Integrated management and prognosis analysis of 30 cases of fetal pulmonary valve abnormalities during pregnancy and perinatal period

Abstract

Abstract Objective This study aims to investigate prenatal testing, integrated management methods, and the prognosis of fetal pulmonary valve abnormalities (PVA) detected by ultrasound during pregnancy. Methods A retrospective analysis was conducted on the integrated perinatal management and prognosis of 30 fetal PVA cases identified during pregnancy by ultrasound at Peking University People's Hospital from January 2019 to March 2023. Results The incidence of fetal PVA diagnosed by prenatal ultrasound during the study period was 0.25% (30/11,923). Among the 30 PVA fetuses, 6 (20.0%) had pulmonary valve atresia with intact ventricular septum (PA/IVS), and 24 (80%) had pulmonary valve stenosis (PS). Of the 6 PA/IVS fetuses, 5 (5/6) had no abnormalities detected via prenatal diagnosis, and 1 did not undergo prenatal diagnosis. Four (4/6) PA/IVS patients were delivered by Caesarean section (CS) at an average gestational week of (38.1 ± 1.2) weeks and an average birth weight of (3172.5 ± 265.5) g. All of them received alprostadil intravenous pumping (6.00~13.00 ng/min/kg) after birth, followed by transthoracic balloon (pulmonary) valvuloplasty(TBV) + modified Blalock-Taussig shunt (BT) + ligation of Ductus arteriosus within an average of (4.5 ± 1.7) days. All patients recovered well after follow-up. Among the 24 patients with PS, 4 had severe PS (4/24), 20 had mild PS (20/24), and 1 had SNP abnormalities (1/24). Of the 24 patients, 7 (7/24) opted for pregnancy termination. Among the 17 (17/24) PS patients who delivered, 7 (7/17) had spontaneous labor, 1 (1/17) had forceps, and 9 (9/17) had CS. The average gestational week of delivery was (37.8 ± 1.0) weeks, and the average birth weight of newborns was (3288.8 ± 404.6) g. Three (3/17) severe PS neonates underwent TBV+ modified BT + ligation of Ductus arteriosus within 7 days after birth and recovered well after follow-up. Among 14 mild PS patients (14/17), 1 died within 1 week after birth (1/14). Two cases (2/14) underwent surgical treatment and recovered well. Seven cases (7/14) diagnosed with fetal mild PS did not require surgical treatment after birth. Four cases (4/14) did not detect PS by echocardiography after birth. The positive predictive value of prenatal ultrasound diagnosis for mild PS is 71.4%. Conclusion For PVA fetuses identified by ultrasound during pregnancy, it is recommended to conduct chromosomal karyotype analysis and SNP-array, and make an individualized evaluation and management based on the condition of fetal PVA and related abnormalities. The mode of delivery can be selected according to the obstetric situation. When necessary, newborns should be administered alprostadil to keep the ductus arteriosus open and be timely transferred to pediatric cardiac surgery for surgery. If the newborns do not experience any other complications after birth, surgery can achieve a good prognosis.