右肺动脉异常起源于升主动脉的超声心动图漏误诊分析

Quality analysis of echocardiography diagnosis of abnormal origin of the right pulmonary artery from the ascending aorta and measures to reduce misdiagnosis and missed diagnosis

目的分析右肺动脉异常起源于升主动脉(AORPA)的超声诊断质量和漏误诊原因,探讨提高AORPA超声诊断准确性的措施。方法选取2009年1月至2018年12月在北京儿童医院经手术证实为AORPA的患儿共16例,男性7例,女性9例,年龄5 d~12岁,中位年龄4个月。分析患儿临床表现、首诊及术前超声心动图表现、超声诊断分级等。结果AORPA患儿临床表现主要为青紫及反复呼吸道感染。其超声心动图直接征象表现为肺动脉分叉结构消失,右肺动脉起自升主动脉不同部位;最重要的间接征象表现为中度及以上的肺动脉高压。13例(81.3%,13/16)首诊超声心动图诊断结果分级为“符合”,3例(18.7%,3/16)诊断结果为“不符合”,其中2例(12.5%,2/16)诊断为粗大动脉动脉导管未闭,漏诊近端型AORPA,1例(6.3%,1/16)远端型AORPA误诊为右肺动脉缺如。16例(100%,16/16)术前超声心动图诊断结果分级均为“符合”。结论AORPA发病率极低,患儿临床表现无特异性,超声医师应熟悉其声像图特征,行超声心动图检查时发现中度及以上肺动脉高压时应注意是否存在AORPA,注意扫查肺动脉分叉、升主动脉及第一支头臂血管附近是否存在异常血管,提高诊断质量。

Objective To analyze the quality of echocardiography report and the causes of misdiagnosis of abnormal origin of the right pulmonary artery from the ascending aorta(AORPA),and to explore the measures to improve the echocardiography diagnosis accuracy for AORPA.Methods From January 2009 to December 2018,patients diagnosed with AORPA by surgery at Beijing Children's Hospital were reviewed.There were 16 cases of AORPA,including 7 boys and 9 girls,aged from 5 days to 12 years,with a median age of 4 months.The clinical manifestation and first and preoperative echocardiographic diagnosis were analyzed.Results The main manifestations were cyanosis and recurrent respiratory tract infection.The direct sign of echocardiography included disappearance of pulmonary artery bifurcation structure,and the right pulmonary artery arising from the aorta;while the indirect sign was moderate and above pulmonary hypertension.The first echocardiography diagnosis results of 13(81.3%)cases were classified as"consistent",and 3(18.7%)classified as"inconsistent",among which 2(17.6%)were diagnosed as patent ductus arteriosus with a missed diagnosis of proximal AORPA,and 1(6.3%)case of distal AORPA misdiagnosed as absence of the right pulmonary artery.The preoperative echocardiography diagnosis results of 16(100%)cases were classified as"consistent".Conclusion AORPA is rare,and its clinical manifestations in children are not specific.Ultrasound doctors should be familiar with the ultrasound features of AORPA.When finding moderate and above pulmonary hypertension,the possibility of AORPA should be considered,and the pulmonary artery bifurcation structure,the ascending aorta,and the first brachiocephalic vessel should be scanned.