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Fontan-associated protein-losing enteropathy and heart transplant: A Pediatric Heart Transplant Study analysis.

Publication ,  Journal Article
Schumacher, KR; Gossett, J; Guleserian, K; Naftel, DC; Pruitt, E; Dodd, D; Carboni, M; Lamour, J; Pophal, S; Zamberlan, M; Gajarski, RJ
Published in: J Heart Lung Transplant
September 2015

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.

Duke Scholars

Published In

J Heart Lung Transplant

DOI

EISSN

1557-3117

Publication Date

September 2015

Volume

34

Issue

9

Start / End Page

1169 / 1176

Location

United States

Related Subject Headings

  • Ventilators, Mechanical
  • Treatment Outcome
  • Surgery
  • Protein-Losing Enteropathies
  • Postoperative Complications
  • Natriuretic Peptide, Brain
  • Male
  • Humans
  • Heart Transplantation
  • Graft Rejection
 

Citation

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Schumacher, K. R., Gossett, J., Guleserian, K., Naftel, D. C., Pruitt, E., Dodd, D., … Gajarski, R. J. (2015). Fontan-associated protein-losing enteropathy and heart transplant: A Pediatric Heart Transplant Study analysis. J Heart Lung Transplant, 34(9), 1169–1176. https://doi.org/10.1016/j.healun.2015.03.022
Schumacher, Kurt R., Jeffrey Gossett, Kristine Guleserian, David C. Naftel, Elizabeth Pruitt, Debra Dodd, Michael Carboni, et al. “Fontan-associated protein-losing enteropathy and heart transplant: A Pediatric Heart Transplant Study analysis.J Heart Lung Transplant 34, no. 9 (September 2015): 1169–76. https://doi.org/10.1016/j.healun.2015.03.022.
Schumacher KR, Gossett J, Guleserian K, Naftel DC, Pruitt E, Dodd D, et al. Fontan-associated protein-losing enteropathy and heart transplant: A Pediatric Heart Transplant Study analysis. J Heart Lung Transplant. 2015 Sep;34(9):1169–76.
Schumacher, Kurt R., et al. “Fontan-associated protein-losing enteropathy and heart transplant: A Pediatric Heart Transplant Study analysis.J Heart Lung Transplant, vol. 34, no. 9, Sept. 2015, pp. 1169–76. Pubmed, doi:10.1016/j.healun.2015.03.022.
Schumacher KR, Gossett J, Guleserian K, Naftel DC, Pruitt E, Dodd D, Carboni M, Lamour J, Pophal S, Zamberlan M, Gajarski RJ. Fontan-associated protein-losing enteropathy and heart transplant: A Pediatric Heart Transplant Study analysis. J Heart Lung Transplant. 2015 Sep;34(9):1169–1176.
Journal cover image

Published In

J Heart Lung Transplant

DOI

EISSN

1557-3117

Publication Date

September 2015

Volume

34

Issue

9

Start / End Page

1169 / 1176

Location

United States

Related Subject Headings

  • Ventilators, Mechanical
  • Treatment Outcome
  • Surgery
  • Protein-Losing Enteropathies
  • Postoperative Complications
  • Natriuretic Peptide, Brain
  • Male
  • Humans
  • Heart Transplantation
  • Graft Rejection