[Hereditary hemorrhagic telangiectasia with hepatic involvement and including gastric vessels. A case report]

Hereditary hemorrhagic teleangiectasia (HHT) (Osler-Weber-Rendu-disease) is characterized by a combination of muccocutaneous vascular malformations with recurrent spontaneous bleeding and a familiar predisposition. Among visceral manifestations of this disease are only a few descriptions of hepatic involvement. We describe a 53-year-old woman with HHT whose first symptoms of hypercirculatory heart failure developed 1.5 years before the final diagnosis of HHT. We measured a heart-time-volume of 14-151/min. by echocardiography and a flow-volume of 5-9 l/min. in the proper hepatic artery by dopplersonography on admission. We were able to demonstrate multiple intra- and extra-hepatic and gastric arteriovenous malformations by arteriography. Branches of the proper hepatic artery and the left gastric artery were embolized in three serial sessions. By this procedure we were able to reduce the flow-volume in the proper hepatic artery from 5-9l/min. to 1.5l/min. and the heart-time-volume to a minimum of 9.8l/min. and thus stop progression of the hyperdynamic heart failure.