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J Vasc Interv Radiol. 2010 Jan;21(1):135-9. doi: 10.1016/j.jvir.2009.09.009. Epub 2009 Nov 18.

Transjugular intrahepatic portosystemic shunt for variceal hemorrhage due to recurrent of hereditary hemorrhagic telangiectasia in a liver transplant.

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Department of Radiology, University of Texas Health Science Center at San Antonio, San Antonio, TX 78229, USA. <>


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.

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