title: Fontan-associated protein-losing enteropathy and heart transplant: A Pediatric Heart Transplant Study analysis.
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(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.
authors.affiliation: Department of Pediatrics, Mott Children's Hospital, University of Michigan, Ann Arbor, Michigan. Electronic address: kurts@med.umich.edu.
authors.forename: Kurt R
authors.initials: KR
authors.lastname: Schumacher
authors.affiliation: Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital, Chicago, Illinois.
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Gossett
authors.affiliation: Department of Cardiothoracic Surgery, Children's Medical Center, University of Texas, Dallas, Texas.
authors.forename: Kristine
authors.initials: K
authors.lastname: Guleserian
authors.affiliation: Department of Surgery, University of Alabama at Birmingham, Birmingham, Alabama.
authors.forename: David C
authors.initials: DC
authors.lastname: Naftel
authors.affiliation: Department of Surgery, University of Alabama at Birmingham, Birmingham, Alabama.
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Pruitt
authors.affiliation: Department of Pediatrics, Monroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, Tennessee.
authors.forename: Debra
authors.initials: D
authors.lastname: Dodd
authors.affiliation: Department of Pediatrics, Duke Children's Hospital, Durham, North Carolina.
authors.forename: Michael
authors.initials: M
authors.lastname: Carboni
authors.affiliation: Department of Pediatrics, The Children's Hospital at Montefiore, New York, New York.
authors.forename: Jacqueline
authors.initials: J
authors.lastname: Lamour
authors.affiliation: Department of Pediatrics, Phoenix Children's Hospital, Phoenix, Arizona.
authors.forename: Stephen
authors.initials: S
authors.lastname: Pophal
authors.affiliation: Department of Pediatrics, Mott Children's Hospital, University of Michigan, Ann Arbor, Michigan.
authors.forename: Mary
authors.initials: M
authors.lastname: Zamberlan
authors.affiliation: Department of Pediatrics, Mott Children's Hospital, University of Michigan, Ann Arbor, Michigan.
authors.forename: Robert J
authors.initials: RJ
authors.lastname: Gajarski
mesh_terms: D000367:Age Factors; D001663:Bilirubin / Q000097:blood; D001830:Body Surface Area; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects*; D006084:Graft Rejection; D016027:Heart Transplantation / Q000401:mortality; D006801:Humans; D008297:Male; D020097:Natriuretic Peptide, Brain / Q000097:blood; D011183:Postoperative Complications; D011504:Protein-Losing Enteropathies / Q000209:etiology*; D016896:Treatment Outcome; D012122:Ventilators, Mechanical
chemical_list: D020097:Natriuretic Peptide, Brain; D001663:Bilirubin
keywords: Fontan; heart transplantation; pediatric; protein-losing enteropathy; waiting list