Welcome to the International Registry of Congenital Portosystemic Shunts website
The purpose of this website is to promote the International Registry of Congenital Porto-Systemic Shunts (IRCPSS) and to provide ressources for a wide audience, from patients to health professional around the globe.
What is a Congenital Portosystemic Shunt?
A Congenital Porto-Systemic Shunt (CPSS) is a rare vascular malformation where blood goes directly from the intestines to the heart without passing through the liver for filtration.
Today we know that if the blood does not get filtered by the liver, it can cause complications at any age.
It is therefore recommended that most CPSS should be closed surgically or by small devices placed in the abnormal vessel.
Natural history and diagnosis
CPSS can be diagnosed fortuitously or because of complications at any age from the fetus to the elderly. During pregnancy, CPSS are diagnosed using ultrasound. After birth, the most frequent type of CPSS – intrahepatic – tend to close spontaneously in the first months of life. Nonetheless, some shunts remain open. According to the position and size of the CPSS, the blood coming from the intestines will be diverted partially or completely thereby bypassing the liver. This means the liver cannot take perform its function of detoxifying the liver. Until now there is no way to predict what complication(s) a patient will present or when it will occur.
The management of CPSS requires a multidisciplinary team to manage potential complications.
Expertise is required to determine whether the patient will benefit from shunt closure by interventional radiologists who can place plugs in the shunt, or if surgery will be required.
Congenital porto-systemic shunts are an anomaly of abdominal veins which lead to the blood bypassing the liver. When the liver is bypassed complications may occur starting at a very young age. Although the prevalence of this rare malformation is not known, the following complications have been described in children: liver tumors, heart failure, vascular anomalies of the lung, developmental delay and more. Therefore, the aim of the international registry is to increase evidence and knowledge on this rare malformation.
The International Registry of Congenital Portosystemic Shunts is a worldwide initiative
Its aim is to help health professionals and patients to find resources, prevent patients from diagnostic wandering, improve the diagnosis and treatments around the Congenital Portosystemic Shunts condition.