胎儿心脏超声三血管气管切面呈“卜”字征的病因分类剖析

Etiological exploration and identification of character“卜”sign in fetal three-vessel and trachea view

目的探讨胎儿心脏超声三血管气管(3VT)切面呈“卜”字征的病因鉴别诊断流程和鉴别要点。方法回顾性分析2020年8月至2023年10月于南京医科大学附属苏州医院行胎儿心脏超声检查过程中3VT切面呈“卜”字征的37例胎儿的超声图像,总结形成“卜”字征的疾病类型,归纳鉴别诊断流程图和鉴别要点。所有病例的诊断均由2位经验丰富的高年资产前超声医师独立完成。结果“卜”字征的发生率为0.14%(37/27019)。37例3VT切面呈“卜”字征的胎儿中有13例(35.14%)完全性大动脉转位(TGA)、11例(29.73%)大动脉关系异常的右心室双出口(DORV/AA)、7例(18.92%)共同动脉干(TA,van Praagh A1、A2和A3型)和6例(16.22%)室间隔缺损型肺动脉闭锁(PA/VSD)。鉴别诊断过程中,可重点分析主动脉和肺动脉存在与否、心室-大动脉连接关系及双肺的血供来源3个方面,并根据流程图逐步推导诊断结果。TGA的鉴别要点是心室-大动脉连接不一致;DORV/AA可探及主动脉和肺动脉均完全或大部分起自右心室,两者失去正常螺旋关系;TA仅探及单一动脉干,肺动脉主干或分支起自动脉干;PA/VSD仅探及主动脉,肺动脉闭锁,双肺血供来源于动脉导管和/或体-肺侧支循环。结论胎儿心脏超声3VT切面呈“卜”字征的鉴别诊断内容包括TGA、DORV/AA、TA(van Praagh A1、A2和A3型)和PA/VSD。超声医师掌握鉴别诊断流程和鉴别要点有助于提高相关畸形的产前检出率和诊断能力。

Objective To explore the flow chart and key points of etiological differential diagnosis of character“卜”sign in fetal three-vessel and trachea(3VT)view.Methods A retrospective study was performed on ultrasound images of 37 fetuses,who had“卜”sign in 3VT view during fetal heart ultrasound scanning in The Affiliated Suzhou Hospital of Nanjing Medical University from Aug.2020 to Oct.2023.The types of diseases with“卜”sign and the flow chart and key points of differential diagnosis were summarized.All cases were diagnosed by 2 experienced senior prenatal ultrasound doctors independently.Results The incidence of“卜”sign was 0.14%(37/27019).Among the 37 fetuses with“卜”sign on 3VT view,13(35.14%)cases had complete transposition of great arteries(TGA),11(29.73%)had double outlet of right ventricle with abnormal relationship of great arteries(DORV/AA),7(18.92%)had truncus arteriosus(TA,van Praagh A1,A2 and A3),and 6(16.22%)had pulmonary atresia with ventricular septal defect(PA/VSD).During the process of differential diagnosis,the presence or absence of aorta and pulmonary arteries,the ventricle-artery connection relationship,and the blood supply to both lungs should be focused on,and the final diagnosis can be derived step by step according to the flow chart.The key points of differential diagnosis were as follows:the ventricle-artery connection was inconsistent in TGA;in DORV/AA both aorta and pulmonary artery were entirely or mostly from right ventricle without normal spiral relationship;in TA only a common artery trunk was detected,and main pulmonary artery or its branches were from the artery trunk;and in PA/VSD pulmonary artery was atresia and only aorta was detected,and the blood supply to both lungs was from ductus arteriosus and/or aortopulmonary collateral arteries.Conclusion The fetal differential diseases with“卜”sign in 3VT view include TGA,DORV/AA,TA(van Praagh A1,A2 and A3),and PA/VSD.Understanding the flow chart and key points of differential diagnosis can improve the prenatal detection rate and diagnosis of related malformations.