ClinicalTrials.gov record NCT06041906

International Registry of Congenital Portosystemic Shunt (IRCPSS)

Record copied from ClinicalTrials.gov: Oct 10, 2026AI summaries generated: Sep 17, 2026

International Registry of Congenital Portosystemic Shunts (IRCPSS) - A Multi-centre, Retrospective and Prospective Registry of Neonates, Children and Adults With Congenital Portosystemic Shunts

About this page. This page shows the study's official ClinicalTrials.gov record ↗, with added help to make it easier to understand. We explain medical terms, highlight key details in plain language, and suggest questions you may want to ask the study team.

The record is periodically copied from ClinicalTrials.gov and may lag behind changes. AI summaries are checked by software, not a clinician.Read moreShow less

The official record is written by the study sponsor and published on ClinicalTrials.gov. It may not always reflect the latest changes. Before making any decisions, please confirm important information with the study team and your own healthcare team.

Where you see "AI summary", AI was used to create a plain-language explanation and software checked it against the source. It has not been reviewed by a clinician, so it may contain mistakes or leave out important details.

StatusEnrolling by invitationThe study enrols only people the study team invites, for example patients already at a particular clinic. It is not open to the public.

Study typeObservationalA study in which researchers do not assign participants to a treatment or other intervention. They observe or measure what happens, which may include study visits, tests, questionnaires or a review of medical records.

AgeUnder 1 month old and older

SexAll

LocationAustralia, Belgium, Canada, France, Germany, Hungary, India, Israel, Italy, Japan, Netherlands, Spain, Switzerland, Turkey, United Kingdom, United States

AI summaryWhat this study is about, in plain languageShow ▾Hide ▴

AI-written from the registry record, not the study's own wording. May contain errors or omissions. Check the registry text on this page and the original record ↗ before acting on it.

This is an international observational registry that gathers retrospective and prospective clinical and biological data on people with congenital portosystemic shunts (CPSS), including neonates, children, and people. It centralizes follow-up information and records events such as spontaneous shunt closure, complications, and shunt-closure procedures. The registry aims to improve understanding of how common CPSS is, how it progresses, and what factors are linked to complications.

What it is studying. The registry is collecting standardized clinical and biological information to define prevalence, natural history, individual risk factors, and physiopathology of CPSS, and to record outcomes such as spontaneous shunt closure, complications, and preemptive or surgical/interventional shunt closure over time.

What the researchers hope to learn. To count how often spontaneous shunt closure occurs; to record how many people develop one or more complications at 5, 10, and 20 years; to record numbers undergoing preemptive closure at 6, 12, and 24 months; and to build knowledge of CPSS prevalence, natural history, risk factors, and physiopathology.

What this study is about

The study's own summary, as published on ClinicalTrials.gov. Underlined terms have a short explanation.

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.

Interventions being studied

Being studied in this trial does not mean the treatment or procedure is approved or available for this condition.

Procedure: Shunt Closure

Eligibility

The criteria exactly as listed on ClinicalTrials.gov. Only the study team can say whether they apply to you.

Inclusion criteria

Individual with CPSS

Exclusion criteria

Secondary shunt without evidence of congenital shunt

Study description and what participation involves

The registry record has no detailed description beyond the summary above.

AI summaryWhat participation may involve, in plain languageShow ▾Hide ▴

AI-written from the registry record, not the study's own wording. May contain errors or omissions. Check the registry text on this page and the original record ↗ before acting on it.

Participants allow collection of their past and ongoing medical records and clinical follow-up data (the registry includes both retrospective and prospective cases). The study records clinical and biological information and any shunt closure events — spontaneous, surgical, interventional, or transplantation — as they occur in usual care. Outcomes tracked include timing of spontaneous or preemptive closure and the development of complications over time (time points in the record include 3, 6, 12, 24 months and 5, 10, 20 years), with follow-up planned for up to 20 years. The registry observes and records care and outcomes; it does not assign treatments.

