{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,14]],"date-time":"2026-09-14T11:34:56Z","timestamp":1789385696966,"version":"build-2803163510"},"reference-count":30,"publisher":"Cambridge University Press (CUP)","issue":"4","license":[{"start":{"date-parts":[[2021,7,23]],"date-time":"2021-07-23T00:00:00Z","timestamp":1626998400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["cambridge.org"],"crossmark-restriction":true},"short-container-title":["Cardiol Young"],"published-print":{"date-parts":[[2022,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>The Patient Registry for Adolescents and Adults with Stable Fontan Circulation aims to describe a contemporary cohort of Fontan patients who could be eligible for a clinical trial investigating macitentan, an endothelin receptor antagonist. This international, non-interventional, multicentre, cross-sectional, observational registry enrolled patients with \u201cstable\u201d Fontan circulation \u226510 years following extra-cardiac conduit or lateral tunnel procedure. Main exclusion criteria were NYHA functional class IV, reoperation of Fontan circulation, or signs of disease worsening. Patient characteristics at enrolment are described; available data were collected during a single registration visit. Of the 266 screened patients, 254 were included in this analysis. At enrolment, median (interquartile range) age was 24 (20;30) years, 37%\/63% of patients were from the USA\/Europe, 54% were male, 54%\/47% had undergone extra-cardiac conduit\/lateral tunnel procedures, and 95% were in NYHA functional class I or II. History of arrhythmia was more common in older patients and patients with lateral tunnel; overall prevalence was 19%. Most laboratory values were within the normal range but mean creatinine clearance was abnormally low (87.7 ml\/min). Angiotensin-converting enzyme inhibitors were used by 48% of patients and their use was associated with creatinine clearance &lt;90 ml\/min (p = 0.007), as was Fontan completion at an older age (p = 0.007). 53.4% of patients had clinical characteristics that could potentially meet an endothelin receptor antagonist trial\u2019s eligibility criteria. The PREpArE-Fontan registry describes a cohort of patients who could potentially participate in an endothelin receptor antagonist trial and identified early subtle signs of Fontan failure, even in \u201cstable\u201d patients.<\/jats:p>","DOI":"10.1017\/s1047951121002791","type":"journal-article","created":{"date-parts":[[2021,7,23]],"date-time":"2021-07-23T03:33:45Z","timestamp":1627011225000},"page":"597-605","update-policy":"https:\/\/doi.org\/10.1017\/policypage","source":"Crossref","is-referenced-by-count":8,"title":["Adolescents and adults with Fontan circulation: insights from the PREpArE-Fontan registry"],"prefix":"10.1017","volume":"32","author":[{"given":"Lars","family":"S\u00f8ndergaard","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jamil","family":"Aboulhosn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yves","family":"d\u2019Udekem","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C\u00e9line","family":"Faure","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wayne J","family":"Franklin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alfred","family":"Hager","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuli Y","family":"Kim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erwan","family":"Muros-Le Rouzic","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Rosenberg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Markus","family":"Schwerzmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8906-7647","authenticated-orcid":false,"given":"Paul","family":"Clift","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"56","published-online":{"date-parts":[[2021,7,23]]},"reference":[{"key":"S1047951121002791_ref10","doi-asserted-by":"publisher","DOI":"10.1001\/jama.2013.281053"},{"key":"S1047951121002791_ref8","unstructured":"8. ClinicalTrials.gov. Clinical study assessing the efficacy and safety of macitentan in Fontan-palliated subjects (RUBATO). Available from: https:\/\/clinicaltrials.gov\/ct2\/show\/NCT03153137."},{"key":"S1047951121002791_ref25","doi-asserted-by":"publisher","DOI":"10.1161\/CIR.0000000000000696"},{"key":"S1047951121002791_ref4","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2015.07.065"},{"key":"S1047951121002791_ref22","doi-asserted-by":"publisher","DOI":"10.1053\/j.pcsu.2015.11.006"},{"key":"S1047951121002791_ref16","doi-asserted-by":"publisher","DOI":"10.1016\/j.hlc.2015.10.005"},{"key":"S1047951121002791_ref28","unstructured":"28. US Department of Health and Human Services. 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Published by Cambridge University Press","name":"copyright","label":"Copyright","group":{"name":"copyright_and_licensing","label":"Copyright and Licensing"}},{"value":"This is an Open Access article, distributed under the terms of the Creative Commons Attribution licence (http:\/\/creativecommons.org\/licenses\/by\/4.0\/), which permits unrestricted re-use, distribution, and reproduction in any medium, provided the original work is properly cited.","name":"license","label":"License","group":{"name":"copyright_and_licensing","label":"Copyright and Licensing"}},{"value":"This content has been made available to all.","name":"free","label":"Free to read"}]}}