Congenital Portosystemic ShuntIRCPSSNCT06041906
International Registry of Congenital Portosystemic Shunt (IRCPSS)
An observational study of Congenital Portosystemic Shunt and CPSS (Congenital Portosystemic Shunt), run by Prof. Valérie Mc Lin at 26 sites in 16 countries.
Trial summary
Congenital Portosystemic Shunt (CPSS) is a rare condition important by the multiplicity and severity of associated complications. CPSS is venous anomaly in which blood coming from the intestines only partially passes through the liver. Read more about this study
- Who is it for?
- Ages 1 day and older
- Conditions the study is for
- Congenital Portosystemic Shunt, CPSS (Congenital Portosystemic Shunt)
- What is being tested?
- Shunt Closure
- Type of treatment
- Surgery or procedure
- How many people
- 500 participants
- Where
- 26 sitesin 16 countries
- Study dates
- 26 Apr 2018to 31 Dec 2028
- How long will I take part?
- 20 Years
- Phase
- Observational
- Are there results?
- Not yet: the study is under way
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 1 day and older
- All sexes
- No healthy volunteers
- Child, Adult, Older adult
Study population: neonates; children; adults
Who may be able to join
- Individual with CPSS
Who cannot join
- Secondary shunt without evidence of congenital shunt
Trial locations and contacts
26 sites in 16 countries. Contact the study team or a site near you to ask about taking part.
26 sites in 16 countries
France3 sites
Beaujon University Hospital
Clichy, 92100
Hôpital Bicêtre-Hôpitaux Universitaires Paris-Sud- Assistance Publique Hôpitaux de Paris.
Le Kremlin-Bicêtre, 94275
Hôpital Necker
Paris, 75743
Germany3 sites
Hannover Medical School
Hanover, 30625
Haunersche Kinderklinik - LMU Munich
Munich, 80337
University of Tuebingen
Tübingen, 72076
Italy3 sites
Papa Giovanni XXIII Hospital
Bergamo, 24127
Children's Hospital Santobono
Naples, 80129
Istituto di Ricovero e Cura a Carattere Scientifico (ISMETT)
Palermo, 90127
United States2 sites
Lurie Children's Hospital
Chicago, Illinois, 60611
Yale University School of Medicine
New Haven, Connecticut, 06520
India2 sites
Amrita institute of Medical Sciences
Kochi, Kerala, 682041
Department of Pediatric Hepatology Institute of Liver and Biliary Sciences
New Delhi, 110070
Netherlands2 sites
Academic Medical Centre
Amsterdam, 1105
University Medical Center Groningen
Groningen, 9700 RB
United Kingdom2 sites
Birmingham Children's Hospital
Birmingham, B4 6NH
Royal Hospital for Children and Young People
Edinburgh, EH16 4TJ
Australia1 site
Royal Children's Hospital Melbourne
Parkville, Victoria, 3052
Hungary1 site
First Department of Pediatrics, Semmelweis University
Budapest, 1083
Israel1 site
Schneider Children's Medical Center of Israel
Petach Tikvah, 49202
Switzerland1 site
Hôpitaux Universitaires de Geneve (HUG)
Geneva, Canton of Geneva, 1205
Turkey (Türkiye)1 site
Gazi University, Faculty of Medicine
Yenimahalle, Ankara, 06560
About this study
Official title: International Registry of Congenital Portosystemic Shunts (IRCPSS) - A Multi-centre, Retrospective and Prospective Registry of Neonates, Children and Adults With Congenital Portosystemic Shunts
Congenital Portosystemic Shunt (CPSS) is a rare condition important by the multiplicity and severity of associated complications.
CPSS is venous anomaly in which blood coming from the intestines only partially passes through the liver.
Read more
This leads to the accumulation of potentially toxic factors that cause systemic effects.
Complications vary among the individuals, and currently, it is challenging to predict which individuals will develop severe complications.
The IRCPSS registry is established with the aim of centralizing detailed clinical follow-up and biological information from participants around the world who suffer from Congenital Portosystemic Shunt (CPSS). A multidisciplinary consortium of experts is collaborating to enhance our understanding of the prevalence, natural history, individual risks, and physiopathology of the disease through the IRCPSS registry.
