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

Vivek N. Iyer, Behnam Saberi, Julie K. Heimbach, Joseph J. Larson, Suresh Raghavaiah, Ivo Ditah, Karen Swanson, Patrick Sequeira Kamath, K. D. Watt, Timucin Taner, Michael Joseph Krowka, Michael D. Leise

Research output: Contribution to journalArticle

Abstract

BACKGROUND: Liver arteriovenous malformations (AVM) in hereditary hemorrhagic telangiectasia (HHT) can necessitate liver transplantation. There is limited data on HHT patients undergoing liver transplantation (LT) in the United States. METHODS: Two sources of data were used: (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 United States 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 to 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 years (interquartile range, 37.0-58.5 years). Median calculated MELD score at time of LT was 8.0 (interquartile range, 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, whereas 2 patients were lost to follow-up. Five of the aforementioned HHT patients underwent LT at Mayo Clinic, 4 with high output cardiac failure, 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 after 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.

Original languageEnglish (US)
Pages (from-to)1418-1424
Number of pages7
JournalTransplantation
Volume103
Issue number7
DOIs
StatePublished - Jul 1 2019

Fingerprint

Hereditary Hemorrhagic Telangiectasia
Liver Transplantation
Databases
Registries
Heart Failure
Transplants
Liver
Arteriovenous Malformations
Information Storage and Retrieval
Lost to Follow-Up
Ischemia

ASJC Scopus subject areas

  • Transplantation

Cite this

Iyer, V. N., Saberi, B., Heimbach, J. K., Larson, J. J., Raghavaiah, S., Ditah, I., ... Leise, M. D. (2019). Liver Transplantation Trends and Outcomes for Hereditary Hemorrhagic Telangiectasia in the United States. Transplantation, 103(7), 1418-1424. https://doi.org/10.1097/TP.0000000000002491

Liver Transplantation Trends and Outcomes for Hereditary Hemorrhagic Telangiectasia in the United States. / Iyer, Vivek N.; Saberi, Behnam; Heimbach, Julie K.; Larson, Joseph J.; Raghavaiah, Suresh; Ditah, Ivo; Swanson, Karen; Kamath, Patrick Sequeira; Watt, K. D.; Taner, Timucin; Krowka, Michael Joseph; Leise, Michael D.

In: Transplantation, Vol. 103, No. 7, 01.07.2019, p. 1418-1424.

Research output: Contribution to journalArticle

Iyer, VN, Saberi, B, Heimbach, JK, Larson, JJ, Raghavaiah, S, Ditah, I, Swanson, K, Kamath, PS, Watt, KD, Taner, T, Krowka, MJ & Leise, MD 2019, 'Liver Transplantation Trends and Outcomes for Hereditary Hemorrhagic Telangiectasia in the United States', Transplantation, vol. 103, no. 7, pp. 1418-1424. https://doi.org/10.1097/TP.0000000000002491
Iyer, Vivek N. ; Saberi, Behnam ; Heimbach, Julie K. ; Larson, Joseph J. ; Raghavaiah, Suresh ; Ditah, Ivo ; Swanson, Karen ; Kamath, Patrick Sequeira ; Watt, K. D. ; Taner, Timucin ; Krowka, Michael Joseph ; Leise, Michael D. / Liver Transplantation Trends and Outcomes for Hereditary Hemorrhagic Telangiectasia in the United States. In: Transplantation. 2019 ; Vol. 103, No. 7. pp. 1418-1424.
@article{c5ab2f029803417fb01c91c3d084c0a7,
title = "Liver Transplantation Trends and Outcomes for Hereditary Hemorrhagic Telangiectasia in the United States",
abstract = "BACKGROUND: Liver arteriovenous malformations (AVM) in hereditary hemorrhagic telangiectasia (HHT) can necessitate liver transplantation. There is limited data on HHT patients undergoing liver transplantation (LT) in the United States. METHODS: Two sources of data were used: (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 United States 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 to 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 years (interquartile range, 37.0-58.5 years). Median calculated MELD score at time of LT was 8.0 (interquartile range, 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, whereas 2 patients were lost to follow-up. Five of the aforementioned HHT patients underwent LT at Mayo Clinic, 4 with high output cardiac failure, 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 after 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.",
author = "Iyer, {Vivek N.} and Behnam Saberi and Heimbach, {Julie K.} and Larson, {Joseph J.} and Suresh Raghavaiah and Ivo Ditah and Karen Swanson and Kamath, {Patrick Sequeira} and Watt, {K. D.} and Timucin Taner and Krowka, {Michael Joseph} and Leise, {Michael D.}",
year = "2019",
month = "7",
day = "1",
doi = "10.1097/TP.0000000000002491",
language = "English (US)",
volume = "103",
pages = "1418--1424",
journal = "Transplantation",
issn = "0041-1337",
publisher = "Lippincott Williams and Wilkins",
number = "7",

}

TY - JOUR

T1 - Liver Transplantation Trends and Outcomes for Hereditary Hemorrhagic Telangiectasia in the United States

AU - Iyer, Vivek N.

AU - Saberi, Behnam

AU - Heimbach, Julie K.

AU - Larson, Joseph J.

AU - Raghavaiah, Suresh

AU - Ditah, Ivo

AU - Swanson, Karen

AU - Kamath, Patrick Sequeira

AU - Watt, K. D.

AU - Taner, Timucin

AU - Krowka, Michael Joseph

AU - Leise, Michael D.

PY - 2019/7/1

Y1 - 2019/7/1

N2 - BACKGROUND: Liver arteriovenous malformations (AVM) in hereditary hemorrhagic telangiectasia (HHT) can necessitate liver transplantation. There is limited data on HHT patients undergoing liver transplantation (LT) in the United States. METHODS: Two sources of data were used: (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 United States 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 to 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 years (interquartile range, 37.0-58.5 years). Median calculated MELD score at time of LT was 8.0 (interquartile range, 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, whereas 2 patients were lost to follow-up. Five of the aforementioned HHT patients underwent LT at Mayo Clinic, 4 with high output cardiac failure, 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 after 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.

AB - BACKGROUND: Liver arteriovenous malformations (AVM) in hereditary hemorrhagic telangiectasia (HHT) can necessitate liver transplantation. There is limited data on HHT patients undergoing liver transplantation (LT) in the United States. METHODS: Two sources of data were used: (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 United States 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 to 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 years (interquartile range, 37.0-58.5 years). Median calculated MELD score at time of LT was 8.0 (interquartile range, 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, whereas 2 patients were lost to follow-up. Five of the aforementioned HHT patients underwent LT at Mayo Clinic, 4 with high output cardiac failure, 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 after 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.

UR - http://www.scopus.com/inward/record.url?scp=85068820156&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85068820156&partnerID=8YFLogxK

U2 - 10.1097/TP.0000000000002491

DO - 10.1097/TP.0000000000002491

M3 - Article

C2 - 30335701

AN - SCOPUS:85068820156

VL - 103

SP - 1418

EP - 1424

JO - Transplantation

JF - Transplantation

SN - 0041-1337

IS - 7

ER -