{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T15:13:51Z","timestamp":1790522031417,"version":"4.1.0"},"reference-count":25,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Circ: Cardiovascular Imaging"],"published-print":{"date-parts":[[2019,3]]},"abstract":"<jats:sec>\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>\n                      Both bicuspid aortic valve (BAV) and Marfan syndrome have been associated with aortic dissection risk, but it is unknown whether the presence of BAV is associated with an increased aortic risk in patients with an\n                      <jats:italic>FBN1<\/jats:italic>\n                      gene mutation. We evaluated aortic diameters, aortic valve function, and aortic shape in Marfan syndrome patients with and without BAV and reported aortic events during follow-up.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>\n                      All patients with an\n                      <jats:italic>FBN1<\/jats:italic>\n                      gene mutation evaluated in our clinic were included. Aortic root diameters were measured, and the aortic valve was studied using echocardiography at each visit.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>\n                      Of the 1437 patients with an\n                      <jats:italic>FBN1<\/jats:italic>\n                      gene mutation, 26 patients (1.8%) had a BAV. Both aortic root maximal diameter and normalized\n                      <jats:italic>Z<\/jats:italic>\n                      score were larger at all ages, in patients with BAV when compared with patients with tricuspid aortic valve. Prophylactic aortic root surgery tended to be performed in younger patients when BAV was present, although aortic diameter threshold was similar in the 2 populations. No aortic dissection was observed in Marfan syndrome patients with BAV.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions:<\/jats:title>\n                    <jats:p>\n                      In patients with a\n                      <jats:italic>FBN1<\/jats:italic>\n                      mutation, BAV is associated with larger aortic root diameter, with no difference in evolution of\n                      <jats:italic>Z<\/jats:italic>\n                      score with age. We found a trend towards prophylactic aortic root surgery at younger ages but similar aortic diameter thresholds without occurrence of aortic dissection. We did not find any evidence for lowering aortic diameter thresholds used to propose preventive aortic root surgery in the presence of BAV in patients with\n                      <jats:italic>FBN1<\/jats:italic>\n                      mutations.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1161\/circimaging.118.008129","type":"journal-article","created":{"date-parts":[[2019,3,7]],"date-time":"2019-03-07T05:00:40Z","timestamp":1551934840000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":22,"title":["Clinical Significance of Aortic Root Modification Associated With Bicuspid Aortic Valve in Marfan Syndrome"],"prefix":"10.1161","volume":"12","author":[{"given":"Olivier","family":"Milleron","sequence":"first","affiliation":[{"name":"Centre National de R\u00e9f\u00e9rence pour le syndrome de Marfan et apparent\u00e9s, Paris, France (O.M., F.A., C. Bouleti, G.D., M.L., M.T., C. Boileau, G.J.)."},{"name":"AP-HP, H\u00f4pital Bichat, Service de Cardiologie, Paris, France (O.M., C. Bouleti, G.J.)."},{"name":"DHU Fire, Paris-Diderot University, France (O.M., C. Bouleti, C. Boileau, G.J.)."},{"name":"INSERM LVTS U1148 75018 Paris (O.M., C. Bouleti, C. Boileau, G.J.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacques","family":"Ropers","sequence":"additional","affiliation":[{"name":"AP-HP, H\u00f4pital Piti\u00e9- Salp\u00eatri\u00e8re, Unit\u00e9 de Recherche Clinique, Paris, France (J.R.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florence","family":"Arnoult","sequence":"additional","affiliation":[{"name":"Centre National de R\u00e9f\u00e9rence pour le syndrome de Marfan et apparent\u00e9s, Paris, France (O.M., F.A., C. Bouleti, G.D., M.L., M.T., C. Boileau, G.J.)."},{"name":"AP-HP, H\u00f4pital Bichat, Service d\u2019explorations fonctionnelles, Paris, France (F.A.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claire","family":"Bouleti","sequence":"additional","affiliation":[{"name":"Centre National de R\u00e9f\u00e9rence pour le syndrome de Marfan et apparent\u00e9s, Paris, France (O.M., F.A., C. Bouleti, G.D., M.L., M.T., C. Boileau, G.J.)."},{"name":"AP-HP, H\u00f4pital Bichat, Service de Cardiologie, Paris, France (O.M., C. Bouleti, G.J.)."},{"name":"DHU Fire, Paris-Diderot University, France (O.M., C. Bouleti, C. Boileau, G.J.)."},{"name":"INSERM LVTS U1148 75018 Paris (O.M., C. Bouleti, C. Boileau, G.J.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriel","family":"Delorme","sequence":"additional","affiliation":[{"name":"Centre National de R\u00e9f\u00e9rence pour le syndrome de Marfan et apparent\u00e9s, Paris, France (O.M., F.A., C. Bouleti, G.D., M.L., M.T., C. Boileau, G.J.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maud","family":"Langeois","sequence":"additional","affiliation":[{"name":"Centre National de R\u00e9f\u00e9rence pour le syndrome de Marfan et apparent\u00e9s, Paris, France (O.M., F.A., C. Bouleti, G.D., M.L., M.T., C. Boileau, G.J.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Tchitchinadze","sequence":"additional","affiliation":[{"name":"Centre National de R\u00e9f\u00e9rence pour le syndrome de Marfan et apparent\u00e9s, Paris, France (O.M., F.A., C. Bouleti, G.D., M.L., M.T., C. Boileau, G.J.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Celine","family":"Guien","sequence":"additional","affiliation":[{"name":"Aix Marseille University, INSERM, GMGF, Marseille, France (C.G. C. Beroud)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christophe","family":"Beroud","sequence":"additional","affiliation":[{"name":"Aix Marseille University, INSERM, GMGF, Marseille, France (C.G. C. Beroud)."},{"name":"AP-HM, D\u00e9partement de G\u00e9n\u00e9tique M\u00e9dicale, H\u00f4pital Timone Enfants, Marseille, France (C. Beroud)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine","family":"Boileau","sequence":"additional","affiliation":[{"name":"Centre National de R\u00e9f\u00e9rence pour le syndrome de Marfan et apparent\u00e9s, Paris, France (O.M., F.A., C. Bouleti, G.D., M.L., M.T., C. Boileau, G.J.)."},{"name":"DHU Fire, Paris-Diderot University, France (O.M., C. Bouleti, C. Boileau, G.J.)."},{"name":"INSERM LVTS U1148 75018 Paris (O.M., C. Bouleti, C. Boileau, G.J.)."},{"name":"AP-HP, H\u00f4pital Bichat, Service de g\u00e9n\u00e9tique, Paris, France (C. Boileau)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guillaume","family":"Jondeau","sequence":"additional","affiliation":[{"name":"Centre National de R\u00e9f\u00e9rence pour le syndrome de Marfan et apparent\u00e9s, Paris, France (O.M., F.A., C. Bouleti, G.D., M.L., M.T., C. Boileau, G.J.)."},{"name":"AP-HP, H\u00f4pital Bichat, Service de Cardiologie, Paris, France (O.M., C. Bouleti, G.J.)."},{"name":"DHU Fire, Paris-Diderot University, France (O.M., C. Bouleti, C. Boileau, G.J.)."},{"name":"INSERM LVTS U1148 75018 Paris (O.M., C. Bouleti, C. 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