How the study is structured. An observational registry (both retrospective and prospective) that follows people with CPSS over time—record duration listed as up to 20 years—collecting medical records, follow-up data, and information about procedures such as spontaneous, surgical, or interventional shunt closure.

Questions you could ask

These questions are suggestions for a conversation with the study team or your own clinician, not advice.

The registry lists this study under Congenital Portosystemic Shunt; CPSS (Congenital Portosystemic Shunt). Does it apply to my diagnosis?
This is an observational study, so the study itself gives no treatment. What would taking part involve for me, and would it change my usual care?
The registry says participants are enrolled by invitation. Who decides who is invited, and how?
The listed sites are in Australia, Belgium, Canada, France, Germany, Hungary, India, Israel, Italy, Japan, Netherlands, Spain, Switzerland, Turkey, United Kingdom, United States. Is any of them reachable for me, and does the study help with travel?
Which of the eligibility criteria would need to be checked with tests before I could join?

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Important dates

Study start: Apr 26, 2018
Primary completion: Oct 26, 2026 (estimated)
Study completion: Dec 31, 2028 (estimated)
Target enrollment: 500 participants (estimated)
Last updated on registry: Jun 18, 2025
Data verified: Oct 10, 2026

Sponsor and research team

The lead sponsor funds and oversees the trial. A pharmaceutical or biotech company sponsor typically means the study is testing that company's drug or device; a hospital, university, or government agency usually indicates investigator-initiated or publicly funded research.

Lead sponsor: Prof. Valérie Mc Lin
Collaborators: European Society of Pediatric Gastroenterology, Hepatology and Nutrition
European Association for the Study of the Liver (EASL)
Fondation Andrea Ferrari

Participating sites (26)

Filter by country:

Yale University School of Medicine
New Haven, Connecticut, 06520 · United States
Lurie Children's Hospital
Chicago, Illinois, 60611 · United States
Royal Children's Hospital Melbourne
Parkville, Victoria, 3052 · Australia
Universitary Clinics Saint-Luc, UCL
Brussels, 1200 · Belgium
The Hospital for Sick Children
Toronto, Ontario, M5G 1X8 · Canada
Beaujon University Hospital
Clichy, 92100 · France
Hôpital Bicêtre-Hôpitaux Universitaires Paris-Sud- Assistance Publique Hôpitaux de Paris.
Le Kremlin-Bicêtre, 94275 · France
Hôpital Necker
Paris, 75743 · France
Hannover Medical School
Hanover, 30625 · Germany
Haunersche Kinderklinik - LMU Munich
Munich, 80337 · Germany

Terms used on this page · explanations by MedicalUpdates.ai

registry: An organised collection of information about people with a particular condition, gathered over time for research. Joining a registry usually means sharing medical information, not receiving a treatment.
follow-up: The period after treatment or enrolment during which participants are observed or contacted, for example to record side effects or outcomes.
natural history: A study that follows how a condition changes over time without assigning an experimental treatment. It may involve study visits and tests, and is often used to plan future trials.
Inclusion Criteria: The conditions a person must meet to be considered for the study, for example a confirmed diagnosis, an age range or a test result.
Exclusion Criteria: The conditions that rule a person out even if they meet the inclusion criteria, for example other illnesses, certain medicines or pregnancy.

The study record on this page is reproduced from ClinicalTrials.gov, a database of the U.S. National Library of Medicine, where it was submitted by the study sponsor. MedicalUpdates.ai does not own this content, is not affiliated with or endorsed by ClinicalTrials.gov, NIH, or the U.S. Government, and does not verify the record. Plain-language notes, the glossary and the questions above are added by this site; text in the boxes marked "AI summary" was written by an AI model and may contain errors. Registry records change over time. Check the original record for the current version and discuss eligibility or participation with a qualified healthcare professional.

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