Review

. 2023 Oct 20;6(1):100933.

doi: 10.1016/j.jhepr.2023.100933. eCollection 2024 Jan.

Expert management of congenital portosystemic shunts and their complications

Valérie Anne McLin^{1

2}, Stéphanie Franchi-Abella^{3

4

5

2

6}, Timothée Brütsch⁷, Atessa Bahadori⁸, Valeria Casotti^{9

6}, Jean de Ville de Goyet¹⁰, Grégoire Dumery^{3

11}, Emmanuel Gonzales^{3

12

13

2

6}, Florent Guérin^{3

14

2

6}, Sebastien Hascoet^{15

16}, Nigel Heaton¹⁷, Béatrice Kuhlmann¹⁸, Frédéric Lador¹⁹, Virginie Lambert^{20

4}, Paolo Marra²¹, Aurélie Plessier^{22

2

23}, Alberto Quaglia²⁴, Anne-Laure Rougemont²⁵, Laurent Savale^{3

26

27

28}, Moinak Sen Sarma²⁹, Olivier Sitbon^{3

26

27

28}, Riccardo Antonio Superina³⁰, Hajime Uchida³¹, Mirjam van Albada³², Hubert Petrus Johannes van der Doef³³, Valérie Vilgrain^{34

35

2

23}, Julie Wacker^{36

37}, Nitash Zwaveling³⁸, Dominique Debray^{39

40

2

6}, Barbara Elisabeth Wildhaber^{41

2}

Affiliations

¹ Swiss Pediatric Liver Center, Gastroenterology, Hepatology and Pediatric Nutrition Unit, Department of Pediatrics, Gynecology and Obstetrics, University of Geneva, Geneva, Switzerland.
² ERN RARE LIVER.
³ Université Paris-Saclay, Faculté de médecine, Le Kremlin-Bicêtre, France.
⁴ AP-HP, Centre de référence des maladies rares du foie de l'enfant, Service de radiologie pédiatrique diagnostique et interventionnelle, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
⁵ BIOMAPS UMR 9011 CNRS, INSERM, CEA, Orsay, France.
⁶ ERN Transplant Child.
⁷ Communication in Science, rue du Tunnel 7, Carouge, Switzerland.
⁸ Department of Pediatrics, Gynecology and Obstetrics, University of Geneva, Geneva, Switzerland.
⁹ Pediatric Hepatology, Gastroenterology and Transplant Centre, ASST Papa Giovanni XXIII Hospital, Bergamo, Italy.
¹⁰ Pediatric Department for the Treatment and Study of Abdominal Diseases and Abdominal Transplantation, ISMETT UPMC, Palermo, Italy.
¹¹ AP-HP, Service de gynécologie et d'obstétrique, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
¹² AP-HP, Centre de référence des maladies rares du foie de l'enfant, FHU Hepatinov, Service d'hépatologie et transplantation hépatique pédiatriques, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
¹³ INSERM UMRS_1193, Orsay, France.
¹⁴ AP-HP, Service de chirurgie pédiatrique, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
¹⁵ Department of Congenital Heart Diseases, Hôpital Marie Lannelongue, France.
¹⁶ INSERM UMR_S 999, Université Paris, France.
¹⁷ Institute of Liver Studies, Kings College Hospital, London, England.
¹⁸ Pediatric Endocrinology, Cantonal Hospital Aarau KSA, Aarau, Switzerland.
¹⁹ Service de Pneumologie, University of Geneva, Geneva, Switzerland.
²⁰ Cardiologie congénitale, Institut Mutualiste Montsouris, Paris, France.
²¹ Department of Radiology, Papa Giovanni XXIII Hospital, School of Medicine and Surgery - University of Milano-Bicocca, Bergamo, Italy.
²² Centre de référence des maladies vasculaires du foie, Service d'hépatologie Hôpital Beaujon, Clichy, France.
²³ VALDIG.
²⁴ Department of Cellular Pathology, Royal Free London NHS Foundation Trust/UCL Cancer Institute, London, England.
²⁵ Swiss Pediatric Liver Center, Division of Clinical Pathology, Diagnostic Department, University of Geneva, Geneva, Switzerland.
²⁶ AP-HP, Centre de référence de l'hypertension pulmonaire, Service de pneumologie et soins intensifs respiratoires, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
²⁷ INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France.
²⁸ ERN Lung.
²⁹ Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
³⁰ Northwestern University Feinberg School of Medicine, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois, USA.
³¹ Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan.
³² Department of paediatric and congenital cardiology, University Medical Center Groningen, University of Groningen, The Netherlands.
³³ Division of paediatric gastroenterology and hepatology, Department of paediatrics, University Medical Center Groningen, Groningen, The Netherlands.
³⁴ Université Paris Cité, CRI, INSERM, Paris, France.
³⁵ AP-HP, Département de Radiologie, Hôpital Beaujon. Nord, Clichy, France.
³⁶ Pediatric Cardiology Unit, Department of pediatrics, Gynecology and Obstetrics, University of Geneva, Geneva, Switzerland.
³⁷ Centre Universitaire Romand de Cardiologie et Chirurgie Cardiaque Pédiatrique, University of Geneva and Lausanne, Switzerland.
³⁸ Department of Pediatric Endocrinology, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, The Netherlands.
³⁹ AP-HP, Unité d'hépatologie pédiatrique et transplantation hépatique, Hôpital Necker, Paris, France.
⁴⁰ Centre de Référence des maladies rares du foie de l'enfant, FILFOIE, France.
⁴¹ Swiss pediatric Liver Center, Division of pediatric surgery, Department of Pediatrics, Gynecology, and Obstetrics, University of Geneva, Geneva, Switzerland.

