单侧肺动脉缺如及单侧肺动脉异常起源于升主动脉的产前超声心动图诊断

Prenatal diagnosis of anomalous origin of one pulmonary artery branch from the aorta and unilateral absence of pulmonary artery by echocardiography

目的通过对比分析单侧肺动脉缺如(UAPA)及单侧肺动脉异常起源于升主动脉(AOPA)的产前超声心动图特征,提高产前对两种疾病的诊断及鉴别诊断能力。方法回顾性总结2012年6月至2018年12月期间经我院产前诊断的AOPA及UAPA胎儿的超声心动图特点,总结其超声心动图特征及鉴别要点。结果共诊断6例AOPA和6例UAPA胎儿;6例AOPA胎儿中5例为右肺动脉异常起源于升主动脉(AORPA)(其中3例为孤立性AORPA,2例合并Berry综合征),1例为左肺动脉异常起源于升主动脉(AOLPA)合并先天性肺动脉瓣缺如征;6例AOPA病例均为近端型;6例UAPA胎儿中,4例为孤立性右肺动脉近端缺如,1例为孤立性左肺动脉近端缺如,1例为左肺动脉近端缺如合并法洛四联症(TOF)。主要的共同的超声心动图特征:多切面显示正常的肺动脉主干分叉处"八"字型结构消失,受累肺动脉分支未与主肺动脉相连;不同的超声心动图特征:三血管气管切面(3VT)及左室长轴切面显示AOPA受累肺动脉发自升主动脉近端右后或左后,3VT及头臂干动脉根部的冠状切面显示UAPA受累的肺动脉分支通过同侧动脉导管(DA)垂直走形,连接于头臂干动脉根部,彩色多普勒血流显像显示受累肺动脉分支在肺野内血流孤立。结论AOPA及UAPA的胎儿期超声心动图特征有很多相似之处;3VT及左室长轴切面是显示近端型AOPA的最佳切面,3VT及头臂干动脉根部的冠状切面是显示UAPA的最佳切面。

Objective To improve the prenatal diagnosis and differential diagnosis ability for anomalous origin of one pulmonary artery branch from the aorta (AOPA) and unilateral absence of pulmonary artery (UAPA) by comparing and analyzing different types of fetal echocardiography features. Methods Fetal echocardiographic features were analyzed retrospectively in fetus with prenatal diagnosis of UAPA or AOPA between June 2012 and December 2018, and the different types of fetal echocardiography characteristics and main points of identification were summarized. Results A total of 6 fetuses with AOPA and 6 fetuses with UAPA were diagnosed.Among the 6 fetuses with AOPA, 5 cases were anomalous origin of right pulmonary artery from the aorta (AORPA), including 3 cases with isolated AORPA and 2 cases combined with Berry syndrome, and 1 case was anomalous origin of left pulmonary artery from the aorta (AOLPA) with an absent pulmonary valve. All 6 cases of AOPA had proximal form. Among the 6 fetuses with UAPA, 4 cases were isolated unilateral absence of right pulmonary artery, 1 case was isolated unilateral absence of left pulmonary artery and 1 case was unilateral absence of left pulmonary artery combined with tetralogy of Fallot (TOF). The main common characteristic sonographical findings included: there was no confluence at the bifurcation of the main pulmonary artery on multiple views, and the affected pulmonary branch was not attached to the main pulmonary artery. The different characteristic sonographical findings in fetus included: the affected pulmonary artery of AOPA arosed from the right posterior or left posterior portion of the ascending aorta on three vessels and trachea view (3VT) and long axial view of left ventricle, and 3VT and the coronary view of brachiocephalic trunk artery showed that the abnormal origin of pulmonary artery branch of UAPA connected with the root of brachiocephalic trunk artery by the ipsilateral vertical arterial duct (DA), and color Doppler flow imaging showed that the blood flow of the affected pulmonary artery branch was isolated in the pulmonary field. Conclusions AOPA and UAPA have many similarities in the characteristics of fetal echocardiography. 3VT and long axial view of left ventricle are the best views for displaying proximal AOPA, 3VT and the coronary views of the root of brachiocephalic trunk artery are the best views for displaying UAPA.