胎儿右主动脉弓畸形合并多脏器畸形尸解病理分析  

The pathological analysis of the right aortic arch deformities combined with multiple organ deformities in fetal autopsies and the review of the literature

作　　者：杜瑞[1] 康小玲[1] 饶金[1] 张佳立[1] 廖雨荷 郭玉娟[1] 李辉斌[1] 

机构地区：[1]广东省妇幼保健院病理科,518017

出　　处：《心肺血管病杂志》2014年第6期870-872,877,共4页Journal of Cardiovascular and Pulmonary Diseases

摘　　要：目的:通过研究胎儿右主动脉弓畸形的血管走行及其合并多脏器畸形,并进行文献复习,提高对此畸形的认识。方法:在我院近10年胎儿尸解1 400余例中,发现4例右主动脉弓发育畸形,对其进行详细解剖和观察,同时复习文献。结果:4例右主动脉弓病例中,男性2例,女性2例;I型2例,Ⅱ型2例,4例均有复杂先天性心脏病,3例合并心外多脏器畸形。文献所见,右主动脉弓存活者不少,有症状者多系血管环压迫所致。结论:右主动脉弓并不罕见,可为单发畸形,亦常合并多种心脏及或心外畸形,其非致死性畸形,伴血管环者可通过手术矫正。B超、MIR、CT等可在孕中期发现此畸形。染色体检查亦多有异常。加深对其认识具有重要的临床实践意义。Objective：To investigate the vascular trend of the right aortic arch malformations and other organ malformations associated with them in fetuses and review the literature in order to improve the understanding of these malformations. Methods： Four cases of the right aortic arch malformations were retrieved in about 1400 fetal autopsies during the past ten years in our hospital. Careful dissections and observations were carried out to reveal the structures in these cases and the literature was reviewed, Results ：2 of the 4 cases were male, and the other two were female. Type I and type H accouted for 50% of all the cases respectively. Moreover, complex congenital heart diseases were also founded in all the four cases and extracardiac malformations were observed in 3 cases. A lot of patients of the fight aortic arch malformations survived and some patients had symptoms for the compressed vascular circle in the literature. Conclusion：Right aortic arch malformations are not rare, and they may occur with or without various cardiac or extracardiac malformations. They are not fatal. Some patients who had vascular circle can be corrected by surgery. The malformations can be detected by ultrasound, magnetic resonance imaging, and computered tomography. Chromosome examinations were abnormal in most cases. More knowledges about them are helpful to clinical practice.

关 键 词：右主动脉弓 降主动脉 心脏畸形 心外畸形 

分 类 号：R54[医药卫生—心血管疾病]