Abstract
Hereditary hemorrhagic telangiectasia (HHT), or Rendu-Osler-Weber syndrome, is an autosomal dominant vascular disorder with low prevalence that presents a wide variety of clinical manifestations due to multiple vascular lesions in a wide variety of organs and tissues. Orthotopic liver transplantation is the only treatment option for arteriovenous malformation in HHT. These patients require complicated anesthetic management. We present the case of a 63-year-old woman with HHT of 18 years’ evolution, intrahepatic portal systemic shunts, and severe pulmonary hypertension, with dyspnea at minimum effort.
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