- Being tested
- Shunt Closure
- Keywords
- shunt, portosystemic, portasystemic, porto-systemic, porta-systemic, registry, liver, rare disease, Abernethy malformation, vascular malformation, Hypertension, Pulmonary, Hepatopulmonary Syndrome, Liver Neoplasms, hyperinsulinemic, hypoglycemia, hyperinsulinism, Insulin resistance, hyperandrogenism, Hepatic Encephalopathy, Hyperammonemia
- Medical subject headings
- Congenital Portosystemic Shunt, Rare Diseases, Vascular Malformations, Hypertension, Pulmonary, Hepatopulmonary Syndrome, Liver Neoplasms, Hypoglycemia, Hyperinsulinism
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
Shunt ClosureProcedure or surgery
Consist in spontaneous, surgical or interventional closure of the shunt. Depending on context, surgical intervention may also be transplantation.
| Study group | Role | What they receive |
|---|---|---|
| Neonate, Children, Adult with CPSS | Not described Receives: Procedure: Shunt Closure |
What is being measured
The primary outcome is the main question the study is designed to answer.
Numbers of patients with spontaneous shunt closure
Measured: 3; 6; 12; 24 months
Number of patients developing one or more complications
Measured: 5; 10; 20 years
Number of patients undergoing preemptive closure
Measured: 6; 12; 24 months
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted18 Sep 2023
- Study start26 Apr 2018
- Primary completion26 Oct 2026 (estimated)
- Study completion31 Dec 2028 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (11)Papers the sponsor has linked to this study.
- Other trials for Congenital Portosystemic ShuntStudies of the same condition, open and under way.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Cohort
- Time perspective
- Other
- Enrolment
- 500 participants (estimated)
- Target duration
- 20 Years
Sponsor and investigators
- Lead sponsor
- Prof. Valérie Mc Lin
- Sponsor type
- University, hospital or other
- Collaborators
- European Society of Pediatric Gastroenterology, Hepatology and Nutrition, European Association for the Study of the Liver, Fondation Andrea Ferrari
- Responsible party
- Sponsor-investigator, Prof. Valérie Mc Lin, University Hospital, Geneva
- Investigators
- Valérie A Mc Lin, Prof. Dr. med., Study director, University Hospital, Geneva
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT06041906
- Sponsor's protocol ID
- 2018-00413
- Other IDs
- 2018
- Acronym
- IRCPSS
- Status last verified
- Dec 2024
- First submitted
- 11 Sep 2023
- Last updated
- 18 Jun 2025
- Results posted
- No
- Data monitoring committee
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- Undecided: undecided
Publications
Hanquinet S, Morice C, Courvoisier DS, Cousin V, Anooshiravani M, Merlini L, McLin VA. Globus pallidus MR signal abnormalities in children with chronic liver disease and/or porto-systemic shunting. Eur Radiol. 2017 Oct;27(10):4064-4071. doi: 10.1007/s00330-017-4808-x. Epub 2017 Apr 6.
PubMed 28386718Cudalbu C, McLin VA, Lei H, Duarte JM, Rougemont AL, Oldani G, Terraz S, Toso C, Gruetter R. The C57BL/6J mouse exhibits sporadic congenital portosystemic shunts. PLoS One. 2013 Jul 23;8(7):e69782. doi: 10.1371/journal.pone.0069782. Print 2013.
PubMed 23936100Joye R, Lador F, Aggoun Y, Farhat N, Wacker J, Wildhaber BE, Vallee JP, Hachulla AL, McLin VA, Beghetti M. Outcome of paediatric portopulmonary hypertension in the modern management era: A case report of 6 patients. J Hepatol. 2021 Mar;74(3):742-747. doi: 10.1016/j.jhep.2020.11.039. Epub 2020 Dec 1.
PubMed 33276028McLin V, Beghetti M, D'Antiga L, Franchi-Abella S; International Registry of Congenital Porto-Systemic Shunts (IRCPSS). Current Quandaries in the Management of Congenital Portosystemic Shunts. Liver Transpl. 2021 Aug;27(8):1212-1213. doi: 10.1002/lt.26055. Epub 2021 Jul 20. No abstract available.
