[Rendu-Osler disease with hepatic involvement: first transplant in Spain]

Rendu-Osler's disease (RO) is a rare systemic vascular disorder due to a fibrovascular dysplasia in the endothelium of vessels. Recurrent epistaxis is the main clinical manifestation, but arteriovenous malformations (AVMs) can involve many organs, including the liver. Hepatic involvement can develop refractory heart failure due to large shunts between the hepatic veins and the hepatic artery. Embolization and hepatic artery ligation have also demonstrated to reduce cardiac output in RO, but these therapeutic options have significant morbidity and complications such as necrosis or liver failure.

We report the case of a 48 years old woman diagnosed in 1987 with RO and significant hepatic involvement, with multiple fistulas between veins and hepatic artery. In the following years she developed progressive heart failure that limited her quality of life.

She was admitted on more times with heart failure and her dyspnea worsened progressively up to NYHA IV. At this time, an echocardiograph control showed an output cardiac about 10.6l/min. On December 2004, although the medical treatment, the worsening of the patient went on, so we finally decided to conduct a liver transplant that resolved the symptoms and the hyperdynamic circulation. Despite the fact that liver transplant has become without doubt into the best treatment for these patients in the last years.

This is the first one done in Spain. There are different therapies available for these patients, but the indications for transplantation are greater each day, mainly due to the risks of the other options. Currently the stated guidelines are heart failure and portal hypertension refractory to medical treatment. So in these situations, liver transplantation should be proposed in the early stages of the disease and may be the only viable option.