Hereditary Hemorrhagic Telangiectasia and Refractory Ascites

Hereditary hemorrhagic telangiectasia is a rare autosomal dominant disease that can involve the liver. The presence of arteriohepatic venous shunts can lead to high output cardiac failure and biliary ischemia, whereas arterioportal venous shunts can result in portal hypertension. Cirrhosis and nodular regenerative hyperplasia are also reported in these patients. Management of these patients in the setting of symptomatic liver disease is challenging. Transarterial embolization and hepatic artery ligation are usually considered palliative options. In selected cases, orthotopic liver transplantation can cure both liver disease and heart failure.