患有遗传性出血性毛细血管扩张症患者肝移植的麻醉考量：一例报告

Anesthetic Considerations in Hepatic Transplant in a Patient With Rendu-Osler-Weber Syndrome: A Case Report.

作者信息

Morales Vicente Padilla, Tuñón Ordoño Alonso, Fuentes Pradera María Ángeles, Linero Inmaculada Benítez

机构信息

Department of Anesthesiology, Resuscitation and Pain Management, Virgen del Rocío University Hospital, Seville, Spain.

出版信息

Transplant Proc. 2020 Mar;52(2):589-591. doi: 10.1016/j.transproceed.2019.12.014. Epub 2020 Feb 12.

DOI:10.1016/j.transproceed.2019.12.014

PMID:32059941

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.

摘要

遗传性出血性毛细血管扩张症（HHT），又称伦杜-奥斯勒-韦伯综合征，是一种常染色体显性遗传性血管疾病，发病率较低，由于多种器官和组织中存在多发性血管病变，可呈现出各种各样的临床表现。原位肝移植是HHT患者动静脉畸形的唯一治疗选择。这些患者需要复杂的麻醉管理。我们报告一例63岁女性患者，患有病程长达18年的HHT，存在肝内门体分流和严重肺动脉高压，轻微活动即出现呼吸困难。