Vascular malformations in HHT predominantly consist in shunts from the hepatic artery to the hepatic veins; direct connections between portal and hepatic veins are less frequent. The typical clinical manifestations depend on the predominance of such shunts. High-output heart failure results from an anastomosis between the hepatic artery and hepatic veins; portal hypertension is caused by shunts from the hepatic artery to the portal vein; porto-systemic encefalopathy is the result of portal to hepatic vein shunting. Finally, anicteric cholestasis is probably due to a hypoperfusion of the peribiliary plexus.[20]

Recently, the abdominal CT multislice scan has been performed in a prospective study revealing that 74% of HHT patients have liver AVMs, most frequently asymptomatic. Doppler ultrasonography has been proposed as the ideal tool for the screening and follow-up of HHT patients with liver involvement. Selective angiography of the hepatic artery is considered the gold standard for diagnosis of hepatic shunting although abdominal CT multislice scan has been demonstrated to be very accurate, safer, cheaper, and better tolerated by patients.[21]

Treatment options for liver involvement in HHT are rather limited. Segmental embolotherapy of the branch hepatic arteries has led to controversial results because of severe complications, such as liver necrosis. Liver transplantation has been performed with good results.[20]

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