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.

Shunt classes

This picture was published in Clinics and Research in Hepatology and Gastroenterology, 44(4): 452-459 - Florent Guérin, Stéphanie Franchi Abella, Valérie McLin, Oanez Ackermann, Muriel Girard, Jean Paul Cervoni, Laurent Savale, Virginia Hernandez-Gea, Dominique Valla, Sophie Hillaire, Danielle Dutheil, Christophe Bureau, Emmanuel Gonzales, Aurélie Plessier - Congenital portosystemic shunts: Vascular liver diseases: Position papers from the francophone network for vascular liver diseases, the French Association for the Study of the Liver (AFEF), and ERN-rare liver – September 2020.

As a relatively new diagnosis, we still don’t know how often CPSS occur. Sometimes they can be diagnosed in patients without any symptoms, but they can also be detected based on signs of liver disorder, neuropsychological disorder, gastrointestinal complications, vascular disorders or cardiopulmonary complications.

SystemsPresenting signs and symptoms
LiverCholestasis - Hyperammonemia - Hypoglycemia - Benign or malignant liver tumor - Enlarged liver
Gastrointestinal, renal and abdominalGastrointestinal bleed - Spleen and kidney malformations - Esophagus and intestine malformations - Kidney malformations - other renal disorders (blood or protein in urine)
NeuropsychologicalNeurodevelopment delay - Impaired concentration and attention - Seizures
Cardiac and vascularShortness of breath - Loss of consciousness - Cardiomegaly - Enlarged heart - Skin angiomas
Other signs and symptomsHypothyroidism - Thyroid problems - Overgrowth