International Registry of Congenital Portosystemic Shunt (IRCPSS)

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

Status

Enrolling by invitation

Phases

Unknown

Study type

Observational

Source

ClinicalTrials.gov

Registry ID

NCT06041906

Acronym

IRCPSS

Enrollment

500

Registered

2023-09-18

Start date

2018-04-26

Completion date

2028-12-31

Last updated

2025-06-18

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

Congenital Portosystemic Shunt, CPSS (Congenital Portosystemic Shunt)

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

Brief 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. 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

PROCEDUREShunt Closure

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

Study design

Observational model

COHORT

Time perspective

OTHER

Eligibility

Sex/Gender

ALL

Age

1 Days to No maximum

Healthy volunteers

Inclusion criteria

Individual with CPSS

Exclusion criteria

Secondary shunt without evidence of congenital shunt

Design outcomes

Primary

Measure	Time frame
Numbers of patients with spontaneous shunt closure	3; 6; 12; 24 months
Number of patients developing one or more complications	5; 10; 20 years
Number of patients undergoing preemptive closure	6; 12; 24 months

Countries

Australia, Belgium, Canada, France, Germany, Hungary, India, Israel, Italy, Japan, Netherlands, Spain, Switzerland, Turkey (Türkiye), United Kingdom, United States

Outcome results

None listed

Source: ClinicalTrials.gov · Data processed: Feb 4, 2026