Long-Term Outcomes and Trends in Liver Transplantation for Hereditary Hemochromatosis in the United States

Authors

Peter Lymberopoulos
Sameer Prakash
Anjiya Shaikh
Anshul Bhatnagar
Anthony K Allam
Karthik Goli
John A Goss
Fasiha Kanwal
Abbas Rana
Kris V Kowdley
Prasun Jalal
George Cholankeril

Publication Date

1-1-2023

Journal

Liver Transplant

DOI

10.1002/lt.26539

PMID

35770428

PMCID

PMC9800641

PubMedCentral® Posted Date

1-14-2023

PubMedCentral® Full Text Version

Author MSS

Published Open-Access

yes

Keywords

Adult, Humans, United States, Hemochromatosis, Liver Transplantation, Liver Diseases, Proportional Hazards Models, Retrospective Studies

Abstract

There have been conflicting data regarding liver transplantation (LT) outcomes for hereditary hemochromatosis (HH), with no recent data on LT outcomes in patients with HH in the past decade. Using the United Network for Organ Sharing registry, we evaluated waitlist and post-LT survival in all adult patients listed for HH without concomitant liver disease from 2003 to 2019. Post-LT survival for HH was compared with a propensity-matched (recipient and donor factors) cohort of recipients with chronic liver disease (CLD). From 2003 to 2019, 862 patients with HH were listed for LT, of which 55.6% ( n = 479) patients underwent LT. The 1- and 5-year post-LT survival rates in patients with HH were 88.7% (95% confidence interval [CI], 85.4%-91.4%) and 77.5% (95% CI, 72.8%-81.4%), respectively, and were comparable with those in the propensity-matched CLD cohort ( p value = 0.96). Post-LT survival for HH was lower than for Wilson's disease, another hereditary metabolic liver disease with similar LT volume ( n = 365). Predictors for long-term (5-year) post-LT mortality included presence of portal vein thrombosis (hazard ratio [HR], 1.96; 95% CI, 1.07-3.58), obesity measurements greater than Class II (HR, 1.98; 95% CI, 1.16-3.39), and Karnofsky performance status (HR, 0.98; 95% CI, 0.97-0.99) at the time of LT. The leading cause of post-LT death ( n = 145) was malignancy (25.5%), whereas cardiac disease was the cause in less than 10% of recipients. In conclusion, short- and long-term survival rates for HH are excellent and comparable with those of other LT recipients. Improving extrahepatic metabolic factors and functional status in patients with HH prior to LT may improve outcomes.

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