探讨法洛四联症合并单侧肺动脉缺如胎儿的超声心动图特征。

回顾性选取2017年10月至2020年12月在河北生殖妇产医院进行胎儿超声心动图检查，诊断为法洛四联症的胎儿177例，其中合并单侧肺动脉缺如4例。分析4例胎儿的超声心动图资料。

177例法洛四联症胎儿中，4例合并单侧肺动脉缺如，单侧肺动脉缺如在法洛四联症胎儿中的发生率为2.26%（4/177）。3例左肺动脉缺如，1例右肺动脉缺如。4例胎儿均有典型法洛四联症超声心动图表现：主动脉瓣下室间隔缺损、肺动脉狭窄、主动脉骑跨；单侧肺动脉缺如表现为肺动脉分叉切面显示“八”字型结构消失。3例左肺动脉缺如胎儿，肺动脉分叉切面左肺动脉近端无法探及，主动脉弓冠状切面显示左肺动脉远端通过动脉导管连接于主动脉弓腹侧，左肺动脉远端由动脉导管逆向灌注供血；1例合并右肺动脉缺如胎儿，肺动脉分叉切面显示右肺动脉近端无法探及，远端分支起源于降主动脉，由体肺侧支供血。1例法洛四联症合并左肺动脉缺如胎儿，另外合并残存左上腔、房间隔缺损；法洛四联症合并右肺动脉缺如胎儿，另外合并右位主动脉弓伴镜像分支。

掌握胎儿期法洛四联症合并单侧肺动脉缺如的超声心动图特征，对于提高该病检出率、为孕妇家庭决策提供科学依据具有重要意义。

To explore prenatal echocardiographic features of tetralogy of Fallot (TOF) with unilateral absence of the pulmonary artery (UAPA).

Fetal echocardiography was performed in 177 fetuses with tetralogy of Fallot in Hebei Maternity Hospital from October 2017 to December 2020, including four cases with UAPA. The echocardiographic data of four fetuses with TOF and UAPA were analyzed retrospectively.

The incidence of UAPA in fetuses with TOF was 2.26% (4/177). The left pulmonary artery was absent in three cases and the right pulmonary artery was absent in one case. All of the four fetuses with TOF and UAPA have typical echocardiographic manifestations of TOF including subaortic ventricular septal defect, pulmonary stenosis, and aorta overriding. The sonographic feature of UAPA in the four fetuses was the disappearance of '8-figure' structure on the pulmonary artery bifurcation view. The proximal left pulmonary arteries cannot be detected in three cases with unilateral absence of the left pulmonary artery on this view. On coronal view of the aortic arch, the left pulmonary artery branches were showed to be connected with the ventral side of the aortic arch by the ductus arteriosus in three cases. The proximal right pulmonary arteries cannot be detected in one case with unilateral absence of the right pulmonary artery on pulmonary artery bifurcation view and the right pulmonary artery branches were showed to be connected with the descending aorta. One case with unilateral absence of the left pulmonary artery had persistent left superior vena cava and atrial septal defect additionally. The case with unilateral absence of the right pulmonary artery had a right aortic arch with mirrored branches additionally.

It is of great significance to understander the prenatal echocardiographic features of TOF with UAPA in order to improve the detection rate of this disease and provide a scientific basis for families to make decisions on pregnancy.