Symptomatic liver involvement in HHT is uncommon but does occur. It has been attributed to three distinct clinical subtypes and is believed to be a consequence of the predominant hepatic shunt pattern:

• high-output cardiac failure
  • shunting that increases cardiac preload
  • typically arteriovenous or portovenous shunts
• portal hypertension
  • increased flow into the portal system (arterioportal shunt)
  • hepatic anatomic abnormalities leading to increased intrahepatic resistance
• biliary disease
  • shunting of the blood away from the peribiliary plexus (arteriovenous or arterioportal shunting)
  • our case – extensive arteriovenous shunting lead to biliary necrosis and bile leak