Rose K McGahan, Jonathan E Tang, Manoj H Iyer, Antolin S Flores, Leonid A Gorelik
{"title":"Combined Liver Kidney Transplant in Adult Patient With Alagille Syndrome and Pulmonary Hypertension.","authors":"Rose K McGahan, Jonathan E Tang, Manoj H Iyer, Antolin S Flores, Leonid A Gorelik","doi":"10.1177/10892532211008742","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":46500,"journal":{"name":"Seminars in Cardiothoracic and Vascular Anesthesia","volume":"25 3","pages":"191-195"},"PeriodicalIF":1.1000,"publicationDate":"2021-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/10892532211008742","citationCount":"4","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Seminars in Cardiothoracic and Vascular Anesthesia","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1177/10892532211008742","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2021/4/14 0:00:00","PubModel":"Epub","JCR":"Q3","JCRName":"ANESTHESIOLOGY","Score":null,"Total":0}
引用次数: 4
Abstract
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.