Analysis of prenatal sonographic characteristics and postnatal outcomes of absent pulmonary valve syndrome

Abstract

Objective To investigate the prenatal ultrasonographic features of absent pulmonary valve syndrome(APVS). Methods The ultrasonographic images, follow-up results and the other clinical data of 17 fetuses suffering from APVS were retrospectively analyzed. According to the difference of the pulmonary artery diameter, subjects were divided into pulmonary artery(PA) dilated group (14 cases) and non-dilated group (3 cases). The sonographic features of the two groups were analyzed and compared. Results All 17 fetuses had rudimentary or absent pulmonary valves, and stenosis of the pulmonary annulus. Moderate or severe regurgitation flowed through pulmonary artery and right ventricular outflow in diastole. PA dilated group might be combined with Tetralogy of Fallot, double outlet of right ventricle or right ventricular aneurysm, there were 85.7% (12/14) with absent ductus arteriosus. The forward flow velocity during systole through pulmonary annulus was significantly fast. PA non-dilated group could be accompanied by Ebstein′s anomaly or tricuspid atresia. Ductus arch was always present. The forward flow velocity during systole through pulmonary annulus was slow. Conclusions The fetal pulmonary artery diameter with APVS can dilate or not. Reverse flow during diastolic period, which rushes from arteriosus ductus to the right ventricular outflow tract, contributes to the prenatal diagnosis of non-dilated PA. Key words: Ultrasonography, prenatal; Absent pulmonary valve syndrome