International registry of congenital porto-systemic shunts

Congenital porto-systemic shunts (CPSS) are rare malformations of the blood vessels. Normally, blood from the intestines passes through the liver before going to the heart. CPSS derive blood from the intestines directly to the heart.

Today we know that if the blood does not pass through the liver, many complications may appear in the more or less long term. According to medical specialists, these CPSS must be closed by surgery or by interventional radiology. 

Although the number of CPSS cases detected in specialized centers is increasing, medical, biological and physiological data are still poorly understood, which is why it is important to create an international register that will gather the largest volume of information possible on CPSS.

This information will help to better understand this malformation and its evolution over time in newborns, children and adults with CPSS, as well as to understand the natural history of CPSS. They will help to better identify patients who are at risk for developing complications and to offer them standardised care and treatments.

Newborns, children or adults who have or have had a congenital porto-systemic shunt (CPSS).

All the information, past and present, is gathered in a large database, the International Register of Congenital Port-Systemic Shunts (International Registry of Congenital Porto-Systemic Shunts, IRCPSS). This information covers symptoms, clinical data, biological analyses, complications and images collected from the date of diagnosis until 5 years post-closure of the CPSS. 

All the data and values collected in this register will be coded, i.e. no name will appear. Only a very limited number of specialists are in possession of the decryption key. They are subject to medical secrecy and have access to the children's data only to perform tasks within the context of the register.

This international registry will also include surgical or radiological data of the closure of the CPSS. Data from all centers will be collected in Geneva, in coded format, and might be reused as part of research projects for other future studies.

No, there will be no change regarding the child's usual follow-up as this is only a collection of data in order to obtain a better knowledge about these rare congenital anomalies. There is no risk, since no additional medical procedure will be performed, and this will not result in additional costs.  

• The International Registry of Porto-Systemic Shunts (IRCPSS)