International Registry of Congenital Portosystemic Shunt (IRCPSS)

Condition(s) studied

Investigational drug(s) / intervention(s)

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

Study 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.

Eligibility

Primary outcome measure(s)

• 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

Trial sites (26)