肺动脉闭锁伴室间隔缺损的产前超声诊断价值分析

Analysis of diagnostic value of pulmonary atresia with ventricular septal defect by prenatal echocardiography

目的评价超声心动图对肺动脉闭锁伴室间隔缺损(PA/VSD)的产前诊断价值,分析超声心动图误诊的原因,旨在提高超声心动图对PA/VSD的产前检出率。方法回顾性分析48例胎儿经MRI或产后超声诊断为PA/VSD的产前超声诊断资料。结果48例PA/VSD胎儿超声心动图诊断正确40例,占83.3%;产前超声漏误诊8例,占16.7%;其中2例误诊为永存动脉干,2例误诊为法洛四联症,2例误诊为单心房、单心室、肺动脉狭窄,1例误诊为右室双出口,1例漏诊粗大侧枝血管及右冠状动脉右室瘘。PA/VSD最常见的超声征象为肺动脉内径细小(43例)或不能显示(5例)、动脉导管内逆向血流(42例)、主动脉骑跨(22例),最常见的合并畸形有完全性房室间隔缺损17例(35.4%)、单心房14例(29.2%)、右位主动脉弓13例(27.1%)、单心室9例(18.8%)、右室双出口9例(18.8%)、左侧上腔静脉残存8例(16.7%)、双侧右房结构7例(14.6%)等。结论联合应用四腔心切面、心室流出道切面及三血管切面,可较为准确地诊断胎儿PA/VSD,但需要与法洛四联症及永存动脉干等畸形鉴别。

Objective To evaluate the diagnostic value of pulmonary atresia with ventricular septal defect （PA /VSD） by prenatal echocardiography ,to analyze the reasons of misdiagnosed ,and to improve the initial echocardiographic diagnosis . Methods A retrospective echocardiography review of 48 fetuses with PA/VSD confirmed by prenatal magnetic resonance imaging （MRI） or postnatal echocardiography was conducted .Results Among 48 PA /VSD fetuses ,40 cases （83 .3% ） were diagnosed correctly by prenatal echocardiography ,in the remaining 8 cases with PA /VSD ,common truncus arterio‐sus were found in 2 cases ,tetralogy of Fallot in 2 cases ,single atrium ,single ventricle and pulmonary valve stenosis in 2 cases ,double outlet of right ventricle in 1 case were misdiagnosed by prenatal echocardiography ,major aortopulmonary collateral arteries and a coronary arteriovenous fistula between the right coronary artery and the right ventricle in 1 case were miss diagnosed .More common findings of PA/VSD were small pulmonary diameter （43 cases） and small pulmonary artery which could not be shown by two dimensional echocardiography （5 cases） ,reversed flow through ductus arteriosus by color Doppler flow imaging （42 cases） ,and overriding aorta （22 cases） .Other associated anomalies were atrioventricu‐lar septal defect in 17 cases （35 .4% ） ,single atrium in 14 cases （29 .2% ） ,right‐side aortic arch in 13 cases （27 .1% ） ,sin‐gle ventricle in 9 cases （18.8% ） ,double outlet of right ventricle in 9 cases （18 .8% ） ,persistent left superior vana cava in 8 cases （16 .7% ） ,right isomerism in 7 cases （14 .6% ） .Conclusion Combining visualization of PA /VSD in four‐chamber view ,outflow tract view ,and three vessels view ,PA/VSD could be accurately diagnosed by prenatal echocardiography , but it should be differentiated from common truncus arteriosus and tetralogy of Fallot .