Liver Transplantation Trends and Outcomes for Hereditary Hemorrhagic Telangiectasia In the United States

Introduction Liver arteriovenous malformations (AVM) in Hereditary Hemorrhagic Telangiectasia (HHT) can necessitate liver transplantation. There is limited data on HHT patients undergoing LT in the United States. Methods Two sources of data were utilized: (1) Scientific Registry of Transplant Recipients (SRTR) database (1998-2016) (2) Single center liver transplant database (Mayo Clinic Rochester, MN). The aims of this study were (1) To determine trends in LT for HHT related liver involvement in the US using the SRTR database (2) To identify clinical characteristics, indications, and outcomes for LT in HHT. Results Thirty-nine HHT patients were listed for LT in the SRTR database from 1998-2016 – 1998-2001 (n = 1); 2002-2005 (n=4); 2006-2010 (n=10) and 2011-2016 (n=24). Twenty-four underwent LT at a median age of 47.5(IQR 37.0, 58.5). Median calculated MELD score at time of LT was 8.0 (IQR 7.0, 9.5) and, 75% received an exception MELD score. Two status 1 patients died during transplant surgery. Nineteen (86%) patients were alive after a median post-LT follow-up of 48 months while 2 patients were lost to follow-up. Five of the aforementioned HHT patients underwent LT at Mayo Clinic, 4 with HOCF and 1 with biliary ischemia. All 5 were alive at the time of last follow-up with good graft function and resolution of heart failure. Conclusions Outcomes following LT for HHT patients are excellent with 86% survival after a median follow-up of 48 months and resolution of heart failure. LT listing for HHT has increased in substantially in more recent eras. Denotes co-first authorship –authors contributed equally to this work, Vivek N. Iyer, Behnam Saberi MD Correspondence: Michael D. Leise, Division of Gastroenterology and Hepatology, Mayo Clinic, 200 First street SW, Rochester, MN 55905; E-mail: Leise.Michael@mayo.edu Conflict of interest: None Financial Support: None • Participated in research design -Vivek Iyer, Behnam Saberi, Julie Heimbach, Joseph Larson, Ivo Ditah, Suresh Raghavaiah, Patrick Kamath, KD Watt, Timucin Taner, Michael Krowka, Michael Leise • Participated in the writing of the paper -Vivek Iyer, Behnam Saberi, Julie Heimbach, Joseph Larson, Ivo Ditah, Suresh Raghavaiah, Karen Swanson, Patrick Kamath, KD Watt, Timucin Taner, Michael Krowka, Michael Leise • Participated in the performance of the research -Vivek Iyer, Behnam Saberi, Julie Heimbach, Joseph Larson, Suresh Raghavaiah, Patrick -Kamath, KD Watt, Timucin Taner, Michael Krowka, Michael Leise • Contributed new reagents or analytic tools N/A • Participated in data analysis -Vivek Iyer, Behnam Saberi, Julie Heimbach, Joseph Larson, Ivo Ditah, Suresh Raghavaiah, Karen Swanson, Patrick Kamath, KD Watt, Timucin Taner, Michael Krowka, Michael Leise Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

https://ift.tt/2ypS0jV