PubMed 33749116Korff S, Mostaguir K, Beghetti M, D'Antiga L, Debray D, Franchi-Abella S, Gonzales E, Guerin F, Hachulla AL, Lambert V, Makrythanasis P, Roduit N, Savale L, Senat MV, Spaltenstein J, van Steenbeek F, Wildhaber BE, Zwahlen M, McLin VA. International registry of congenital porto-systemic shunts: a multi-centre, retrospective and prospective registry of neonates, children and adults with congenital porto-systemic shunts. Orphanet J Rare Dis. 2022 Jul 19;17(1):284. doi: 10.1186/s13023-022-02412-8.
PubMed 35854389Lambert V, Ladarre D, Fortas F, Durand P, Herve P, Gonzales E, Guerin F, Savale L, McLin VA, Ackermann O, Franchi-Abella S; International Registry of Congenital Portosystemic Shunts (IRCPSS). Cardiovascular disorders in patients with congenital portosystemic shunts: 23 years of experience in a tertiary referral centre. Arch Cardiovasc Dis. 2021 Mar;114(3):221-231. doi: 10.1016/j.acvd.2020.10.003. Epub 2020 Dec 3.
PubMed 33281106McLin VA, D'Antiga L. The current pediatric perspective on type B and C hepatic encephalopathy. Anal Biochem. 2022 Apr 15;643:114576. doi: 10.1016/j.ab.2022.114576. Epub 2022 Jan 29.
PubMed 35104444McLin VA, Franchi Abella S, Debray D, Guerin F, Beghetti M, Savale L, Wildhaber BE, Gonzales E; Members of the International Registry of Congenital Porto-Systemic Shunts. Congenital Portosystemic Shunts: Current Diagnosis and Management. J Pediatr Gastroenterol Nutr. 2019 May;68(5):615-622. doi: 10.1097/MPG.0000000000002263.
PubMed 30628988Bahadori A, Kuhlmann B, Debray D, Franchi-Abella S, Wacker J, Beghetti M, Wildhaber BE, McLin VA, On Behalf Of The Ircpss. Presentation of Congenital Portosystemic Shunts in Children. Children (Basel). 2022 Feb 11;9(2):243. doi: 10.3390/children9020243.
PubMed 35204963Guerin F, Franchi Abella S, McLin V, Ackermann O, Girard M, Cervoni JP, Savale L, Hernandez-Gea V, Valla D, Hillaire S, Dutheil D, Bureau C, Gonzales E, Plessier A. Congenital portosystemic shunts: Vascular liver diseases: Position papers from the francophone network for vascular liver diseases, the French Association for the Study of the Liver (AFEF), and ERN-rare liver. Clin Res Hepatol Gastroenterol. 2020 Sep;44(4):452-459. doi: 10.1016/j.clinre.2020.03.004. Epub 2020 Apr 9. No abstract available.
PubMed 32279979Rock NM, Beghetti M, Tissot C, Willi JP, Bouhabib M, McLin VA, Maggio ABR. Reliable Detection of Intrapulmonary Shunts Using Contrast-Enhanced Echocardiography in Children With Portal Hypertension or Portosystemic Shunt. J Pediatr Gastroenterol Nutr. 2021 Jul 1;73(1):73-79. doi: 10.1097/MPG.0000000000003079.
PubMed 33605662
Study at a glance
From the registry record, last updated by the sponsor on 18 Jun 2025. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- International Registry of Congenital Portosystemic Shunts (IRCPSS) - A Multi-centre, Retrospective and Prospective Registry of Neonates, Children and Adults With Congenital Portosystemic Shunts
- ClinicalTrials.gov ID
- NCT06041906
- Acronym / sponsor ID
- IRCPSS / 2018-00413
- Keywords
- shunt, portosystemic, portasystemic, porto-systemic, porta-systemic, registry, liver, rare disease
- Study type
- Observational
- Recruitment status
- Enrolling by invitation
- Enrolment
- 500 participants (estimated)
- Lead sponsor
- Prof. Valérie Mc LinUniversity, hospital or other
- Collaborators
- European Society of Pediatric Gastroenterology, Hepatology and Nutrition, European Association for the Study of the Liver, Fondation Andrea Ferrari
- Study start
- 26 Apr 2018 (actual)
- Primary completion
- 26 Oct 2026 (estimated)
- Study completion
- 31 Dec 2028 (estimated)
- First posted
- 18 Sep 2023 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
Congenital Portosystemic Shunt (CPSS) is a rare condition important by the multiplicity and severity of associated complications.
