Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2023 Jun 6;81(22):2149-2160.
doi: 10.1016/j.jacc.2023.03.421.

Clinical Outcomes of Adult Fontan-Associated Liver Disease and Combined Heart-Liver Transplantation

Matthew J Lewis  1 , Leigh C Reardon  2 , Jamil Aboulhosn  2 , Christiane Haeffele  3 , Sharon Chen  3 , Yuli Kim  4 , Stephanie Fuller  5 , Lisa Forbess  6 , Laith Alshawabkeh  7 , Marcus A Urey  7 , Wendy M Book  8 , Fred Rodriguez 3rd  8 , Jonathan N Menachem  9 , Daniel E Clark  9 , Anne Marie Valente  10 , Matthew Carazo  10 , Alexander Egbe  11 , Heidi M Connolly  11 , Eric V Krieger  12 , Jilian Angiulo  12 , Ari Cedars  13 , Jong Ko  13 , Roni M Jacobsen  14 , Michael G Earing  14 , Jonathan W Cramer  15 , Peter Ermis  16 , Christopher Broda  16 , Natalia Nugaeva  17 , Heather Ross  17 , Jordan D Awerbach  18 , Richard A Krasuski  19 , Marlon Rosenbaum  20
Affiliations
Free article

Clinical Outcomes of Adult Fontan-Associated Liver Disease and Combined Heart-Liver Transplantation

Matthew J Lewis et al. J Am Coll Cardiol. .
Free article

Abstract

Background: The impact of Fontan-associated liver disease (FALD) on post-transplant mortality and indications for combined heart-liver transplant (CHLT) in adult Fontan patients remains unknown.

Objectives: The purpose of this study was to assess the impact of FALD on post-transplant outcomes and compare HT vs CHLT in adult Fontan patients.

Methods: We performed a retrospective-cohort study of adult Fontan patients who underwent HT or CHLT across 15 centers. Inclusion criteria were as follows: 1) Fontan; 2) HT/CHLT referral; and 3) age ≥16 years at referral. Pretransplant FALD score was calculated using the following: 1) cirrhosis; 2) varices; 3) splenomegaly; or 4) ≥2 paracenteses.

Results: A total of 131 patients (91 HT and 40 CHLT) were included. CHLT recipients were more likely to be older (P = 0.016), have a lower hemoglobin (P = 0.025), require ≥2 diuretic agents pretransplant (P = 0.051), or be transplanted in more recent decades (P = 0.001). Postmatching, CHLT demonstrated a trend toward improved survival at 1 year (93% vs 74%; P = 0.097) and improved survival at 5 years (86% vs 52%; P = 0.041) compared with HT alone. In patients with a FALD score ≥2, CHLT was associated with improved survival (1 year: 85% vs 62%; P = 0.044; 5 years: 77% vs 42%; P = 0.019). In a model with transplant decade and FALD score, CHLT was associated with improved survival (HR: 0.33; P = 0.044) and increasing FALD score was associated with worse survival (FALD score: 2 [HR: 14.6; P = 0.015], 3 [HR: 22.2; P = 0.007], and 4 [HR: 27.8; P = 0.011]).

Conclusions: Higher FALD scores were associated with post-transplant mortality. Although prospective confirmation of our findings is necessary, compared with HT alone, CHLT recipients were older with higher FALD scores, but had similar survival overall and superior survival in patients with a FALD score ≥2.

Keywords: Fontan-associated liver disease; adult Fontan; combined heart-liver transplant; heart transplant.

PubMed Disclaimer

Conflict of interest statement

Funding Support and Author Disclosures This work was supported by the National Institutes of Health/National Heart, Lung, and Blood Institute (K23 HL1143136). Drs Valente and Carazo were supported by the Sarah Marie Liamos Fund for Adult Congenital Heart Disease Research. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Comment in

  • The Last Stage of the Fontan Operation?
    Stout K, Buber J. Stout K, et al. J Am Coll Cardiol. 2023 Jun 6;81(22):2172-2174. doi: 10.1016/j.jacc.2023.04.023. J Am Coll Cardiol. 2023. PMID: 37257952 No abstract available.

Publication types

LinkOut - more resources