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Long-term outcomes and trends in liver transplantation for hereditary hemochromatosis in the United States.

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.

SUBMITTER: Lymberopoulos P

PROVIDER: S-EPMC9800641 | biostudies-literature | 2023 Jan

REPOSITORIES: biostudies-literature

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Long-term outcomes and trends in liver transplantation for hereditary hemochromatosis in the United States.

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

Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society 20220721 1

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 chron ...[more]

PMID: 35770428

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Project description:ImportanceAlcohol-associated liver disease (ALD) has emerged as the most common indication for liver transplant in the United States, but data on the reasons for this increase and long-term post-liver transplant outcomes among liver transplant recipients are sparse.ObjectiveTo characterize trends and long-term outcomes of liver transplant for ALD in the United States between 2002 and 2016.Design, setting, and participantsThis multicenter, prospective, national cohort study used data from the United Network for Organ Sharing database to evaluate all liver transplants performed in the United States between January 1, 2002, and December 31, 2016.Main outcomes and measuresNational and regional trends in liver transplant for ALD, with a sensitivity analysis with hepatitis C virus (HCV) infection and hepatocellular carcinoma (HCC) included, and early (≤90 days after liver transplant) and late (>90 days after liver transplant) patient and graft survival.ResultsThe cohort consisted of 32 913 patients, including 9438 with ALD and 23 475 without ALD (patients who had HCV infection and HCC indications were excluded). Median age of patients with ALD was 54 years (interquartile range, 47-60 years) and of patients without ALD was 54 years (interquartile range, 44-61 years). Patients with ALD (vs non-ALD) were more frequently male (7197 of 9438 [76.2%] vs 11 767 of 23 475 [50.1%]; P < .001) and white (7544 [80.0%] vs 17 251 [73.5%]; P < .001). The proportion of liver transplants for ALD increased from 24.2% (433 of 1791) in 2002 to 27.2% (556 of 2044) in 2010 and 36.7% (1253 of 3419) in 2016. With HCV infection included, the proportions of liver transplant for ALD were 15.3% in 2002, 18.6% in 2010, and 30.6% in 2016, representing a 100% increase in liver transplant for ALD, of which 48% was associated with a decrease in HCV infection as an indication for liver transplant. The magnitude of increase in ALD was regionally heterogeneous and associated with changes in patient characteristics suggestive of alcoholic hepatitis: decreasing age (χ2 = 36.5; P = .005) and increasing model for end-stage liver disease score (χ2 = 69.1; P < .001). Cumulative unadjusted 5-year posttransplant survival was 79% (95% CI, 78%-80%) for ALD vs 80% (95% CI, 79%-80%) for non-ALD; cumulative unadjusted 10-year posttransplant survival was 63% (95% CI, 61%-64%) for ALD vs 68% (95% CI, 67%-69%) for non-ALD (P = .006). In multivariable analysis, ALD was associated with increased risk of late death after liver transplant (adjusted hazard ratio, 1.11; 95% CI, 1.03-1.20; P = .006).Conclusions and relevanceThe findings suggest that early liver transplant for alcoholic hepatitis may be leading to broader acceptance of ALD for liver transplant. Late survival among liver transplant recipients with ALD was inferior to that among recipients with non-ALD indications, suggesting a need for future studies to identify patient profiles associated with best outcomes. Regional differences suggest heterogeneity in policies toward liver transplant for ALD.

Project description:To compare long-term survival of living donor liver transplant (LDLT) at experienced transplant centers with outcomes of deceased donor liver transplant and identify key variables impacting patient and graft survival.The Adult-to-Adult Living Donor Liver Transplantation Cohort Study is a prospective multicenter National Institutes of Health study comparing outcomes of LDLT and deceased donor liver transplant and associated risks.Mortality and graft failure for 1427 liver recipients (963 LDLT) enrolled in the Adult-to-Adult Living Donor Liver Transplantation Cohort Study who received transplant between January 1, 1998, and January 31, 2014, at 12 North American centers with median follow-up 6.7 years were analyzed using Kaplan-Meier and multivariable Cox models.Survival probability at 10 years was 70% for LDLT and 64% for deceased donor liver transplant. Unadjusted survival was higher with LDLT (hazard ratio = 0.76, P = 0.02) but attenuated after adjustment (hazard ratio = 0.98, P = 0.90) as LDLT recipients had lower mean model for end-stage liver disease (15.5 vs 20.4) and fewer received transplant from intensive care unit, were inpatient, on dialysis, were ventilated, or with ascites. Posttransplant intensive care unit days were less for LDLT recipients. For all recipients, female sex and primary sclerosing cholangitis were associated with improved survival, whereas dialysis and older recipient/donor age were associated with worse survival. Higher model for end-stage liver disease score was associated with increased graft failure. Era of transplantation and type of donated lobe did not impact survival in LDLT.LDLT provides significant long-term transplant benefit, resulting in transplantation at a lower model for end-stage liver disease score, decreased death on waitlist, and excellent posttransplant outcomes. Recipient diagnosis, disease severity, renal failure, and ages of recipient and donor should be considered in decision making regarding timing of transplant and donor options.Clinical Trials ID: NCT00096733.

Dataset Information

Long-term outcomes and trends in liver transplantation for hereditary hemochromatosis in the United States.

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Long-term outcomes and trends in liver transplantation for hereditary hemochromatosis in the United States.

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