{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,27]],"date-time":"2026-03-27T19:24:46Z","timestamp":1774639486276,"version":"3.50.1"},"reference-count":25,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2025,1,17]],"date-time":"2025-01-17T00:00:00Z","timestamp":1737072000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,2,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>OBJECTIVES<\/jats:title>\n                  <jats:p>This study investigated the longitudinal changes in ventricular function and atrioventricular valve function to clarify the timing and the mechanisms of failing Fontan.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>METHODS<\/jats:title>\n                  <jats:p>Patients who underwent total cavopulmonary connection between 1994 and 2023 were reviewed, and longitudinal echocardiographic examinations of ventricular function and atrioventricular valve regurgitation were analysed.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>RESULTS<\/jats:title>\n                  <jats:p>A total of 650 patients were included. The most frequent primary diagnosis was hypoplastic left heart syndrome in 175 patients. Dominant right ventricle was observed in 337 patients (51.8%). The median age at total cavopulmonary connection was 2.3 (1.8\u20133.3) years. Failing Fontan was observed in 78 patients (12%) during the median follow-up of 6.2\u2009years. Among them, dominant right ventricle was observed in 51 patients (65.4%). Patients with protein-losing enteropathy or plastic bronchitis (n\u2009=\u200937) developed Fontan failure early (median 2.6\u2009years post-total cavopulmonary connection). Still, patients maintained ventricular function (93.1% normal at 5\u2009years) and atrioventricular valve competence (no case of moderate\/severe regurgitation at 5\u2009years) over time. Patients who developed failing Fontan associated with progression of heart failure (n\u2009=\u200941) had later onset (median 8.3\u2009years post-total cavopulmonary connection) but indicated progressive ventricular dysfunction (68.3% normal at 5\u2009years, 53.8% normal at 10\u2009years) and atrioventricular valve regurgitation (12.3% moderate\/severe at 5\u2009years, 15.3% moderate\/severe at 10\u2009years).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>CONCLUSIONS<\/jats:title>\n                  <jats:p>Patients with failing Fontan indicated different serial ventricular and atrioventricular valve function profiles. Ventricular function was preserved in failing Fontan patients with protein-losing enteropathy or plastic bronchitis, whereas progressive ventricular dysfunction was observed in failing Fontan patients with heart failure.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ejcts\/ezaf005","type":"journal-article","created":{"date-parts":[[2025,1,17]],"date-time":"2025-01-17T15:12:59Z","timestamp":1737126779000},"source":"Crossref","is-referenced-by-count":2,"title":["Serial changes of systemic ventricular function and atrioventricular valve function in patients with failing Fontan"],"prefix":"10.1093","volume":"67","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7163-2724","authenticated-orcid":false,"given":"Muneaki","family":"Matsubara","sequence":"first","affiliation":[{"name":"Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universit\u00e4t M\u00fcnchen , Munich,","place":["Germany"]},{"name":"Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universit\u00e4t M\u00fcnchen , Munich,","place":["Germany"]},{"name":"Europ\u00e4isches Kinderherzzentrum M\u00fcnchen , Munich,","place":["Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vincent","family":"Dahmen","sequence":"additional","affiliation":[{"name":"Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universit\u00e4t M\u00fcnchen , Munich,","place":["Germany"]},{"name":"Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universit\u00e4t M\u00fcnchen , Munich,","place":["Germany"]},{"name":"Europ\u00e4isches Kinderherzzentrum M\u00fcnchen , Munich,","place":["Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paula","family":"Gaebert","sequence":"additional","affiliation":[{"name":"Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universit\u00e4t M\u00fcnchen , Munich,","place":["Germany"]},{"name":"Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universit\u00e4t M\u00fcnchen , Munich,","place":["Germany"]},{"name":"Europ\u00e4isches Kinderherzzentrum M\u00fcnchen , Munich,","place":["Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jonas","family":"Palm","sequence":"additional","affiliation":[{"name":"Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, Technische Universit\u00e4t M\u00fcnchen , Munich,","place":["Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carolin","family":"Niedermaier","sequence":"additional","affiliation":[{"name":"Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universit\u00e4t M\u00fcnchen , Munich,","place":["Germany"]},{"name":"Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universit\u00e4t M\u00fcnchen , Munich,","place":["Germany"]},{"name":"Europ\u00e4isches Kinderherzzentrum M\u00fcnchen , Munich,","place":["Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takuya","family":"Osawa","sequence":"additional","affiliation":[{"name":"Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universit\u00e4t M\u00fcnchen , Munich,","place":["Germany"]},{"name":"Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universit\u00e4t M\u00fcnchen , Munich,","place":["Germany"]},{"name":"Europ\u00e4isches Kinderherzzentrum M\u00fcnchen , Munich,","place":["Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6842-2988","authenticated-orcid":false,"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,","place":["Germany"]},{"name":"Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universit\u00e4t M\u00fcnchen , Munich,","place":["Germany"]},{"name":"Europ\u00e4isches Kinderherzzentrum M\u00fcnchen , Munich,","place":["Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2606-0557","authenticated-orcid":false,"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,","place":["Germany"]},{"name":"Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universit\u00e4t M\u00fcnchen , Munich,","place":["Germany"]},{"name":"Europ\u00e4isches Kinderherzzentrum M\u00fcnchen , Munich,","place":["Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christoph","family":"R\u00f6hlig","sequence":"additional","affiliation":[{"name":"Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, Technische Universit\u00e4t M\u00fcnchen , Munich,","place":["Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nicole","family":"Piber","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Surgery, German Heart Center Munich, Technische Universit\u00e4t M\u00fcnchen , Munich,","place":["Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"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, Technische Universit\u00e4t M\u00fcnchen , 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