What is the purpose of the International Registry of Porto-Systemic Shunts (IRCPSS)?
Although the number of CPSS cases detected in specialized centers is increasing, little is understood about the underlying causes and about which patients will develop signs and symptoms. Therefore the aim of the registry is to better identify patients who are at risk for developing complications and to offer them standardized care.

IRCPSS- what does it mean for the patient?
No additional blood draws or imaging will be required for the patient. Only results from routine exams will be entered into the registry. Data and images will be collected at each participating center. Data will be stored in a registry and images in a repository, both based in Geneva, Switzerland.

Data protection?
All data and images collected in this registry are pseudonymised, i.e. no name will appear, only codes, of which only a very small number of specialists will have the decryption key. Each participating center obtains ethical clearance to include patients in the registry.

Who can participate?
Neonates, children and adults with congenital porto-systemic shunts or a history of a CPSS.

50 participating centers in 21 countries Argentina (1), Australia (1), Belgium (2), Canada (2), France (5), Germany (4), Greece (2), India (2), Israel (2), Italy (5), Japan (1), The Netherlands (3), Portugal (1), Romania (1), Spain (1), Sweden (1), Switzerland (2), Turkey (3), The United Kingdom (5), The United States of America (5) and Uzbekistan (1)

Principal Investigator Prof. Valérie McLin
Hôpitaux Universitaires de Genève
Dpt Pédiatrie, Serv. spécialités pédiatriques
Rue Gabrielle-Perret-Gentil 4
1211 Geneva 4, Switzerland
E-mail: Valerie.McLin@hcuge.ch

For general or membership questions: Simona Korff, PhD
E-mail: Simona.Korff@hcuge.ch

Maurice Beghetti (Pediatric Cardiology)
Lorenzo D'Antiga (Pediatric Hepatology)
Dominique Debray (Pediatric Hepatology)
Stéphanie Franchi-Abella (Interventional Radiology)
Emmanuel Gonzales (Pediatric Hepatology)
Florent Guerin (Pediatric Surgery)
Anne-Lise Hachulla Lemaire (Interventional Radiology)
Virginie Lambert (Cardiology)
Valérie McLin (Pediatric Hepatology)
Periklis Makrythanasis (Genetics)
Laurent Savale (Pulmonology)
Marie-Victoire Senat (Obstetrics)
Frank van Steenbeek (Genetics)
Barbara E. Wildhaber (Pediatric Surgery)

The SC is assisted by the expertise of members of the Advisory Committee (AC) which includes experts in disciplines of interest in the assessment of the natural history of congenital porto-systemic shunts (CPSS):

Olivia Boyer (Nephrology)
Marco Colledan (Surgery)
Mark Davenport (Surgery)
Jean de Ville de Goyet (Surgery)
Catherine Guettier (Pathology)
Hubert van der Doef (Hepatology)
Christian Korff (Pediatric Neurology)
Virginie Lambert (Pediatric Cardiology)
Frédéric Lador (Pulmonology)
Hélène Maurey (Pediatric Neurology)
Aurélie Plessier (Hepatology and vascular liver diseases)
Maxime Ronot (Radiology)
Anne-Laure Rougemont (Pathology)

The registry is build using SecuTrial©, an up-to-date web-based interface which meets the most demanding international security standards. Database design and maintenance will be performed by the Clinical Research Center (CRC), University Hospitals Geneva (HUG). The CRC has expertise in the use of SecuTrial© for the design and maintenance of databases and clinical trials.

The image repository is hosted on a DICOM-compliant PACS server located in the data center of the Campus Biotech in Geneva under the responsibility of the Institute of Translational Molecular Imaging (ITMI). This institute is affiliated with the University of Geneva and the Swiss Federal Institute of Technology (EPFL).