Original Research

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

  • Liu Jianjun ,
  • Zhang Jun ,
  • Xue Yafang ,
  • Chen Ying ,
  • Qiu Mei ,
  • Guo Yanli
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  • Department of Ultrasound, Southwest Hospital, Army Military Medical University (Third Military Medical University), Chongqing, China
First author contact:

1Jianjun Liu and Jun Zhang contributed equally to this study.

Department of Ultrasound, Southwest Hospital, Army Military Medical University (Third Military Medical University), 30 Gaotanyan Street, Shapingba District, Chongqing, China. e-mail: guoyanli@tmmu.edu.cn

Received date: 2024-10-24

  Revised date: 2024-05-31

  Accepted date: 2024-11-01

  Online published: 2025-07-06

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.

Cite this article

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[J]. ADVANCED ULTRASOUND IN DIAGNOSIS AND THERAPY, 2025 , 9(2) : 181 -188 . DOI: 10.37015/AUDT.2025.240019

References

[1] Achiron R, Kivilevitch Z. Fetal umbilical-portal-systemic venous shunt: in-utero classification and clinical significance. Ultrasound Obstet Gynecol 2016;47:739-747.
[2] Chocarro G, Amesty MV, Encinas JL, Vilanova SA, Hernandez F, Andres AM, et al. Congenital portosystemic shunts: Clinic heterogeneity requires an individual management of the patient. Eur J Pediatr Surg 2016;26:74-80.
[3] Sokollik C, Bandsma RH, Gana JC, van den Heuvel M, Ling SC. Congenital portosystemic shunt: characterization of a multisystem disease. J Pediatr Gastroenterol Nutr 2013;56:675-681.
[4] Lu L, Yao L, Wei H, Hu J, Li D, Yin Y, et al. Ultrasonographic classification of 26 cases of fetal umbilical-portal-systemic venous shunts and the correlations with fetal chromosomal abnormalities. BMC Pregnancy Childbirth 2023;23:236.
[5] Jimenez-Gomez J, Guizzo JR, Betancourth AJ, Santiago MS, Gaspar PM, Pina PS, et al. Correlation of prenatal and postnatal diagnosis in umbilical-portal-systemic venous shunts. Eur J Pediatr Surg 2023;33:90-95.
[6] Yagel S, Kivilevitch Z, Cohen SM, Valsky DV, Messing B, Shen O, et al. The fetal venous system, part I: normal embryology, anatomy, hemodynamics, ultrasound evaluation and Doppler investigation. Ultrasound Obstet Gynecol 2010;35:741-750.
[7] Abernethy J.Account of two instances of uncommon formation in the viscera of the human body: from the philosophical transactions of the royal society of London. Med Facts Obs 1797;7:100-108.
[8] Papamichail M, Pizanias M, Heaton N. Congenital portosystemic venous shunt. Eur J Pediatr Surg 2018;177:285-294.
[9] Dauvillée J, Ingargiola I, Jouret M, Biard JM, Steenhaut P, Bernard P.Fetal umbilical-systemic shunt with a positive issue. J Gynecol Obstet Hum Reprod 2020;49:101656.
[10] Wang Y, Yan Y, Yang Z, Wei J, Liu G, Pei Q. Prenatal diagnosis of congenital portosystemic shunt: A single-center study. J Obstet Gynaecol Res 2020;46:1988-1993.
[11] Stringer MD. The clinical anatomy of congenital portosystemic venous shunts. Clin Anat 2008;21:147-157.
[12] Zhu L, Wu H, Cong X, Li S, Li Q, Dong X, et al. Prenatal ultrasonographic features and follow-up outcomes of 19 cases of congenital intrahepatic portosystemic venous shunts diagnosed during the foetal period. Insights Imaging 2022;13:169.
[13] Steg Saban O, Weissbach T, Achiron R, Pekar Zlotin M, Haberman Y, Anis Heusler A, et al. Intrahepatic portosystemic shunts, from prenatal diagnosis to postnatal outcome: a retrospective study. Arch Dis Child 2023;108:910-915.
[14] Czeiger S, Weissbach T, Zloto K, Wiener A, Nir O, Massarwa A, et al. Umbilical-portal-systemic venous shunt and intrauterine growth restriction: an inquiry from a prospective study. Am J Obstet Gynecol 2024;231:340.e1-340.e16.
[15] Delle CL, Neuberger P, Von Kalle T. Congenital intrahepatic portosystemic shunt: prenatal diagnosis and possible influence on fetal growth. Ultrasound Obstet Gynecol 2008;32:233-235.
[16] Tchirikov M, Kertschanska S, Schroder HJ. Obstruction of ductus venosus stimulates cell proliferation in organs of fetal sheep. Placenta 2001;22:24-31.
[17] Wu H, Tao G, Cong X, Li Q, Zhang J, Ma Z, et al. Prenatal sonographic characteristics and postnatal outcomes of umbilical-portal-systemic venous shunts under the new in-utero classification: A retrospective study. Medicine (Baltimore) 2019;98:e14125.
[18] Golewale N, Paltiel HJ, Fishman SJ, Alomari AI. Portal vascular anomalies in Down syndrome: spectrum of clinical presentation and management approach. J Pediatr Surg 2010;45:1676-1681.
[19] Demirci O, Akay H?. Prenatal diagnosis of abnormality of the umbilical portal DV complex: difficulty in universal classification due to various alternative routes in hepatic circulation for placental drainage. J Matern Fetal Neonatal Med 2022;35:3872-3884.
[20] Nagy RD, Iliescu DG.Prenatal diagnosis and outcome of umbilical-portal-systemic venous shunts: Experience of a tertiary center and proposal for a new complex type. Diagnostics (Basel) 2022;12:873.
[21] Dong X, Wu H, Zhu L, Cong X, Li Q, Tang F, et al. Prenatal ultrasound analysis of umbilical-portal-systemic venous shunts concurrent with trisomy 21. J Ultrasound Med 2021;40:1307-1312.
[22] Han BH, Park SB, Song MJ, Lee KS, Lee YH, Ko SY, et al. Congenital portosystemic shunts: prenatal manifestations with postnatal confirmation and follow-up. J Ultrasound Med 2013;32:45-52.
[23] Zhu L, Wu H, Cong X, Ma Z, Tao G. Ultrasonographic characteristics and outcome of Type III umbilical-portal-systemic venous shunt. Med Ultrason 2022;24:14-18.
[24] Ponce-Dorrego MD, Hernandez-Cabrero T, Garzon-Moll G. Endovascular treatment of congenital portosystemic shunt: A single-center prospective study. Pediatr Gastroenterol Hepatol Nutr 2022;25:147-162.
[25] Pomeranz CB, Baad M, Kovanlikaya A, Collins LK. Imaging features, classification and clinical features of intrahepatic congenital portosystemic shunts. J Ultrasound 2025;28:227-238.
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