International Registry of Congenital Portosystemic Shunt (IRCPSS)

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.

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Key information

Conditions

Congenital Portosystemic Shunt 46, XX Disorders of Sex Development Adrenogenital Syndrome Brain Diseases Brain Diseases, Metabolic CPSS (Congenital Portosystemic Shunt) Cardiovascular Abnormalities Cardiovascular Diseases Central Nervous System Diseases Congenital Abnormalities Congenital, Hereditary, and Neonatal Diseases and Abnormalities Digestive System Diseases Digestive System Neoplasms Disease Attributes Disorders of Sex Development Endocrine System Diseases Female Urogenital Diseases Female Urogenital Diseases and Pregnancy Complications Glucose Metabolism Disorders Gonadal Disorders Hepatic Encephalopathy Hepatic Insufficiency Hepatopulmonary Syndrome Hyperammonemia Hyperandrogenism Hyperinsulinism Hypertension Hypertension, Pulmonary Hypoglycemia Insulin Resistance Liver Diseases Liver Failure Liver Neoplasms Lung Diseases Male Urogenital Diseases Metabolic Diseases Neoplasms Neoplasms by Site Nervous System Diseases Nutritional and Metabolic Diseases Pathologic Processes Pathological Conditions, Signs and Symptoms Rare Diseases Respiratory Tract Diseases Urogenital Abnormalities Urogenital Diseases Vascular Diseases Vascular Malformations

Age range

1 day and older

Sex eligibility

All sexes

Study type

Observational

Primary location

Royal Children's Hospital Melbourne, Parkville, Victoria, Australia

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

Individual with CPSS

Exclusion criteria

Secondary shunt without evidence of congenital shunt

Treatment and study plan

Shunt Closure

Procedure

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

Primary outcomes

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

Sponsors and collaborators

Lead sponsor

Prof. Valérie Mc Lin

Other

Collaborators

European Association for the Study of the Liver (EASL)
European Society of Pediatric Gastroenterology, Hepatology and Nutrition
Fondation Andrea Ferrari

Registry information

Official study title

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

Acronym: IRCPSS

Important dates

Study start: 2018
Primary completion: 2026
Study completion: 2028
First posted: Sep 18, 2023
Registry last updated: Jun 18, 2025

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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