{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T23:50:01Z","timestamp":1791503401440,"version":"4.3.4"},"reference-count":17,"publisher":"Georg Thieme Verlag KG","issue":"CP","license":[{"start":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T00:00:00Z","timestamp":1791417600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Deutsche\n                        Forschungsgemeinschaft","award":["413668513"],"award-info":[{"award-number":["413668513"]}]},{"name":"Deutsche\n                        Herzstiftung","award":["S\/03\/23"],"award-info":[{"award-number":["S\/03\/23"]}]},{"name":"Interdisciplinary Center of Clinical Research of the Medical\n                        Faculty Jena"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Aorta (Stamford)"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Marfan syndrome (MFS) and Loeys\u2013Dietz syndrome (LDS) are hereditary\n                        connective tissue diseases, both characterized by aortic root and vessel\n                        involvement with an increased risk of aneurysms and dissections. Despite\n                        advances in surgical techniques and improved postoperative follow-up, the\n                        impact of surgery on long-term outcomes in these patient groups is still\n                        unknown.<\/jats:p>\n                  <jats:p>We performed a systematic review and a meta-analysis to compare the\n                        postoperative long-term events. The main outcome was long-term survival.\n                        Additional outcomes were freedom from dissection and reoperation. After\n                        reconstruction of time-to-event data for the individual survival analysis,\n                        pooled Kaplan\u2013Meier curves for the endpoints were generated.<\/jats:p>\n                  <jats:p>A total of 1,794 studies were identified, of which 5 were included in the\n                        analysis, encompassing 1,005 patients (MFS: 778; LDS: 227). With MFS as the\n                        reference group, there was no significant difference in long-term survival\n                        between both groups, based on age (hazard ratio [HR]: 0.919; 95% confidence\n                        interval [CI]: 0.58\u20131.45; p=0.717) or postoperative follow-up (HR:\n                        0.997; 95% CI: 0.45\u20132.19; p=0.995). However, LDS was associated with\n                        higher risk of dissection (HR: 0.331; 95% CI: 0.21\u20130.53; p&lt;0.005)\n                        and reoperation (HR: 0.603; 95% CI: 0.40\u20130.90; p=0.014), reflecting\n                        unadjusted comparisons reconstructed from Kaplan\u2013Meier curves.<\/jats:p>\n                  <jats:p>Although long-term survival is similar between MFS and LDS patients, those\n                        with LDS face a significantly higher risk of aortic dissection and\n                        reoperation. This elevated risk may be partly explained by the intrinsic\n                        genotypic heterogeneity of the LDS population. However, our pooled analysis\n                        could not fully adjust for this heterogeneity, underscoring the need for\n                        vigilant lifelong imaging surveillance and individualized surgical\n                        thresholds in LDS.<\/jats:p>","DOI":"10.1055\/a-2965-6400","type":"journal-article","created":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T23:09:57Z","timestamp":1791500997000},"source":"Crossref","is-referenced-by-count":0,"special_numbering":"S","title":["Marfan Syndrome versus Loeys\u2013Dietz Syndrome after Aortic Surgery: A\n                    Systematic Review and Meta-analysis of Reconstructed Time-to-Event\n                    Data"],"prefix":"10.1055","volume":"14","author":[{"given":"Johannes","family":"Fischer","sequence":"additional","affiliation":[{"name":"Friedrich-Schiller-University Jena, Germany","place":["Jena"],"department":["Department of Cardiothoracic Surgery"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steve","family":"Wehner","sequence":"additional","affiliation":[{"name":"Friedrich-Schiller-University Jena, Germany","place":["Jena"],"department":["Department of Cardiothoracic Surgery"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ayodeji","family":"Arobo","sequence":"additional","affiliation":[{"name":"College of Health Sciences, Obafemi Awolowo\n                    