Angiography of Hepatic vascular malformations associated with hereditary hemorrhagic telangiectasia

• Published in: Cardiovascular and interventional radiology 2003-03, Vol.26 (2), p.177-180
• Main Authors: HASHIMOTO, Manabu, TATE, Etuko, NISHII, Toshiaki, WATARAI, Jiro, SHIOYA, Takanobu, WHITE, Robert I
• Format: Article
• Language: English
• Subjects: Adult; Aged; Arteriovenous Malformations - diagnosis; Arteriovenous Malformations - etiology; Arteriovenous Malformations - surgery; Arteriovenous Shunt, Surgical; Biological and medical sciences; BIOMEDICAL RADIOGRAPHY; BLOOD VESSELS; BYPASSES; Female; Gastroenterology. Liver. Pancreas. Abdomen; Hepatic Artery - abnormalities; Hepatic Artery - surgery; Hepatic Veins - abnormalities; Hepatic Veins - surgery; Humans; LIVER; Liver. Biliary tract. Portal circulation. Exocrine pancreas; Male; MALFORMATIONS; Medical sciences; Middle Aged; PATIENTS; Portal Vein - abnormalities; Portal Vein - surgery; Portasystemic Shunt, Surgical; RADIOLOGY AND NUCLEAR MEDICINE; Retrospective Studies; Severity of Illness Index; Splenic Artery - abnormalities; Splenic Artery - surgery; Telangiectasia, Hereditary Hemorrhagic - diagnosis; Telangiectasia, Hereditary Hemorrhagic - etiology; Telangiectasia, Hereditary Hemorrhagic - surgery; Treatment Outcome; WOMEN
• ISSN: 0174-1551; 1432-086X
• DOI: 10.1007/s00270-002-1507-Y

To describe the angiographic features of hepatic involvement in hereditary hemorrhagic telangiectasia (HHT), particularly the presence of portovenous shunts. 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. 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). 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.