부산대학교 도서관

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. · Rendu-Osler-Weber syndrome is an infrequent pathology with few recorded cases of patients with liver transplantation and without specific clinical practice guidelines for anesthetic management. · Rendu-Osler-Weber syndrome is a complicated multiorgan alteration that requires multidisciplinary management: hepatologists, cardiologists, anesthesiologists, liver transplant surgeons, and so on. · Intraoperative anesthetic management is a challenge. [ABSTRACT FROM AUTHOR]