Perinatal outcomes in cases of umbilical-portal-systemic venous shunts: experience of a tertiary center

BackgroundThe umbilical-ductal-portal system attracts significant interest due to its specific connections in fetal life. Advances in the detailed examination of hepatic circulation have further intensified this interest in recent years. Present study aimed to evaluate the obstetric and perinatal outcomes of patients diagnosed with umbilical-portal-systemic venous shunts (UPSVS) at our clinic.MethodsThis study involved a retrospective analysis covering the period from September 2019 to June 2024. Detailed anatomical screening, fetal echocardiography, and invasive genetic testing (for patients who consent) were performed on cases of fetal UPSVS. Accompanying anomalies, ductus venosus, the intrahepatic portal system, and hepatic veins were examined in detail.ResultsDuring the specified period, 20 patients were identified to have UPSVS, with a mean gestational age of 25 weeks at the time of diagnosis. Of these cases, five (25%) were classified as umbilical-systemic shunt (USS), three (15%) as ductus venosus-systemic shunt (DVSS), twelve (60%) as intrahepatic portal-systemic shunt (IHPSS). Ductus venosus was absent in 70% (n = 14) of the cases. Chromosomal analysis was conducted on 11 fetuses either prenatally or postnatally. Trisomy 21 was detected in two fetuses, 45,X0 in one fetus, a variant of Adams-Oliver syndrome in one fetus, and Adams-Oliver syndrome in another. Pregnancy was terminated in three cases due to chromosomal or additional congenital anomalies. In one case, intrauterine fetal death occurred at 22 weeks of gestation. Sixteen live births were recorded, of which two neonates died due to accompanying congenital anomalies, one at 2 h postnatally and the other on the 10 th postnatal day.ConclusionUPSVS are increasingly detected in the presence of major structural or chromosomal anomalies. The prognosis of isolated cases is generally favorable; however, the exact incidence remains unknown.