The Ohio State University Wexner Medical Center, Columbus, OH, USA.
Semin Cardiothorac Vasc Anesth. 2021 Sep;25(3):191-195. doi: 10.1177/10892532211008742. Epub 2021 Apr 14.
In this article, we describe a case of a 33-year-old female with Alagille syndrome complicated by bilateral branch pulmonary artery stenosis resulting in moderate pulmonary hypertension, end-stage liver disease complicated by portal hypertension, and chronic renal disease who presented for combined liver-kidney transplant. Alagille syndrome is an autosomal dominant disease affecting the liver, heart, and kidneys. Multidisciplinary preoperative evaluation was performed with a team consisting of a congenital heart disease cardiologist, a cardiac anesthesiologist, a nephrologist, and a transplant surgeon. We describe Alagille syndrome and our intraoperative management. To our knowledge, this is the first description of a combined liver-kidney transplant in an adult patient with Alagille syndrome.
本文描述了一位 33 岁女性病例，患有 Alagille 综合征，并发双侧肺动脉分支狭窄导致中度肺动脉高压、终末期肝病合并门静脉高压和慢性肾脏病，需要进行肝肾联合移植。Alagille 综合征是一种常染色体显性遗传疾病，影响肝脏、心脏和肾脏。由先天性心脏病心脏病专家、心脏麻醉师、肾脏病专家和移植外科医生组成的多学科团队进行了术前评估。我们描述了 Alagille 综合征及其术中管理。据我们所知，这是首例 Alagille 综合征成人患者进行肝肾联合移植的描述。
