Morbidity and Mortality in Adult Fontan Patients After Heart or Combined Heart-Liver Transplantation

Matthew J. Lewis, Leigh C. Reardon, Jamil Aboulhosn, Christiane Haeffele, Sharon Chen, Yuli Kim, Stephanie Fuller, Lisa Forbess, Laith Alshawabkeh, Marcus A. Urey, Wendy M. Book, Fred Rodriguez, Jonathan N. Menachem, Daniel E. Clark, Anne Marie Valente, Matthew Carazo, Alexander Egbe, Heidi M. Connolly, Eric V. Krieger, Jilian AngiuloAri Cedars, Jong Ko, Roni M. Jacobsen, Michael G. Earing, Jonathan W. Cramer, Peter Ermis, Christopher Broda, Natalia Nugaeva, Heather Ross, Jordan D. Awerbach, Richard A. Krasuski, Marlon Rosenbaum

Research output: Contribution to journalArticlepeer-review

10 Scopus citations


Background: An increasing number of adult Fontan patients require heart transplantation (HT) or combined heart-liver transplant (CHLT); however, data regarding outcomes and optimal referral time remain limited. Objectives: The purpose of this study was to define survivorship post-HT/CHLT and predictors of post-transplant mortality, including timing of referral, in the adult Fontan population. Methods: A retrospective cohort study of adult Fontan patients who underwent HT or CHLT across 15 centers in the United States and Canada was performed. Inclusion criteria included the following: 1) Fontan; 2) HT/CHLT referral; and 3) age ≥16 years at the time of referral. Date of “failing” Fontan was defined as the earliest of the following: worsening fluid retention, new ascites, refractory arrhythmia, “failing Fontan” diagnosis by treating cardiologist, or admission for heart failure. Results: A total of 131 patients underwent transplant, including 40 CHLT, from 1995 to 2021 with a median post-transplant follow-up time of 1.6 years (Q1 0.35 years, Q3 4.3 years). Survival was 79% at 1 year and 66% at 5 years. Survival differed by decade of transplantation and was 87% at 1 year and 76% at 5 years after 2010. Time from Fontan failure to evaluation (HR/year: 1.23 [95% CI: 1.11-1.36]; P < 0.001) and markers of failure, including NYHA functional class IV (HR: 2.29 [95% CI: 1.10-5.28]; P = 0.050), lower extremity varicosities (HR: 3.92 [95% CI: 1.68-9.14]; P = 0.002), and venovenous collaterals (HR: 2.70 [95% CI: 1.17-6.20]; P = 0.019), were associated with decreased post-transplant survival at 1 year in a bivariate model that included transplant decade. Conclusions: In our multicenter cohort, post-transplant survival improved over time. Late referral after Fontan failure and markers of failing Fontan physiology, including worse functional status, lower extremity varicosities, and venovenous collaterals, were associated with post-transplant mortality.

Original languageEnglish (US)
Pages (from-to)2161-2171
Number of pages11
JournalJournal of the American College of Cardiology
Issue number22
StatePublished - Jun 6 2023


  • Fontan-associated liver disease
  • adult Fontan
  • combined heart-liver transplant
  • heart transplant
  • referral timing

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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