How is hepatic arteriovenous malformation (AVM) treated in Osler-Weber-Rendu disease (OWRD) (hereditary hemorrhagic telangiectasia [HHT])?

Updated: Oct 06, 2020
  • Author: Klaus-Dieter Lessnau, MD, FCCP; Chief Editor: Vincent Lopez Rowe, MD  more...
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Answer

Embolization of the hepatic artery is a potential option for selected patients with liver involvement, as is liver transplantation. [120, 121]

Liver transplantation may be considered in patients with symptomatic hepatic HHT who present with life-threatening conditions such as hepatobiliary sepsis and severe cardiopulmonary changes. [121] In a study involving 40 patients followed up for a median of 70 months, patient and graft actuarial survival was 82.5% at 10 years. Indications for transplantation included cardiac failure (n = 14), biliary necrosis with hepatic failure (n = 12), severe portal hypertension (n = 5), or a combination of two or more of these conditions (n = 9).

In a brief report of two HHT patients who underwent liver transplantation for cardiac failure, the patients had normal cardiac function 2 and 3 years after transplantation. [122] Hepatic embolization in patients with extensive liver involvement has been discouraged because of the potential complications of liver parenchymal or biliary necrosis with ensuing sepsis. [121]

Mitchell et al reported a case in which the use of bevacizumab reversed the need for liver transplantation in an HHT patient with heart failure from hepatic vascular malformations. [123]


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