先天性原发隔异位的多普勒超声心动图诊断

Diagnosis of congenital malposition of septum primum by Doppler echocardiography

目的分析先天性原发隔异位(malposition of septum primum,MSP)的多普勒超声心动图特征,评价多普勒超声心动图对MSP的诊断价值,分析超声心动图漏误诊的原因,提高超声心动图对MSP诊断的准确性。方法回顾性分析2009年1月至2019年10月在上海交通大学医学院附属新华医院及上海交通大学医学院附属上海儿童医学中心8例经CTA、心外科手术或尸体解剖诊断为MSP患儿的超声心动图检查资料。结果超声心动图显示肺静脉开口于解剖左房后壁,继发隔上边缘带缺如,原发隔向左偏移,肺静脉异常引流入原发隔右侧的心房。本组7例合并完全性肺静脉异位引流,1例合并部分性肺静脉异位引流。8例患儿中超声诊断正确6例;超声误诊2例,其中1例误诊为单心房,1例误诊为三房心。结论超声心动图技术可以准确地诊断MSP,但需与单心房、三房心等畸形鉴别。

Objective To review the imaging characteristics and evaluate the diagnostic value of Doppler echocardiography for congenitally malposition of septum primum(MSP).Methods Images of Doppler echocardiography were retrospectirely rewiewed and compared with CTA,operative and necropsy findings in 8 cases with MSP in Xinhua Hospital,and Shanghai Children′s Medical Center,Shanghai Jiaotong University School of Medicine from January 2009 to October 2019.Results MSP was characterized by the absent of superior limbic band of septum secundum and different degrees of the leftward deviation of septum primum,and the pulmonary veins which connected with the posterior wall of the anatomical left atrium incorporated intothe right atrium.The associated malformations included totally anomalous(7 cases)and partially anomalous(1 case)pulmonary venous drainage directly to the right atrium.Six of 8 cases were diagnosed correctly.In the remaining 2 cases,1 case misdiagnosed as single atrium,and the other case misdiagnosed as cor triatriatum.Conclusions MSP could be diagnosed accurately by Doppler echocardiography.This malformation should be distinguished from single atrium and cor triatriatum.