单侧肺动脉缺如的产前超声心动图诊断

Prenatal diagnosis of unilateral absence of pulmonary artery by echocardiography

目的探讨单侧肺动脉缺如(UAPA)的产前超声心动图特征.方法回顾性分析总结2012年6月至2018年10月期间经我院产前诊断的6例UAPA胎儿及3例在外院产检、出生后我院诊断为UAPA患儿的超声表现,总结该病胎儿期的超声心动图特征.结果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).UAPA胎儿超声心动图特征为:①孤立性UAPA心内结构正常;②多切面显示正常的肺动脉主干分叉处"八"字型结构消失,正常的肺动脉分支内径增宽;③在三血管气管切面(3VT)的基础上上下移动做横切面扫查,二维及彩色多普勒显像(CDFI)可显示发自主动脉弓的粗大血管(即头臂干动脉),其具有双重血管特征,无法区分是肺动脉分支还是主动脉弓分支;④沿粗大血管做冠状切面扫查,二维及CDFI可显示发自主动脉弓的粗大血管为头臂干动脉,并可清楚显示向肺内走形的肺动脉分支通过同侧垂直走行的动脉导管(DA)与头臂干动脉根部相连,而胎儿左肺动脉近端缺如合并TOF时,其左肺动脉远端则通过DA连接于主动脉弓的腹侧;⑤频谱多普勒显示向肺内走形的血管为肺动脉血流频谱,其血流孤立,与主肺动脉缺乏确切连接.结论超声心动图可对胎儿期UAPA做出准确的诊断,头臂干动脉冠状切面对诊断本病至关重要,早期诊断对产前咨询及预后评估起到至关重要的作用.

Objective To analyze the echocardiography features of unilateral absence of pulmonary artery (UAPA) features and improve the prenatal diagnosis accuracy of UAPA by analyzing fetal echocardiography features. Methods Fetal echocardiographic features were analyzed retrospectively in 6 cases with prenatal diagnosis of UAPA between June 2012 and October 2018. At the same time, ultrasonic manifestations of 3 patients whose antenatal examinations were performed in other hospitals and diagnosed as UAPA in our hospital were retrospectively analyzed. The fetal echocardiography characteristics of UAPA were summarized. Results Among the 6 fetuses with UAPA, 4 cases were diagnosed as isolated unilateral absence of proximal right pulmonary artery, 1 was unilateral absence of proximal left pulmonary artery with tetralogy of Fallot (TOF) and 1 was isolated unilateral absence of proximal left pulmonary artery. Characteristic sonographical findings included:①When other intracardiac abnormalities were absent, the inner diameter and proportion of the heart cavity was normal.②Multi-view showed that the normal confluence structure disappeared at the branch of the main pulmonary artery, and the diameter of normal pulmonary artery branch was widened.③On the basis of three vessels and trachea view (3VT), moved the probe up and down to make a transverse scan, two-dimensional and color Doppler flow imaging (CDFI) showed a large vessel (the brachiocephalic trunk artery) originating from the aortic arch which had dual vascular features, it means that it was difficult to distinguish the pulmonary artery branch from the aortic branch.④On coronal view of the large vessel, two-dimensional and CDFI clearly showed that the large vessel originating from the aortic arch was the brachiocephalic trunk artery, and the pulmonary artery branch connected with the brachiocephalic trunk artery by the ipsilateral vertical arterial duct (DA) which originated from the root of brachiocephalic trunk artery, but the left pulmonary artery branch connected with the ventral side of the aortic arch by the left DA in UAPA fetus with TOF.⑤Spectrum Doppler of the anomalous vessels of the lungs confirmed that it was the pulmonary artery, and the blood flow of the anomalous PA branch was isolated and no exact connection with the main pulmonary artery. Conclusions UAPA can be accurately diagnosed prenatally by echocardiography. The brachiocephalic trunk artery coronary view is very important for the diagnosis of this disease.Early diagnosis plays an important role in prenatal counseling and prognosis assessment.