Abstract 17178: The Influence of Fontan-associated Protein-losing Enteropathy on Outcomes in Patients Referred for Heart Transplant: a Multicenter Study

IntroductionFontan-associated protein-losing enteropathy (PLE) is an indication for heart transplant (HTx). Timing of referral varies widely, and the influence of PLE’s severity, duration, and treatment on HTx outcomes is unknown.HypothesisLong-standing PLE and PLE requiring more intensive therapy are associated with increased post-HTx mortality.MethodsThis is a 12-center, retrospective cohort study of all post-Fontan pts with PLE referred for HTx from 2003-2015. Demographic, medical, surgical, and catheterization, as well as PLE-specific data, including duration of disease, intensity and details of treatment, hospitalizations, growth, and complications, were collected. Factors associated with waitlist and post-HTx outcomes including death, rejection, infection, and PLE resolution were sought.ResultsEighty pts (median 5/center, range 1-20) were referred for HTx evaluation. Median time from Fontan to PLE diagnosis was 4.5 yr (IQR 1.2-8.6 yr) and from diagnosis to evaluation was 1.5 yr (IQR 0.3-6.5 yr). Of 68 pts listed for HTx, 8 were removed due to deterioration, 4 died waiting, and 4 remain listed. In 52 pts undergoing HTx, median time from PLE diagnosis to HTx was 2.4 yr (IQR 1-4.6 yr). Post-HTx 1-mo survival was 92% and 1-yr was 83%. PLE-specific factors including duration of PLE prior to HTx, pre-HTx hospitalizations, need for/frequency of albumin replacement, PLE-specific therapies, and weight or height z-score had no association with post-HTx mortality. Deviation from institution standard post-HTx immunosuppressant regimen was associated with increased mortality (p=.04). Specifically, withholding MMF, azathioprine, or mTOR inhibitor occurred in 56% of deaths vs. 9% of survivors (p=.005). Rejection (53%) and infection (44%) at any time post-HTx were common, but not associated with PLE-specific factors. PLE resolved completely in all but one HTx survivor at a median of 1 mo (IQR 1-3, range 0-20 mo). Duration, severity, or prior treatment did not affect time to PLE resolution.ConclusionsIndividuals with PLE have a risk of post-HTx mortality similar to published outcomes for other Fontan pts, and PLE resolves in nearly all survivors. PLE severity, duration, and treatment do not influence post-HTx outcome.