CPSS is venous anomaly in which blood coming from the intestines only partially passes through the liver.
This leads to the accumulation of potentially toxic factors that cause systemic effects.
Complications vary among the individuals, and currently, it is challenging to predict which individuals will develop severe complications.
The IRCPSS registry is established with the aim of centralizing detailed clinical follow-up and biological information from participants around the world who suffer from Congenital Portosystemic Shunt (CPSS). A multidisciplinary consortium of experts is collaborating to enhance our understanding of the prevalence, natural history, individual risks, and physiopathology of the disease through the IRCPSS registry.
Study design
- Observational model
- Cohort
- Time perspective
- Other
- Target follow-up
- 20 Years
- Number of arms
- 1
| Arm | Type | Receives |
|---|---|---|
| Neonate, Children, Adult with CPSS | – | Procedure: Shunt Closure |
Who may qualify
- Ages
- 1 day and older
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Child, Adult, Older adult
- Study population
- neonates; children; adults
Key inclusion criteria (1 listed)
- Individual with CPSS
Key exclusion criteria (1 listed)
- Secondary shunt without evidence of congenital shunt
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| Shunt ClosureConsist in spontaneous, surgical or interventional closure of the shunt. Depending on context, surgical intervention may also be transplantation. | Procedure or surgery | Neonate, Children, Adult with CPSS |
Primary outcome
Numbers of patients with spontaneous shunt closure
Time frame: 3; 6; 12; 24 months
Number of patients developing one or more complications
Time frame: 5; 10; 20 years
Number of patients undergoing preemptive closure
Time frame: 6; 12; 24 months
Results and safety
This study is still under way. Results are not available in the source record yet.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 18 Jun 2025.
- Background: PubMed 28386718Hanquinet S, Morice C, Courvoisier DS, Cousin V, Anooshiravani M, Merlini L, McLin VA. Globus pallidus MR signal abnormalities in children with chronic liver disease and/or porto-systemic shunting. Eur Radiol. 2017 Oct;27(10):4064-4071. doi: 10.1007/s00330-017-4808-x. Epub 2017 Apr 6.
- Background: PubMed 23936100Cudalbu C, McLin VA, Lei H, Duarte JM, Rougemont AL, Oldani G, Terraz S, Toso C, Gruetter R. The C57BL/6J mouse exhibits sporadic congenital portosystemic shunts. PLoS One. 2013 Jul 23;8(7):e69782. doi: 10.1371/journal.pone.0069782. Print 2013.
- Background: PubMed 33276028Joye R, Lador F, Aggoun Y, Farhat N, Wacker J, Wildhaber BE, Vallee JP, Hachulla AL, McLin VA, Beghetti M. Outcome of paediatric portopulmonary hypertension in the modern management era: A case report of 6 patients. J Hepatol. 2021 Mar;74(3):742-747. doi: 10.1016/j.jhep.2020.11.039. Epub 2020 Dec 1.
- Background: PubMed 33749116McLin V, Beghetti M, D'Antiga L, Franchi-Abella S; International Registry of Congenital Porto-Systemic Shunts (IRCPSS). Current Quandaries in the Management of Congenital Portosystemic Shunts. Liver Transpl. 2021 Aug;27(8):1212-1213. doi: 10.1002/lt.26055. Epub 2021 Jul 20. No abstract available.
- Background: PubMed 35854389Korff S, Mostaguir K, Beghetti M, D'Antiga L, Debray D, Franchi-Abella S, Gonzales E, Guerin F, Hachulla AL, Lambert V, Makrythanasis P, Roduit N, Savale L, Senat MV, Spaltenstein J, van Steenbeek F, Wildhaber BE, Zwahlen M, McLin VA. International registry of congenital porto-systemic shunts: a multi-centre, retrospective and prospective registry of neonates, children and adults with congenital porto-systemic shunts. Orphanet J Rare Dis. 2022 Jul 19;17(1):284. doi: 10.1186/s13023-022-02412-8.
