{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,8]],"date-time":"2026-09-08T22:09:27Z","timestamp":1788905367001,"version":"build-2803163510"},"reference-count":16,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2023,1,2]],"date-time":"2023-01-02T00:00:00Z","timestamp":1672617600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2023,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Patients after Fontan palliation represent a growing pediatric population requiring heart transplant (HTx) and often have lymphopenia (L) and\/or hypogammaglobinemia that may be exacerbated by protein\u2010losing enteropathy (PLE, P). The post\u2010HTx effects of this altered immune phenotype are not well studied.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In this study of the Pediatric Heart Transplant Society Registry, 106 Fontan patients who underwent HTx between 2005 and 2018 were analyzed. The impact of lymphopenia and PLE on graft survival, infection, rejection, and malignancy was analyzed at 1 and 5\u2009years post\u2010HTx.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The following combinations of lymphopenia and PLE were noted: +L+P, <jats:italic>n<\/jats:italic>\u00a0=\u00a037; +L\u2212P, <jats:italic>n<\/jats:italic>\u00a0=\u00a023; \u2212L+P, <jats:italic>n<\/jats:italic>\u00a0=\u00a010; and \u2212L\u2212P, <jats:italic>n<\/jats:italic>\u00a0=\u00a036. Graft survival between the groups was similar within the first year after transplant (+L+P: 86%, +L\u2212P: 86%, \u2212L+P: 87%, \u2212L\u2212P: 89%, <jats:italic>p<\/jats:italic>\u00a0=\u00a0.9). Freedom from first infection post\u2010HTx was greatest among \u2212L\u2212P patients compared to patients with either PLE, lymphopenia, or both; with a 22.1% infection incidence in the \u2212L\u2212P group and 41.4% in all others. These patients had a significantly lower infection rate in the first year after HTx (+L+P: 1.03, +L\u2212P: 1, \u2212L+P: 1.3, \u2212L\u2212P: 0.3 infections\/year, <jats:italic>p<\/jats:italic>\u2009&lt;\u2009.001) and were similar to a non\u2010single ventricle CHD control group (0.4 infections\/year). Neither freedom from rejection nor freedom from malignancy 1 and 5\u2009years post\u2010HTx, differed among the groups.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Fontan patients with altered immunophenotype, with lymphopenia and\/or PLE, are at increased risk of infection post\u2010HTx, although have similar early survival and freedom from rejection and malignancy. These data may encourage alternative immunosuppression strategies and enhanced monitoring for this growing subset of patients.<\/jats:p><\/jats:sec>","DOI":"10.1111\/petr.14456","type":"journal-article","created":{"date-parts":[[2023,1,2]],"date-time":"2023-01-02T10:53:53Z","timestamp":1672656833000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["The Fontan immunophenotype and post\u2010transplant outcomes in children: A multi\u2010institutional study"],"prefix":"10.1111","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7700-6975","authenticated-orcid":false,"given":"Benjamin S.","family":"Mantell","sequence":"first","affiliation":[{"name":"Division of Pediatric Cardiology, Morgan Stanley Children's Hospital Columbia University Irving Medical Center of NewYork\u2010Presbyterian  New York New York 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USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maryanne","family":"Chrisant","sequence":"additional","affiliation":[{"name":"The Heart Institute, Joe DiMaggio Children's Hospital  Hollywood Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John C.","family":"Dykes","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiology Lucile Packard Children's Hospital Stanford  Palo Alto California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timothy M.","family":"Hoffman","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiology North Carolina Children's Hospital  Chapel Hill North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James K.","family":"Kirklin","sequence":"additional","affiliation":[{"name":"Kirklin Institute for Research in Surgical Outcomes The University of Alabama at Birmingham  Birmingham Alabama USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Devin","family":"Koehl","sequence":"additional","affiliation":[{"name":"Kirklin Institute for Research in Surgical Outcomes The University of Alabama at Birmingham  Birmingham Alabama USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas J.","family":"L'Ecuyer","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiology University of Virginia Health System  Charlottesville Virginia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennie M.","family":"McAllister","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiology, Morgan Stanley Children's Hospital Columbia University Irving Medical Center of NewYork\u2010Presbyterian  New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adriana C.","family":"Prada\u2010Ruiz","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiology 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