Ultrasonographic characteristics and outcome of 40 cases of fetal umbilical-portal-systemic venous shunts: a single-center study

Background: To investigate the ultrasonographic characteristics and outcome of fetal umbilical-portal-systemic venous shunt (UPSVS). Methods The study studied cases of UPSVS retrospectively at an single center from January 2015 to December 2021. The ultrasonographic features, types and postnatal outcomes of fetal UPSVS were retrospectively analyzed and the outcomes were followed up. Results A total of forty cases with UPSVS including 4 Type I, 18 Type II, 18 cases of Type III (16 cases of IIIa, 2 cases of IIIb ) were identified. Two cases of Type I were terminated due to complete absence of the portal venous (PV) system. Genetic assessment revealed one case of Type I had deletion of fragment. Type II were mainly complicated by structural malformations, particularly cardiovascular abnormalities. And the parents opted for pregnancy termination in 4 cases. The most common associated anomalies was cardiac enlargement, followed by fetal growth restriction in Type III shunts. Termination of pregnancy occurred in 6 cases of Type IIIa as requested by the couples, 1 case of Type IIIa resulted from premature and 2 cases of Type IIIb due to the completely absent PV. Genetic tests showed trisomy X had microduplication in one case. The shunt was spontaneous closed after birth in all Type IIIa cases. Conclusions Prenatal diagnosis of UPSVS by ultrasonography is feasible, which is valuable for perinatal management and prenatal consultation. Fetal postnatal prognosis is determined by the presence of anomalies in the PV system, genetics or structure.