Congenital porto-systemic shunts (CPSS) are rare vascular malformations that direct blood from the intestines directly to the heart. The blood is no longer filtered by the liver. Today we know that if the blood does not run through the liver, it can cause complications at any age. It is currently accepted that most CPSS should be closed radiologically or surgically.

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.

To date, the prevalence of CPSS is not known, but the severity of its repercussions is recognized. CPSS can be diagnosed fortuitously on an asymptomatic patient or be detected based on signs and symptoms in various fields as liver disorders, neuropsychological disorders, gastrointestinal complications, vascular disorders and 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