Ultrasonographic Characteristics and Outcomes of Fetal Umbilical-portal-systemic Venous Shunts: A Single-center Study

To investigate the ultrasonographic characteristics and outcomes of fetal umbilical-portal-systemic venous shunts (UPSVS).

UPSVS cases at a single center between January 2015 and December 2022 were retrospectively investigated. The ultrasonographic features, types, and postnatal outcomes of fetal UPSVS were analyzed and the outcomes were followed up on. The study was approved by our institutional review board (Approval Number KY2021130).

Forty UPSVS cases, including 4 Type Ⅰ, 18 Type Ⅱ, and 18 Type Ⅲ (16 with Ⅲa and 2 with Ⅲb) were identified. Two Type Ⅰ cases were terminated because of the complete absence of the portal venous (PV) system. Genetic assessment revealed that one patient with Type Ⅰ had a q22.11 deletion in trisomy 21. Type Ⅱ is characterized by structural malformations, particularly cardiovascular abnormalities. Couples opted for pregnancy termination in four cases of Type Ⅱ. The most common anomaly in Type Ⅲ shunts was cardiac enlargement, followed by fetal growth restriction. Pregnancy was terminated in six cases of Type Ⅲa as requested by the couples, one resulting from premature birth, and two because of the completely absent PV system. Genetic tests showed that trisomy X had a microduplication in one patient. The shunt spontaneously closed after birth in all Type Ⅲa cases.

Prenatal diagnosis of UPSVS using ultrasonography is feasible and valuable for perinatal management and prenatal consultation. The fetal postnatal prognosis is determined by the presence of anomalies in the PV system, genetics, or structure.