International Registry of Congenital Portosystemic Shunt (IRCPSS)

Launched by PROF. VALÉRIE MC LIN · Sep 11, 2023

Keywords

ClinConnect Summary

This study is the International Registry of Congenital Portosystemic Shunts (IRCPSS). It’s an international, observational registry that collects information about people born with a congenital portosystemic shunt (CPSS), a rare liver blood-vessel condition where blood from the gut can bypass the liver. The goal is to learn how common CPSS is, how it behaves over time, what problems may show up, and how best to manage it in real life. It tracks neonates, children, and adults at many expert centers around the world, and there are no experimental drugs or new treatments being tested by the registry itself.

People who have CPSS (of any sex and any age, from 1 day old to older adults) may be eligible to participate, as long as the shunt is congenital and not caused by another condition (secondary shunt). The registry collects data on those who undergo closure of the shunt—whether the closure happens spontaneously, surgically, or with interventional procedures (and sometimes even transplantation, if needed). The main things they plan to measure are how many people experience spontaneous shunt closure, how many have one or more complications over time, and how many have preemptive closure at several time points (3, 6, 12, 24 months and then later). Enrollment is ongoing, with about 500 participants expected over a 20-year period, and follow-up extending for many years; results are not yet available.

Gender

ALL

Eligibility criteria

• Inclusion Criteria:
• • Individual with CPSS
• Exclusion Criteria:
• • Secondary shunt without evidence of congenital shunt