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

Kurt R. Schumacher, Jeffrey Gossett, Kristine Guleserian, David C. Naftel, Elizabeth Pruitt, Debra Dodd, Michael Carboni, Jacqueline M. Lamour, Stephen Pophal, Mary Zamberlan, Robert J. Gajarski

Research output: Contribution to journalArticle

19 Citations (Scopus)

Abstract

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<sup>2</sup> vs 0.9 m<sup>2</sup>; 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.

Original languageEnglish (US)
Article number6003
Pages (from-to)1169-1176
Number of pages8
JournalJournal of Heart and Lung Transplantation
Volume34
Issue number9
DOIs
StatePublished - Sep 1 2015

Fingerprint

Protein-Losing Enteropathies
Pediatrics
Transplants
Waiting Lists
Mortality
Morbidity
Brain Natriuretic Peptide
Body Surface Area
Mechanical Ventilators
Heart Transplantation
Infection
Bilirubin

Keywords

  • Fontan
  • heart transplantation
  • pediatric
  • protein-losing enteropathy
  • waiting list

ASJC Scopus subject areas

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

Cite this

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. Journal of Heart and Lung Transplantation, 34(9), 1169-1176. [6003]. https://doi.org/10.1016/j.healun.2015.03.022

Fontan-associated protein-losing enteropathy and heart transplant : A Pediatric Heart Transplant Study analysis. / Schumacher, Kurt R.; Gossett, Jeffrey; Guleserian, Kristine; Naftel, David C.; Pruitt, Elizabeth; Dodd, Debra; Carboni, Michael; Lamour, Jacqueline M.; Pophal, Stephen; Zamberlan, Mary; Gajarski, Robert J.

In: Journal of Heart and Lung Transplantation, Vol. 34, No. 9, 6003, 01.09.2015, p. 1169-1176.

Research output: Contribution to journalArticle

Schumacher, KR, Gossett, J, Guleserian, K, Naftel, DC, Pruitt, E, Dodd, D, Carboni, M, Lamour, JM, Pophal, S, Zamberlan, M & Gajarski, RJ 2015, 'Fontan-associated protein-losing enteropathy and heart transplant: A Pediatric Heart Transplant Study analysis', Journal of Heart and Lung Transplantation, vol. 34, no. 9, 6003, pp. 1169-1176. https://doi.org/10.1016/j.healun.2015.03.022
Schumacher, Kurt R. ; Gossett, Jeffrey ; Guleserian, Kristine ; Naftel, David C. ; Pruitt, Elizabeth ; Dodd, Debra ; Carboni, Michael ; Lamour, Jacqueline M. ; Pophal, Stephen ; Zamberlan, Mary ; Gajarski, Robert J. / Fontan-associated protein-losing enteropathy and heart transplant : A Pediatric Heart Transplant Study analysis. In: Journal of Heart and Lung Transplantation. 2015 ; Vol. 34, No. 9. pp. 1169-1176.
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abstract = "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 m2 vs 0.9 m2; 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.",
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AU - Gossett, Jeffrey

AU - Guleserian, Kristine

AU - Naftel, David C.

AU - Pruitt, Elizabeth

AU - Dodd, Debra

AU - Carboni, Michael

AU - Lamour, Jacqueline M.

AU - Pophal, Stephen

AU - Zamberlan, Mary

AU - Gajarski, Robert J.

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N2 - 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 m2 vs 0.9 m2; 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.

AB - 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 m2 vs 0.9 m2; 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.

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