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

doi:10.37015/AUDT.2025.240019

Abstract

Abstract:

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

Methods: 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).

Results: 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.

Conclusions: 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.

Key words: Fetus; Portal vein; Shunt; Prenatal ultrasound

Liu Jianjun, Zhang Jun, Xue Yafang, Chen Ying, Qiu Mei, Guo Yanli. Ultrasonographic Characteristics and Outcomes of Fetal Umbilical-portal-systemic Venous Shunts: A Single-center Study. Advanced Ultrasound in Diagnosis and Therapy, 2025, 9(2): 181-188.

Figures/Tables 7

Figure 1

Table 1

Table 2

Figure 2

Table 3

Characteristics, pregnancy outcomes, and follow-up results of all HPSS cases"

Case	Maternal age (years)	Gestational age at diagnosis (weeks)	Type	Ductus venosus (DV)	Portal vein (PV)	Shunt position	Associated anomalies	Outcome	Time to shunt closure
1	27	34	IIIa	+	+	Left PV (LPV)-left hepatic vein (LHV)	Fetal growth restriction (FGR); Placental chorioangioma	Full-term birth (FTB)	8 months
2	37	36⁺³	IIIa	+	+	LPV-middle hepatic vein (MHV)	FGR; Cardiac enlargement; Enlarged hepatic artery (HA)	FTB	9 months
3	23	37	IIIa	+	+	LPV-MHV	FGR; Cardiac enlargement; Enlarged HA; Vein of Galen dilatation	FTB	7 months
4	24	26⁺⁴	IIIa	+	+	LPV-LHV	-	FTB	6 months
5	21	37⁺³	IIIa	-	+	LPV-MHV	FGR	FTB	18 months
6	29	32	IIIa	+	+	LPV-LHV	Cardiac enlargement	FTB	12 months
7	20	31	IIIa	-	+	Right portal vein (RPV)-right hepatic vein	FGR; Cardiac enlargement; Decreased middle cerebral artery pulse index	C-section at 37 weeks	Not applicable (NA)
8	42	25	IIIa	+	+	LPV-LHV	Placental chorioangioma; Large stomach; Polyhydramnios	C-section at 31 weeks	10 months
9	27	32	IIIa	+	+	LPV-LHV	Cardiac enlargement; Enlarged HA	FTB	6 months
10	30	24	IIIa	+	+	LPV-LHV	Enlarged HA	C-section at 35 weeks	40 days
11	37	33	IIIa	+	+	LPV-LHV	Bowel dilatation; Non-visible gallbladder	termination of pregnancy(TOP)	NA
12	22	27	IIIa	+	+	LPV-LHV	FGR; Cardiac enlargement; Micromelia; Reversed DV a-wave	TOP	NA
13	23	30	IIIa	+	+	RPV-MHV	Cardiac enlargement; Enlarged HA; Deepened DV a-wave	TOP	NA
14	38	23⁺⁶	IIIa	+	+	LPV-LHV	Cardiac enlargement; Placental chorioangioma; Polyhydramnios	TOP	NA
15	30	30	IIIa	+	+	LPV-LHV	FGR; Enlarged HA; Galen venous blood flow showed pulsatile changes; Jugular vein dilatation	TOP	NA
16	27	24⁺⁵	IIIa	+	+	LPV-MHV	FGR; Cardiac enlargement; The anterior horn of the right lateral ventricle communicates with CSP	TOP	NA
17	31	29⁺³	IIIb	+	-	umbilical vein (UV)-Inferior vena cava (IVC)	Cardiac enlargement; Tricuspid regurgitation; Enlarged HA; IVC and azygos vein (AzV) dilatation	TOP	NA
18	38	27	IIIb	+	-	UV-right atrium /AzV	Dextrocardia; Spinal deformities; Polyhydramnios	TOP	NA

Table 3

Figure 3

Figure 4

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