To investigate the echocardiographic features of fetal pulmonary artery atresia with ventricular septal defec(PA-VSD) during fetal period. Methods The pathomorphology of fetal specimens with PA-VSD were detected.Prenatal echocardiographic images were analysed retrospectively. Result：the ultrasonographic findings of PA-VSD of fetus were as follows:(1) direct signs: major pulmonary artery and its main branches dysplasia which were showed color flow reverse from ductus arteriosus to pulmonary artery with CDFI or pulmonary artery absent， ventricular septal defect（2）indirect signs:aorta dextroposition and overriding.major aortopulmonary collateral artery formed around descending aorta.Conclusion There is higher accuracy of prenatal echocardiography in diagnosing fetal PA-VSD.