- Background: PubMed 33281106Lambert V, Ladarre D, Fortas F, Durand P, Herve P, Gonzales E, Guerin F, Savale L, McLin VA, Ackermann O, Franchi-Abella S; International Registry of Congenital Portosystemic Shunts (IRCPSS). Cardiovascular disorders in patients with congenital portosystemic shunts: 23 years of experience in a tertiary referral centre. Arch Cardiovasc Dis. 2021 Mar;114(3):221-231. doi: 10.1016/j.acvd.2020.10.003. Epub 2020 Dec 3.
- Background: PubMed 35104444McLin VA, D'Antiga L. The current pediatric perspective on type B and C hepatic encephalopathy. Anal Biochem. 2022 Apr 15;643:114576. doi: 10.1016/j.ab.2022.114576. Epub 2022 Jan 29.
- Background: PubMed 30628988McLin VA, Franchi Abella S, Debray D, Guerin F, Beghetti M, Savale L, Wildhaber BE, Gonzales E; Members of the International Registry of Congenital Porto-Systemic Shunts. Congenital Portosystemic Shunts: Current Diagnosis and Management. J Pediatr Gastroenterol Nutr. 2019 May;68(5):615-622. doi: 10.1097/MPG.0000000000002263.
- Background: PubMed 35204963Bahadori A, Kuhlmann B, Debray D, Franchi-Abella S, Wacker J, Beghetti M, Wildhaber BE, McLin VA, On Behalf Of The Ircpss. Presentation of Congenital Portosystemic Shunts in Children. Children (Basel). 2022 Feb 11;9(2):243. doi: 10.3390/children9020243.
- Background: PubMed 32279979Guerin F, Franchi Abella S, McLin V, Ackermann O, Girard M, Cervoni JP, Savale L, Hernandez-Gea V, Valla D, Hillaire S, Dutheil D, Bureau C, Gonzales E, Plessier A. Congenital portosystemic shunts: Vascular liver diseases: Position papers from the francophone network for vascular liver diseases, the French Association for the Study of the Liver (AFEF), and ERN-rare liver. Clin Res Hepatol Gastroenterol. 2020 Sep;44(4):452-459. doi: 10.1016/j.clinre.2020.03.004. Epub 2020 Apr 9. No abstract available.
- Background: PubMed 33605662Rock NM, Beghetti M, Tissot C, Willi JP, Bouhabib M, McLin VA, Maggio ABR. Reliable Detection of Intrapulmonary Shunts Using Contrast-Enhanced Echocardiography in Children With Portal Hypertension or Portosystemic Shunt. J Pediatr Gastroenterol Nutr. 2021 Jul 1;73(1):73-79. doi: 10.1097/MPG.0000000000003079.
- International Registry of Congenital Portosystemic Shunts website, for patients and professionalsLink provided by the sponsor.
- Individual participant dataSharing planned: Undecided. undecided
Related trials for Congenital Portosystemic Shunt
Not yet recruitingObservational study
Genetic Hallmarks of Patients With Congenital Portosystemic Shunts and Portopulmonary Hypertension
Portopulmonary Hypertension, Pulmonary Arterial Hypertension (PAH)
Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part.
Valérie A Mc Lin, Prof. Dr. med. study director
University Hospital, Geneva
26 sites in 16 countries: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Congenital Portosystemic Shunt and CPSS (Congenital Portosystemic Shunt), run by Prof. Valérie Mc Lin at 26 sites in 16 countries.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 1 inclusion and 1 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: enrolling by invitation, as of the sponsor's last update on 18 Jun 2025. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 26 sites in France, Germany, Italy, United States, India and 11 more countries. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
This study is still under way. Results are not available in the source record yet.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Numbers of patients with spontaneous shunt closure (time frame: 3; 6; 12; 24 months). The study lists 3 primary outcomes in all. It is what the study was designed to measure, not a finding.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT06041906, which also lists 11 linked publications.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 18 Jun 2025.