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