic arch,left aortic arch,right aortic arch,other abnormalities of aortic arch and anomalous origin of pulmonary artery branch from the aortic arch.Results:There were 144 cases of abnormal aortic arch in 400 cases of fetal congenital heart dis-ease,including 5 cases of abnormal branches of aortic arch,3 cases of double aortic arch,34 cases of abnormal left aortic arch,44 cases of abnormal right aortic arch,55 cases of other abnormal origins of pulmonary artery and 5 cases of anomalous origin of pulmonary artery branch from the aortic arch.There were 11 cases of absence of ductus arteriosus,4 cases of atresia of ductus arteriosus,18 cases of aberrant right subclavian artery,and 1 case of isolated right subclavian artery in left aortic arch malformation.There were 33 cases of mirror right aortic arch,7 cases of aberrant left subclavian artery,1 case of isolated left subclavian artery,2 cases of isolated left innominate artery in right aortic arch.There were 45 cases of coarctation of aortic arch,9 cases of interrupted aortic arch and 1 case of cervical aortic arch in other malformations.There were 3 cases of pulmonary artery sling,1 case of Berry syndrome and 1 case of anomalous origin of pulmonary artery branch from the descending aorta.Conclusions:Abnormal aortic arch are complex and varied that coarctation of aortic arch is the most common.The aberrant right subclavian artery is the most common in left aortic arch deformity,and the mirror right aortic arch is the main right aortic arch deformity.