{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T16:21:08Z","timestamp":1787502068466,"version":"build-2736575974"},"reference-count":22,"publisher":"SAGE Publications","issue":"6","license":[{"start":{"date-parts":[[2023,8,7]],"date-time":"2023-08-07T00:00:00Z","timestamp":1691366400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["World J Pediatr Congenit Heart Surg"],"published-print":{"date-parts":[[2023,11]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>We aimed to evaluate incidence, outcomes, and predictors of protein-losing enteropathy (PLE) and plastic bronchitis (PB) in a cohort of total cavopulmonary connection (TCPC).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We included 620 consecutive patients undergoing TCPC between 1994 and 2021. Prevalence and predictors for onset of PLE\/PB were evaluated. Death and heart transplantation after onset of PLE\/PB were examined.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>A total of 41 patients presented with PLE\/PB (31 with PLE, 15 with PB, and 5 developed both PLE and PB). Their median age at TCPC was 2.2 (interquartile ranges [IQRs], 1.7-3.7) years, and time period to onset for PLE was 2.6 (IQR: 1.0-6.6) years and for PB was 1.1 (IQR: 0.3-4.1) years after TCPC. Independent factors for developing PLE\/PB were dominant right ventricle (RV, hazard ratio [HR], 2.243; 95% confidence interval [CI], 1.129-4.458, P\u2009=\u2009.021) and prolonged pleural effusion after TCPC (HR, 2.101; 95% CI, 1.090-4.049, P\u2009=\u2009.027). In PLE\/PB population, freedom from death or transplantation after PLE\/PB diagnosis at 5 and 10 years were 88.7% and 76.4%, respectively. Eleven surgical interventions were performed in 10 patients, comprising atrioventricular valve repairs (n\u2009=\u20094), Fontan pathway revisions (n\u2009=\u20092), pacemaker implantation (n\u2009=\u20092), secondary fenestration (n\u2009=\u20091), diaphragm plication (n\u2009=\u20091), and ventricular assist device implantation (n\u2009=\u20091). In nine patients, a recovery from PLE with the resolution of PLE symptoms and normal protein levels was achieved. Eight patients died and the remaining continued to have challenging protein loss.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Protein-losing enteropathy and PB remain severe complications in the cohort of TCPC. Patients with dominant RV, and prolonged pleural effusions, were at risk for PLE\/PB.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/21501351231185111","type":"journal-article","created":{"date-parts":[[2023,8,8]],"date-time":"2023-08-08T02:56:25Z","timestamp":1691463385000},"page":"691-698","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":10,"title":["Protein-Losing Enteropathy and Plastic Bronchitis Following the Total Cavopulmonary Connections"],"prefix":"10.1177","volume":"14","author":[{"given":"Veronika","family":"Hammer","sequence":"first","affiliation":[{"name":"Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universit\u00e4t M\u00fcnchen, Munich, Germany"},{"name":"Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universit\u00e4t, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thibault","family":"Schaeffer","sequence":"additional","affiliation":[{"name":"Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universit\u00e4t M\u00fcnchen, Munich, Germany"},{"name":"Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universit\u00e4t, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helena","family":"Staehler","sequence":"additional","affiliation":[{"name":"Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universit\u00e4t M\u00fcnchen, Munich, Germany"},{"name":"Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universit\u00e4t, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul Philipp","family":"Heinisch","sequence":"additional","affiliation":[{"name":"Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universit\u00e4t M\u00fcnchen, Munich, Germany"},{"name":"Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universit\u00e4t, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melchior","family":"Burri","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Surgery, German Heart Center Munich, Technische Universit\u00e4t M\u00fcnchen, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole","family":"Piber","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Surgery, German Heart Center Munich, Technische Universit\u00e4t M\u00fcnchen, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia","family":"Lemmer","sequence":"additional","affiliation":[{"name":"Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich at the Technical University of Munich, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2481-2017","authenticated-orcid":false,"given":"Alfred","family":"Hager","sequence":"additional","affiliation":[{"name":"Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich at the Technical University of Munich, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Ewert","sequence":"additional","affiliation":[{"name":"Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich at the Technical University of Munich, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00fcrgen","family":"H\u00f6rer","sequence":"additional","affiliation":[{"name":"Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universit\u00e4t M\u00fcnchen, Munich, Germany"},{"name":"Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universit\u00e4t, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masamichi","family":"Ono","sequence":"additional","affiliation":[{"name":"Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universit\u00e4t M\u00fcnchen, Munich, Germany"},{"name":"Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universit\u00e4t, Munich, 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