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Fontan-associated protein-losing enteropathy and heart transplant: A Pediatric Heart Transplant Study analysis
Kurt R Schumacher1, Jeffrey Gossett2, Kristine Guleserian3
1Department of Pediatrics, Mott Children's Hospital, University of Michigan, Ann Arbor, Michigan.
Insights
Protein-losing enteropathy (PLE) in Fontan patients does not increase waiting list or heart transplant mortality. Pediatric PLE patients show similar outcomes to non-PLE patients, suggesting PLE alone is not a significant risk factor.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Organ Transplantation
Background:
- Protein-losing enteropathy (PLE) is a serious complication after the Fontan procedure, linked to increased morbidity and mortality.
- Heart transplantation (HTx) offers a potential cure for Fontan-associated PLE, but PLE may complicate pre- and post-transplant outcomes.
- This study investigates the impact of PLE on pediatric patients undergoing evaluation for or having undergone HTx.
Purpose of the Study:
- To analyze the influence of protein-losing enteropathy (PLE) on the outcomes of pediatric Fontan patients listed for heart transplantation (HTx).
- To assess the association between PLE and waiting list mortality, post-HTx survival, rejection, and infection rates.
- To determine if PLE diagnosis alone impacts HTx outcomes in a pediatric cohort.
Main Methods:
- A retrospective analysis of pediatric Fontan patients from the Pediatric Heart Transplant Study (1999-2012).
- Patients were stratified into groups with and without a diagnosis of protein-losing enteropathy (PLE).
- Statistical analysis was performed to compare waiting list and post-HTx outcomes, including mortality, rejection, and infection.
Main Results:
- Patients with PLE were older and had larger body surface areas compared to non-PLE patients.
- PLE patients exhibited lower waiting list mortality than their non-PLE counterparts (p < 0.0001).
- No significant differences were observed in post-HTx survival, time to first infection, or rejection between the PLE and non-PLE groups.
Conclusions:
- In this pediatric cohort, a diagnosis of protein-losing enteropathy (PLE) alone was not associated with increased waiting list mortality or post-heart transplantation (HTx) morbidity.
- Pediatric PLE patients demonstrated comparable outcomes to non-PLE patients after HTx.
- Further multicenter studies with detailed PLE-specific data are needed to fully elucidate the risks associated with PLE in the context of HTx.
Background:
Post-Fontan protein-losing enteropathy (PLE) is associated with significant morbidity and mortality. Although heart transplantation (HTx) can be curative, PLE may increase the risk of morbidity before and after HTx. This study analyzed the influence of PLE influence on waiting list and post-HTx outcomes in a pediatric cohort.
Methods:
Fontan patients listed for HTx and enrolled in the Pediatric Heart Transplant Study from 1999 to 2012 were stratified by a diagnosis of PLE, and the association of PLE with waiting list and post-HTx mortality, rejection, and infection was analyzed.
Results:
Compared with non-PLE Fontan patients (n = 260), PLE patients listed for HTx (n = 96) were older (11.9 years vs 7.6 years; p = 0.003), had a larger body surface area (1.1 m(2) vs 0.9 m(2); p = 0.0001), had lower serum bilirubin (0.5 vs 0.9 mg/dl; p = 0.01), lower B-type natriuretic peptide (59 vs 227 pg/ml; p = 0.006), and were less likely to be on a ventilator (3% vs 13%; p = 0.006). PLE patients had lower waiting list mortality than non-PLE Fontan patients (p < 0.0001). There were no intergroup differences for post-HTx survival or times to the first infection or rejection. PLE was not independently associated with increased post-HTx mortality at any time point.
Conclusions:
In this multicenter cohort, the diagnosis of PLE alone was not associated with increased waiting list mortality or post-HTx morbidity or mortality. Given the limitations of our data, this analysis suggests that PLE patients in the pediatric age group have outcomes similar to their non-PLE counterparts. Additional multicenter studies of PLE patients with targeted collection of PLE-specific information will be necessary to fully delineate the risks conferred by PLE for HTx.
