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

Medicina clínica
Miguel Angel Núñez ViejoRoberto Zarrabeitia

Abstract

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 circulatio...Continue Reading

References

Apr 1, 1990·Journal of Clinical Gastroenterology·N BourgeoisE Lecocq
Oct 29, 1998·Liver Transplantation and Surgery : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·J S OdoricoM Kalayoglu
Sep 28, 2000·The New England Journal of Medicine·G Garcia-TsaoR I White
Mar 13, 2001·Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·C HillertX Rogiers
Jan 25, 2003·Journal of Clinical Gastroenterology·Anne M Larson

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