PMID: 38234409
PMCID: PMC10792643
DOI: 10.1016/j.jhepr.2023.100933

Review

Expert management of congenital portosystemic shunts and their complications

Valérie Anne McLin et al. JHEP Rep. 2023.

. 2023 Oct 20;6(1):100933.

doi: 10.1016/j.jhepr.2023.100933. eCollection 2024 Jan.

Authors

Affiliations

¹ Swiss Pediatric Liver Center, Gastroenterology, Hepatology and Pediatric Nutrition Unit, Department of Pediatrics, Gynecology and Obstetrics, University of Geneva, Geneva, Switzerland.
² ERN RARE LIVER.
³ Université Paris-Saclay, Faculté de médecine, Le Kremlin-Bicêtre, France.
⁴ AP-HP, Centre de référence des maladies rares du foie de l'enfant, Service de radiologie pédiatrique diagnostique et interventionnelle, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
⁵ BIOMAPS UMR 9011 CNRS, INSERM, CEA, Orsay, France.
⁶ ERN Transplant Child.
⁷ Communication in Science, rue du Tunnel 7, Carouge, Switzerland.
⁸ Department of Pediatrics, Gynecology and Obstetrics, University of Geneva, Geneva, Switzerland.
⁹ Pediatric Hepatology, Gastroenterology and Transplant Centre, ASST Papa Giovanni XXIII Hospital, Bergamo, Italy.
¹⁰ Pediatric Department for the Treatment and Study of Abdominal Diseases and Abdominal Transplantation, ISMETT UPMC, Palermo, Italy.
¹¹ AP-HP, Service de gynécologie et d'obstétrique, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
¹² AP-HP, Centre de référence des maladies rares du foie de l'enfant, FHU Hepatinov, Service d'hépatologie et transplantation hépatique pédiatriques, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
¹³ INSERM UMRS_1193, Orsay, France.
¹⁴ AP-HP, Service de chirurgie pédiatrique, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
¹⁵ Department of Congenital Heart Diseases, Hôpital Marie Lannelongue, France.
¹⁶ INSERM UMR_S 999, Université Paris, France.
¹⁷ Institute of Liver Studies, Kings College Hospital, London, England.
¹⁸ Pediatric Endocrinology, Cantonal Hospital Aarau KSA, Aarau, Switzerland.
¹⁹ Service de Pneumologie, University of Geneva, Geneva, Switzerland.
²⁰ Cardiologie congénitale, Institut Mutualiste Montsouris, Paris, France.
²¹ Department of Radiology, Papa Giovanni XXIII Hospital, School of Medicine and Surgery - University of Milano-Bicocca, Bergamo, Italy.
²² Centre de référence des maladies vasculaires du foie, Service d'hépatologie Hôpital Beaujon, Clichy, France.
²³ VALDIG.
²⁴ Department of Cellular Pathology, Royal Free London NHS Foundation Trust/UCL Cancer Institute, London, England.
²⁵ Swiss Pediatric Liver Center, Division of Clinical Pathology, Diagnostic Department, University of Geneva, Geneva, Switzerland.
²⁶ AP-HP, Centre de référence de l'hypertension pulmonaire, Service de pneumologie et soins intensifs respiratoires, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
²⁷ INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France.
²⁸ ERN Lung.
²⁹ Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
³⁰ Northwestern University Feinberg School of Medicine, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois, USA.
³¹ Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan.
³² Department of paediatric and congenital cardiology, University Medical Center Groningen, University of Groningen, The Netherlands.
³³ Division of paediatric gastroenterology and hepatology, Department of paediatrics, University Medical Center Groningen, Groningen, The Netherlands.
³⁴ Université Paris Cité, CRI, INSERM, Paris, France.
³⁵ AP-HP, Département de Radiologie, Hôpital Beaujon. Nord, Clichy, France.
³⁶ Pediatric Cardiology Unit, Department of pediatrics, Gynecology and Obstetrics, University of Geneva, Geneva, Switzerland.
³⁷ Centre Universitaire Romand de Cardiologie et Chirurgie Cardiaque Pédiatrique, University of Geneva and Lausanne, Switzerland.
³⁸ Department of Pediatric Endocrinology, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, The Netherlands.
³⁹ AP-HP, Unité d'hépatologie pédiatrique et transplantation hépatique, Hôpital Necker, Paris, France.
⁴⁰ Centre de Référence des maladies rares du foie de l'enfant, FILFOIE, France.
⁴¹ Swiss pediatric Liver Center, Division of pediatric surgery, Department of Pediatrics, Gynecology, and Obstetrics, University of Geneva, Geneva, Switzerland.

