先天性主动脉弓及其分支畸形产前超声诊断及围产期结局

Prenatal sonographic diagnosis of fetal anomalies for arch branches

目的研究主动脉弓及其分支畸形的产前超声诊断方法及声像图特征,以提高其产前超声符合率,并回顾性分析其围产期结局。方法对我院诊断的184例先天性主动脉弓及其分支畸形胎儿主动脉弓及其分支畸形的不同类型及其是否合并心内外畸形与围产期结局的关系等临床及超声图像资料进行回顾性分析,并对各种不同类型主动脉弓及其分支畸形的产前超声特征进行总结分析。结果 5年间我院产前诊断的先天性主动脉弓及其分支畸形发生率为0.20%(184/94321),其中主动脉弓缩窄32.07%(59/184);主动脉弓离断6.52%(12/184);右位主动脉弓、左位动脉导管12.50%(23/184);右位主动脉弓、右位动脉导管3.26%(6/184);永存动脉干并右位动脉弓2.72%(5/184);双主动脉弓1.63%(3/184);右位主动脉弓并迷走左锁骨下动脉、左位动脉导脉22.28%(41/184);左位主动脉弓并迷走右锁骨下动脉、左位动脉导管占17.39%(32/184);绕食管后主动脉弓1.63%(3/184)。55例(29.89%,55/184)胎儿经病理解剖及手术证实诊断正确,误诊6例,诊断符合率为89.09%(49/55)。65例行产前染色体核型分析,10例(15.38%,10/65)为非整倍体。单发主动脉弓及分支畸形围产期预后较好,合并其他心内外畸形其临床预后较差。结论先天性主动脉弓及其分支畸形各类型均有特征性的产前超声诊断图像,掌握主动脉弓及其分支正常与异常产前超声图像特征,可有效发现异常并且鉴别诊断各种类型主动脉弓畸形。三血管气管切面是诊断主动脉弓畸形的敏感且有效切面,其他切面是诊断与鉴别诊断的重要辅助切面。围产期结局与主动脉弓及其分支畸形的类型以及是否合并心内外严重畸形有关。

Objective To improve prenatal detection rate of kinds of aortic arch anomalies by reviewing prenatal sonogram features. Methods A total of 184 reuses with congenital aortic arch and branches anomalies were retrospectively reviewed. Their prenatal and postnatal medical records, including the following data were analyzed ： （ 1 ） Type of aortic arch anomalies. （ 2 ） Anomalies of the aorta and pulmonary artery. （ 3 ） Additional cardiac anomalies. （4） Extraeardiac anomalies. （ 5 ） Outcome. Results The overall incidence of fetal anomalies for aortic arch and branches was 0.20% （ 184/94 321 ） , including coarctation of the aorta 32.07% （59/184） , interruption of aortic arch 6.52% （12/184） , right aortic arch with left ductus arteriosus 12.50% （23/184）, right aortic arch with right ductus arteriosus 3.26% （6/184）, right aortic arch with aberrant left subelavian artery and left duetus arteriosus 22.28% （41/184） ; left aortic arch with aberrant right subclavian artery and left duetus arteriosus 17.39% （32/184） , aortic arch after esophagus 1.63% （3/184）. Fifty five fetuses （29. 89%, 55/184） were confirmed by pathological anatomy and operation, 6 misdiagnosised, yielding the coincidence rate 89.09% （49/55）. Chromosomal analysis was performed prenatally in 65 pregnancies, and 10 cases （ 15.38% , 10/65 ）of fetal aneuploidy were identified. The prognosis of isolate congenital aortic arch and branches anomalies was better than the others. Conclusions All kinds of congenital aortic arch and branches anomalies have ultrasonographie features Based on these features, it is possible to diagnose and classify these anomalies prenatally. The three-vessel and trachea view is sensitive and effective for diagnosing congenital aortic arch and branches anomalies.Prenatal outcome of these fetuses is depend on the type of aortic arch and branches anomalies and whether combined with other cardiac or extra-cardic malformations.