In this tenth ERN Rare-Liver webinar, held on 9 November 2021, Prof. McLin and Dr Gonzales provided an overview of the natural history of Congenital Portosystemic Shunts (CPSS) and presented the International Registry of Congenital Portosystemic Shunts (IRCPSS). A first case was presented by Prof. Savale about CPSS revealed by cardiopulmonary complications in an adult patient, followed by a second case about CPSS revealed by a liver nodule in an adult patient discussed by Prof. Ronot and Dr Baiges.

Targeted Audience

  • General practitioners
  • Hepatologists
  • Oncologists
  • Health care professionals

Background: natural history of CPSS and presentation of the IRCPSS


First case: CPSS revealed by cardiopulmonary complications in an adult patient



Second case: CPSS revealed by a liver nodule in an adult patient – radiological approach and management



Emmanuel Gonzales Emmanuel Gonzales is a pediatric hepatologist working in Bicêtre Hospital, AP-HP Université Paris-Saclay, France. He is actively involved in the field of rare liver diseases both at the national and at the ERN level.
Valérie McLin Pr Valérie McLin is the Medical Director of the Swiss Pediatric Liver Center and the Principal Investigator of the International Registry of Congenital Portosystemic Shunts (IRCPSS).


Anna Baiges Anna Baiges is a physician researcher in Hepatology at Hospital Clínic de Barcelona, Spain. Her research interest covers portal hypertension and vascular liver diseases, with a special interest in the adult manifestations of congenital portosystemic shunts.
Maxime Ronot Maxime Ronot, MD, PhD, is professor at the Université de Paris, and works in the Department of Medical Imaging at the Beaujon University Hospital in Clichy, France. His main interests are in abdominal imaging, mainly liver and pancreas diseases and tumors, interventional abdominal oncology, and abdominal vascular diseases.
Laurent Savale Laurent Savale, MD, PhD, is professor of respiratory medicine at the French Referral Centre for Pulmonary Hypertension, Department of Respiratory and Intensive Care Medicine, Bicêtre University Hospital. He leads the intensive care unit, and is also involved in the INSERM Research Unit “Pulmonary Hypertension: Pathophysiology and Innovative Therapies”.