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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), and one case of umbilical systemic shunt (USS) combined with intrahepatic portal-systemic shunt (IHPSS), 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), cardiothoracic ratio increase in 45.5% (5/11), fetal growth restriction in 36.4% (4/11), edema was in 9.1% (1/11) and live birth was in 72.7% (8/11).    Conclusion:  The incidence of IHPSS is the highest and the outcome is the best. Shunt of DVSS and IHPSS can be closed spontaneously after birth. When the prenatal diagnosis is congenital UPSVs, chromosomal abnormalities and other ultrasonic abnormalities should be required further examination, and the growth and development of the fetus should be closely monitored.

The relationship between umbilical–portal–systemic venous shunt and outcome in 11 fetuses