PMID: 38234409
PMCID: PMC10792643
DOI: 10.1016/j.jhepr.2023.100933

Erratum in

Corrigendum to "Expert management of congenital portosystemic shunts and their complications" [JHEP Reports 6 (2024)].
McLin VA, Franchi-Abella S, Brütsch T, Bahadori A, Casotti V, de Ville de Goyet J, Dumery G, Gonzales E, Guérin F, Hascoet S, Heaton N, Kuhlmann B, Lador F, Lambert V, Marra P, Plessier DA, Quaglia A, Rougemont AL, Savale L, Sarma MS, Sitbon O, Superina RA, Uchida H, van Albada M, Johannes van der Doef HP, Vilgrain V, Wacker J, Zwaveling-Soonawala N, Debray D, Wildhaber BE. McLin VA, et al. JHEP Rep. 2024 Jan 30;6(3):101024. doi: 10.1016/j.jhepr.2024.101024. eCollection 2024 Mar. JHEP Rep. 2024. PMID: 38481635 Free PMC article.

Abstract

Congenital portosystemic shunts are often associated with systemic complications, the most challenging of which are liver nodules, pulmonary hypertension, endocrine abnormalities, and neurocognitive dysfunction. In the present paper, we offer expert clinical guidance on the management of liver nodules, pulmonary hypertension, and endocrine abnormalities, and we make recommendations regarding shunt closure and follow-up.

Keywords: adenoma; congenital portosystemic shunt; focal nodular hyperplasia; hepatocellular carcinoma; hyperandrogenism; hyperinsulinism; hypoglycemia; occlusion test; portal pressure; puberty; pulmonary hypertension; β-catenin.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflicts of interest that pertain to this work. Please refer to the accompanying ICMJE disclosure forms for further details.

Figures

**Fig. 1**
Five anatomic forms of congenital portosystemic shunts. (A) Colour-Doppler ultrasound of the liver in a newborn showing a direct communication between the left portal vein and the left hepatic vein (arrow) consistent with an intrahepatic portosystemic shunt. (B) Axial image of contrast-enhanced computed tomography in the portal phase showing a wide side-to-side extrahepatic communication between the portal bifurcation and the inferior vena cava (arrow). (C) Coronal reconstruction of contrast-enhanced computed tomography in the portal phase showing an abnormal, large ductus venosus (arrow). (D) Axial image of contrast-enhanced computed tomography in the portal phase showing a wide end-to-side extrahepatic communication between the origin of the main portal vein and the inferior vena cava (arrow). (E) Axial image of contrast-enhanced computed tomography in the portal phase showing a wide side-to-side extrahepatic communication between the splenic vein and the left renal vein (arrow).

**Fig. 2**
**Approach to the patient with suspected CPSS.** ∗If portopulmonary hypertensionn present, treat according to recommendations prior to CPSS closure. CPSS, congenital portal systemic shunt(s).

**Fig. 3**
Management algorithm in the presence of a liver mass(es) and/or CPSS. ∗Management algorithem applicable to each liver module. ∗∗Beware of intra-nodular heterogeniety or sampling error. ∗∗∗See section on contraindications to surgical or endovascular closure. AFP, alpha-fetoprotein; CPSS, congenital portal systemic shunts; FU, follow-up; US, ultrasound.

**Fig. 4**
Diagnostic algorithm for all forms of pulmonary hypertension in patients with CPSS. Echocardiographic probability of pulmonary hypertension is based on the value of the tricuspid regurgitation velocity and the detection of other echocardiographic signs suggestive of pulmonary hypertension. Low probability of pulmonary hypertension: tricuspid regurgitation velocity ≤2.8 m/s and no other echo pulmonary hypertension signs. Intermediate probability of pulmonary hypertension: tricuspid regurgitation velocity ≤2.8 m/s with echo pulmonary hypertension signs, or tricuspid regurgitation velocity 2.9-3.4 without other echo pulmonary hypertension signs. High probability: all other conditions. CHD, congenital heart disease; CO, cardiac output; CPSS, congenital portal systemic shunts; HPS, hepatopulmonary syndrome; mPAP, mean pulmonary arterial pressure; PAWP, pulmonary arterial wedge pressure; PoPH, portopulmonary hypertension; PVR, pulmonary vascular resistance; RHC, right heart catheterisation; WU, Wood units.

**Fig. 5**
Treatment algorithm of portopulmonary hypertension associated with CPSS. CHD, congenital heart disease; CPSS, congenital portal systemic shunts; PAH, pulmonary arterial hypertension; PVR, pulmonary vascular resistance; RHC, right heart catheterisation.

See this image and copyright information in PMC

References

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Expert management of congenital portosystemic shunts and their complications

Affiliations

Expert management of congenital portosystemic shunts and their complications

Authors

Affiliations

Erratum in

Abstract

Conflict of interest statement

Figures

References

Publication types

LinkOut - more resources

Full Text Sources