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

Abstract

Background: Fetal pulmonary valve anomaly (PVA) can be detected during pregnancy, and is necessary to reconstruct the right ventricle-pulmonary artery circulation as soon as possible after birth. Currently, there are limited reports on prenatal consultation, integrated management during the perinatal period, and prognosis evaluation of fetal PVA especially in China. This study aims to investigate integrated management methods, and the prognosis of fetal PVA.

Methods: A retrospective analysis was conducted on the integrated perinatal management and prognosis of 30 fetal PVA cases at Peking University People's Hospital from January 2019 to March 2023.

Results: Among the 30 PVA fetuses, 6 (20.0%) had pulmonary atresia with intact ventricular septum (PA/IVS), and 24 (80%) had pulmonary stenosis (PS). Of the 6 PA/IVS fetuses, 5 (5/6) had no abnormalities detected via chromosome analysis, and 1 did not undergo amniocentesis. Four (4/6) PA/IVS patients were delivered by Caesarean section (CS) at the gestational week of (37.0-39.2) weeks and birth weight of (3,000-3,560) 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 3-7 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). One of them had single-nucleotide polymorphism microarray (SNP array) 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 3,288.8±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. PS was not detected in 4 cases (4/14) by echocardiography after birth. The positive predictive value of prenatal ultrasound diagnosis for mild PS was 71.4%.

Conclusions: For PVA fetuses, 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. If the newborns do not experience any other complications after birth, surgery can achieve a good prognosis.

Keywords: Pulmonary stenosis (PS); perinatal integrated management; prenatal diagnosis; pulmonary valve atresia; ultrasonography.

Figure 1

Prenatal screening, prenatal diagnosis and prognosis of PA/IVS. PA/IVS, pulmonary atresia with intact ventricular septum; NT, nuchal translucency; NIPT, non-invasive prenatal testing; ASD, atrial septal defect; CKA, chromosome karyotype analysis; SNP, singlenucleotide polymorphism; CS, cesarean section; TBV, transthoracic balloon (pulmonary) valvuloplasty; BT, Blalock-Taussig shunt; LDA, ligation of ductus arteriosus.

Figure 2

Prenatal screening, prenatal diagnosis and prognosis of PS. PS, pulmonary valve stenosis; MCDA, monochorionic diamniotic; DCDA, dichorionic diamniotic; IVF-ET, in vitro fertilization-embryo transfer; NT, nuchal translucency; NIPT, non-invasive prenatal testing; VSD, ventricular septal defect; CKA, chromosome karyotype analysis; SNP, single-nucleotide polymorphism; CS, cesarean section.