Transjugular Intrahepatic Portosystemic Shunt for Variceal Hemorrhage due to Recurrent of Hereditary Hemorrhagic Telangiectasia in a Liver Transplant

• Published in: Journal of vascular and interventional radiology Vol. 21; no. 1; pp. 135 - 139
• Main Authors: Cura, Marco A., MD, Postoak, Darren, MD, Speeg, Kermit V., MD, Vasan, Rajiv, MD
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 2010
• Subjects: Gastrointestinal Hemorrhage - etiology; Gastrointestinal Hemorrhage - prevention & control; Humans; Liver Transplantation - adverse effects; Male; Middle Aged; Portasystemic Shunt, Transjugular Intrahepatic; Radiology; Secondary Prevention; Telangiectasia, Hereditary Hemorrhagic - complications; Telangiectasia, Hereditary Hemorrhagic - surgery; Treatment Outcome; portal vein; portosystemic gradient; AV; HHT; PSG; transjugular intrahepatic portosystemic shunt; OLT; PV; arteriovenous; hereditary hemorrhagic telangiectasia; orthotopic liver transplantation; TIPS
• ISSN: 1051-0443; 1535-7732
• DOI: 10.1016/j.jvir.2009.09.009

Hepatic involvement in hereditary hemorrhagic telangiectasia (HHT) consists of vascular malformations associated with arteriovenous (AV), arterioportal, and/or portovenous shunting. Most patients with HHT have liver involvement. Symptoms, although rare, consist of cardiac failure, pulmonary hypertension, portal hypertension, portosystemic encephalopathy, cholangitis, and atypical cirrhosis. Reported treatments for symptomatic AV malformations have been associated with substantial morbidity and mortality. This report describes a case of hepatic HHT that required liver transplantation after hepatic artery embolization. Recurrent vascular malformations developed in the transplant, resulting in portal hypertension and life-threatening variceal hemorrhage that was controlled with transjugular intrahepatic portosystemic shunt creation.