{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,9]],"date-time":"2026-04-09T17:01:55Z","timestamp":1775754115113,"version":"3.50.1"},"reference-count":33,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2026,3,24]],"date-time":"2026-03-24T00:00:00Z","timestamp":1774310400000},"content-version":"vor","delay-in-days":82,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100005630","name":"French Society of Cardiology","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005630","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,1,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>Dural ectasia (DE) is a criterion for Marfan syndrome (MFS) diagnosis. However, there is no agreement on its diagnosis. We identified new DE criteria on CT-scan imaging using machine-learning (ML) and aimed to evaluate their performance for MFS and Loeys-Dietz detection.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods and results<\/jats:title>\n                    <jats:p>MFS patients with FBN1 pathogenic variant, who underwent a complete CT-scan were included and matched 1:1 with controls. The conventional criteria for DE were assessed, as well as new criteria identified by ML (generalized linear model and random forest), regarding MFS diagnostic performance. The new DE criteria were then evaluated for identification of Loeys-Dietz patients with TGF\u00dfR1\/2 and SMAD3 pathogenic variants. A user-friendly online interface was finally developed to predict the probability of MFS or Loeys-Dietz diagnosis.<\/jats:p>\n                    <jats:p>Between November 2010 and January 2017, 93 MFS patients and their matched controls were included. Mean age was 39 \u00b1 13 years with 45% of women. Conventional definitions of DE showed poor diagnostic performance for MFS: AUC 0.68 (0.61\u20130.74).<\/jats:p>\n                    <jats:p>The new DE criteria, based on simple CT-scan measurements (antero-posterior diameter of the spinal canal and scalloping) on vertebrae L1, L2 and S1, achieved AUC 0.84 (0.69\u20130.94). These criteria outperformed the Ghent-criteria for MFS diagnosis. Moreover, in 46 patients with TGF\u00dfR1\/2 and 40 with SMAD3 pathogenic variants, the new criteria also achieved good diagnostic performance (AUC 0.83 (0.73\u20130.90) and 0.80 (0.70\u20130.88) respectively).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>New CT-scan DE criteria showed good performance for detecting MFS or Loeys-Dietz syndromes. Given the importance of early diagnosis, these new simple criteria offer a promising screening tool.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ehjimp\/qyag051","type":"journal-article","created":{"date-parts":[[2026,3,19]],"date-time":"2026-03-19T12:47:08Z","timestamp":1773924428000},"source":"Crossref","is-referenced-by-count":0,"title":["New CT-based dural ectasia criteria using machine learning to diagnose Marfan and Loeys-Dietz syndromes"],"prefix":"10.1093","volume":"4","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6922-0093","authenticated-orcid":false,"given":"Claire","family":"Bouleti","sequence":"first","affiliation":[{"name":"Clinical Investigation Center (INSERM 1402), FACT and Poitiers Hospital, Cardiology Department, University of Poitiers , CHU de Poitiers, 2 Rue de la Mil\u00e9trie, Poitiers 86000 ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Raphael","family":"Thuillier","sequence":"additional","affiliation":[{"name":"Inserm Unit Isch\u00e9mie Reperfusion, M\u00e9tabolisme et Inflammation St\u00e9rile en Transplantation (IRMETIST), UMR U1313 , Poitiers F-86073 ,","place":["France"]},{"name":"Faculty of Medicine and Pharmacy, University of Poitiers , Poitiers F-86073 ,","place":["France"]},{"name":"Biochemistry Department, CHU Poitiers , Poitiers F-86021 ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoann","family":"Moeuf","sequence":"additional","affiliation":[{"name":"Cardiology Department, Saint-Joseph Hospital , Paris 75014 ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2273-1528","authenticated-orcid":false,"given":"Gaspard","family":"Suc","sequence":"additional","affiliation":[{"name":"Cardiology Department, Bichat University Hospital, AP-HP , Paris 75018 ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Laurent","family":"Gouya","sequence":"additional","affiliation":[{"name":"Reference Center for Marfan Disease, Genetic Department, Universit\u00e9 Paris Cit\u00e9, Bichat University Hospital, AP-HP , Paris 75018 ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Florence","family":"Arnoult","sequence":"additional","affiliation":[{"name":"Reference Center for Marfan Disease, Cardiology Department, U1148 LVTS, INSERM, Universit\u00e9 Paris Cit\u00e9, Bichat University Hospital, AP-HP , Paris 75018 ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Noemie","family":"Tence","sequence":"additional","affiliation":[{"name":"Cardiology Department, Private Hospital Jacques Cartier , Massy 91300 ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Florence","family":"Nicot","sequence":"additional","affiliation":[{"name":"Cardiology Department, Hospital of Versailles , Le Chesnay 78150 ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Benjamin","family":"Alos","sequence":"additional","affiliation":[{"name":"Clinical Investigation Center (INSERM 1402), FACT and Poitiers Hospital, Cardiology Department, University of Poitiers , CHU de Poitiers, 2 Rue de la Mil\u00e9trie, Poitiers 86000 ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Margaux","family":"Gouysse","sequence":"additional","affiliation":[{"name":"Biostatistics Department, Ecole Nationale de la Statistique et de L\u2019Administration Economique ENSAE ParisTech , Palaiseau 91120 ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6517-0559","authenticated-orcid":false,"given":"Olivier","family":"Milleron","sequence":"additional","affiliation":[{"name":"Reference Center for Marfan Disease, Cardiology Department, U1148 LVTS, INSERM, Universit\u00e9 Paris Cit\u00e9, Bichat University Hospital, AP-HP , Paris 75018 ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2034-5001","authenticated-orcid":false,"given":"Phalla","family":"Ou","sequence":"additional","affiliation":[{"name":"Radiology Department, Bichat University Hospital, AP-HP , Paris 75018 ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nicolas","family":"Glatt","sequence":"additional","affiliation":[{"name":"R&D Department, CEO Clinityx Company , Boulogne-Billancourt 92100 ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria","family":"Tchitchinadze","sequence":"additional","affiliation":[{"name":"Reference Center for Marfan Disease, Cardiology Department, U1148 LVTS, INSERM, Universit\u00e9 Paris Cit\u00e9, Bichat University Hospital, AP-HP , Paris 75018 ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Philippe Gabriel","family":"Steg","sequence":"additional","affiliation":[{"name":"INSERM U1148, FACT and AP-HP, H\u00f4pital Bichat, Universit\u00e9 Paris-Cit\u00e9 , Paris 75018 ,","place":["France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0101-2076","authenticated-orcid":false,"given":"Guillaume","family":"Jondeau","sequence":"additional","affiliation":[{"name":"Reference Center for Marfan Disease, Cardiology Department, U1148 LVTS, INSERM, Universit\u00e9 Paris Cit\u00e9, Bichat University Hospital, AP-HP , Paris 75018 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