Objective: To investigate the relationship between congenital umbilical–portal–systemic venous shunt (UPSVs) and fetal outcome.
Methods: The ultrasonographic and genetic characteristics of 11 cases of UPSVS were retrospectively analyzed and followed up to postnatal.
Results: Four cases of ductus venosus–systemic shunt (DVSS), one case of extrahepatic portal–systemic shunt (EHPSS), one case of umbilical systemic shunt (USS) combined with intrahepatic portal–systemic shunt (IHPSS), and six cases of intrahepatic portal–systemic shunt (IHPSS) were observed. Chromosomal abnormalities were observed in 9.1% (1/11), other ultrasonic abnormalities in 54.5% (6/11), an increased cardiothoracic ratio in 45.5% (5/11), fetal growth restriction in 36.4% (4/11), edema in 9.1% (1/11), and live birth in 72.7% (8/11).
Conclusion: The incidence of IHPSS is the highest and the outcome is the best. Shunts in DVSS and IHPSS can close spontaneously after birth. When the prenatal diagnosis is congenital UPSVs, further examination for chromosomal abnormalities and other ultrasonic abnormalities should be performed, and the growth and development of the fetus should be closely monitored.