Sonographic Features and Perinatal Outcomes in Fetuses with Ductus Venosus Agenesis: Single Center Experience

Abstract

Objective: The purpose of this study is to evaluate the prenatal sonographic features and postnatal outcomes in fetuses with ductus venosus agenesis (DVA) in a tertiary center. Material and Methods: We performed a retrospective study of 15 consecutive cases of DVA diagnosed in our perinatology department between January 2020 and October 2023. All clinic records, fetal echocardiograms, any accompanying anomalies, obstetrical ultrasounds, and postnatal echocardiograms were reviewed. Results: Of the 15 cases detected, umbilical vein had extrahepatic type connection in 8 fetuses (53.3%) and intrahepatic type connection in 7 fetuses (46.7%). 11 patients had associated anomalies including hydrops (n=3, 20%), cardiac (n=6, 40%), extracardiac structural (n=7, 46.7%) and chromosomal anomalies (n=3, 20%). In our patient group, only 4 cases (26.7%) presented with isolated DVA, of which 3 had intrahepatic type connection. Prenatal genetic testing including karyotype and microarray was performed in 8 patients (53.3%) and 3 (20%) of them had abnormal results. 4 women (26.7%) underwent legal termination of pregnancy. There were 2 (13.3%) neonatal deaths, and the remaining 9 cases (60%) were alive at last follow-up. Conclusion: DVA is associated with cardiac, extracardiac, and genetic anomalies independent of the site of umbilical venous connection. Postnatal outcomes in cases with DVA depend on the presence of additional anomalies. Fetuses with DVA and extrahepatic connection have additional risk for cardiac failure, hydrops and portal venous system agenesis which worsen the outcomes. DVA cases with intrahepatic connection associated with no or minor anomaly tend to have more favorable outcomes.

Keywords

• Ductus venosus agenesis
• portosystemic shunt
• intrahepatic drainage
• extrahepatic drainage
• hydrops

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