Angiography of hepatic vascular malformations associated with hereditary hemorrhagic telangiectasia

Purpose: To describe the angiographic features of hepatic involvement in hereditary hemorrhagic telangiectasia (HHT), particularly the presence of portovenous shunts. Methods: We reviewed the angiographic findings of seven patients with HHT. The patients comprised three women and four men with a mean age of 51 years. Results: Intrahepatic telangiectasias were found in all seven patients and shunts between three vascular channels were found in six of seven patients. In the four patients who had portovenous shunts combined with arterioportal shunts, the portovenous shunts were large. Three patients had no portovenous shunts. Two of these patients had arteriovenous shunts, and one had no shunt. The mean age (69 years) of the patients with portovenous shunts was older than those without portovenous shunts (26 years). Conclusions: Hepatic vascular lesions in HHT are varied, ranging from telangiectasias to large shunts between three vascular channels. In an advanced stage of involvement, large portovenous shunts are present.