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Enrolling by invitation NCT06041906

International Registry of Congenital Portosystemic Shunt (IRCPSS)

— Observational Congenital Portosystemic Shunt CPSS (Congenital Portosystemic Shunt)

For patients and families

In plain language

An automatic summary of structured registry data. It is an orientation aid, not a substitute for the official protocol or a physician assessment.

What is being studied
The protocol lists: Shunt Closure.
Who it may be relevant to
Registry conditions: Congenital Portosystemic Shunt, CPSS (Congenital Portosystemic Shunt). Basic parameters: from 1 Day · All.
What needs checking
Age, condition and sex are only basic indicators. Prior treatment, laboratory values and other mandatory requirements appear in the eligibility criteria below.
Where it takes place
United States, Australia, Belgium, Canada, France +11
Next step
Save the trial, show it to the treating physician, and confirm current recruitment with the study center. Costs, documents and travel →

Unsure about the terms? Read our patient guide →

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

Overview

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

  • Procedure Shunt Closure
    Consist in spontaneous, surgical or interventional closure of the shunt. Depending on context, surgical intervention may also be transplantation.

Primary outcome measures

  • 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]

Eligibility criteria

Inclusion criteria

Individual with CPSS

Exclusion criteria

Secondary shunt without evidence of congenital shunt

Criteria are shown verbatim from the registry (in English). Final eligibility is always assessed by the study center.

Healthy volunteers: No

Study design

Observational model
Cohort

Study locations

France · 3 centers
  • Beaujon University Hospital — Clichy
  • Hôpital Bicêtre-Hôpitaux Universitaires Paris-Sud- Assistance Publique Hôpitaux de Paris. — Le Kremlin-Bicêtre
  • Hôpital Necker — Paris
Germany · 3 centers
  • Hannover Medical School — Hanover
  • Haunersche Kinderklinik - LMU Munich — Munich
  • University of Tuebingen — Tübingen
Italy · 3 centers
  • Papa Giovanni XXIII Hospital — Bergamo
  • Children's Hospital Santobono — Naples
  • Istituto di Ricovero e Cura a Carattere Scientifico (ISMETT) — Palermo
United States · 2 centers
  • Yale University School of Medicine — New Haven
  • Lurie Children's Hospital — Chicago
India · 2 centers
  • Amrita institute of Medical Sciences — Kochi
  • Department of Pediatric Hepatology Institute of Liver and Biliary Sciences — New Delhi
Netherlands · 2 centers
  • Academic Medical Centre — Amsterdam
  • University Medical Center Groningen — Groningen
United Kingdom · 2 centers
  • Birmingham Children's Hospital — Birmingham
  • Royal Hospital for Children and Young People — Edinburgh
Australia · 1 center
  • Royal Children's Hospital Melbourne — Parkville
Belgium · 1 center
  • Universitary Clinics Saint-Luc, UCL — Brussels
Canada · 1 center
  • The Hospital for Sick Children — Toronto
Hungary · 1 center
  • First Department of Pediatrics, Semmelweis University — Budapest
Israel · 1 center
  • Schneider Children's Medical Center of Israel — Petach Tikvah
Japan · 1 center
  • National Center for Child Health and Development — Tokyo
Spain · 1 center
  • Hospital Clínic de Barcelona — Barcelona
Switzerland · 1 center
  • Hôpitaux Universitaires de Geneve (HUG) — Geneva
Turkey (Türkiye) · 1 center
  • Gazi University, Faculty of Medicine — Yenimahalle

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. PMID 28386718
  • Cudalbu 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. PMID 23936100
  • Joye 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. PMID 33276028
  • McLin 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. PMID 33749116
  • Korff 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 p PMID 35854389
  • Lambert 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. PMID 33281106
  • McLin 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. PMID 35104444
  • McLin 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. PMID 30628988

Identifiers

NCT: NCT06041906 · 2018-00413

Primary sources (government registries)

View this study on ClinicalTrials.gov ↗