Fontan-associated protein-losing enteropathy and post‒heart transplant outcomes: A multicenter study

Kurt R. Schumacher, SunkyungYu, Ryan Butts, Chesney Castleberry, Sharon Chen, Erik Edens, Justin Godown, Jonathan Johnson, Mariska Kemna, Kimberly Lin, Ray Lowery, Kathleen Simpson, Shawn West, Ivan Wilmot, Jeffrey G. Gossett

Research output: Contribution to journalArticlepeer-review

37 Scopus citations


BACKGROUND: The influence of Fontan-associated protein-losing enteropathy's (PLE) severity, duration, and treatment on heart transplant (HTx) outcomes is unknown. We hypothesized that long-standing PLE and PLE requiring more intensive therapy are associated with increased post-HTx mortality. METHODS: This 12-center, retrospective cohort study of post-Fontan patients with PLE referred for HTx from 2003 to 2015 involved collection of demographic, medical, surgical, and catheterization data, as well as PLE-specific data, including duration of disease, intensity/details of treatment, hospitalizations, and complications. Factors associated with waitlist and post-HTx outcomes and PLE resolution were sought. RESULTS: Eighty patients (median of 5 per center) were referred for HTx evaluation. Of 68 patients listed for HTx, 8 were removed due to deterioration, 4 died waiting, and 4 remain listed. In 52 patients undergoing HTx, post-HTx 1-month survival was 92% and 1-year survival was 83%. PLE-specific factors, including duration of PLE pre-HTx, pre-HTx hospitalizations, need for/frequency of albumin replacement, PLE therapies, and growth parameters had no association with post-HTx mortality. Immunosuppressant regimen was associated with mortality; standard mycophenolate mofetil immunotherapy was used in 95% of survivors compared with only 44% of non-survivors (p = 0.03). Rejection (53%) and infection (42%) post-HTx were common, but not associated with PLE-specific factors. PLE resolved completely in all but 1 HTx survivor at a median of 1 month (interquartile range 1 to 3 months); resolution was not affected by PLE-specific factors. CONCLUSIONS: PLE severity, duration, and treatment do not influence post-HTx outcome, but immunosuppressive regimen may have an impact on survival. PLE resolves in nearly all survivors.

Original languageEnglish (US)
Pages (from-to)17-25
Number of pages9
JournalJournal of Heart and Lung Transplantation
Issue number1
StatePublished - Jan 2019


  • Fontan
  • congenital heart disease, protein-losing enteropathy
  • heart transplant
  • pediatric transplant
  • protein-losing enteropathy

ASJC Scopus subject areas

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine
  • Transplantation


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