University, Nigeria","place":["Ile-ife"],"department":["Medical School"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abdullah","family":"Almehandi","sequence":"additional","affiliation":[{"name":"University College London, United Kingdom","place":["London"],"department":["Department of Cardiovascular Science"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0001-7381-2806","authenticated-orcid":false,"given":"Felipe S.","family":"Passos","sequence":"additional","affiliation":[{"name":"MaterDei Hospital, Bahia, Brazil","place":["Salvador"],"department":["Department of Thoracic Surgery"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Eitel","sequence":"additional","affiliation":[{"name":"West German Heart and Vascular Center, University\n                    Hospital Essen, Germany","place":["Essen"],"department":["Department of Cardiology and Vascular Medicine"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hristo","family":"Kirov","sequence":"additional","affiliation":[{"name":"Friedrich-Schiller-University Jena, Germany","place":["Jena"],"department":["Department of Cardiothoracic Surgery"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xander","family":"Jacquemyn","sequence":"additional","affiliation":[{"name":"UPMC Heart and Vascular Institute, University of\n                    Pittsburgh Medical Center, Pennsylvania, United States","place":["Pittsburgh"],"department":["Department of Cardiothoracic Surgery"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ibrahim","family":"Sultan","sequence":"additional","affiliation":[{"name":"UPMC Heart and Vascular Institute, University of\n                    Pittsburgh Medical Center, Pennsylvania, United States","place":["Pittsburgh"],"department":["Department of Cardiothoracic Surgery"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Torsten","family":"Doenst","sequence":"additional","affiliation":[{"name":"Jena University Hospital, Universitatsklinikum\n                    Jena, Germany","place":["Jena"],"department":["Department of Cardiothoracic Surgery"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5697-2659","authenticated-orcid":false,"given":"Tulio","family":"Caldonazo","sequence":"additional","affiliation":[{"name":"Friedrich-Schiller-University Jena, Germany","place":["Jena"],"department":["Department of Cardiothoracic Surgery"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2026,10,8]]},"reference":[{"issue":"01","key":"ref1","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1097\/HCO.0000000000000023","article-title":"Heritable thoracic aortic disorders","volume":"29","author":"R E Pyeritz","year":"2014","journal-title":"Curr Opin Cardiol"},{"issue":"09","key":"ref2","doi-asserted-by":"crossref","first-page":"1238","DOI":"10.1136\/hrt.2005.081638","article-title":"Beyond the root: dilatation of the distal aorta in Marfan\u2019s syndrome","volume":"92","author":"P M Engelfriet","year":"2006","journal-title":"Heart"},{"issue":"25","key":"ref3","doi-asserted-by":"crossref","first-page":"2541","DOI":"10.1161\/CIRCULATIONAHA.109.887042","article-title":"Comparison of clinical presentations and outcomes between patients with TGFBR2\n                    and FBN1 mutations in Marfan syndrome and related disorders","volume":"120","author":"D Attias","year":"2009","journal-title":"Circulation"},{"issue":"05","key":"ref4","doi-asserted-by":"crossref","first-page":"1413","DOI":"10.1016\/j.athoracsur.2012.05.111","article-title":"Surgical experience with aggressive aortic pathologic process in Loeys-Dietz\n                    syndrome","volume":"94","author":"Y Iba","year":"2012","journal-title":"Ann Thorac Surg"},{"issue":"05","key":"ref5","doi-asserted-by":"crossref","first-page":"1344","DOI":"10.1016\/j.athoracsur.2009.01.073","article-title":"Aortic root replacement in 372 Marfan patients: evolution of operative repair\n                    over 30 years","volume":"87","author":"D E Cameron","year":"2009","journal-title":"Ann Thorac Surg"},{"issue":"15","key":"ref6","doi-asserted-by":"crossref","first-page":"1569","DOI":"10.1161\/CIRCULATIONAHA.113.001457","article-title":"Acute aortic dissection determines the fate of initially untreated aortic\n          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Aortic Consortium)","volume":"9","author":"G Jondeau","year":"2016","journal-title":"Circ Cardiovasc Genet"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1186\/1471-2288-12-9","article-title":"Enhanced secondary analysis of survival data: reconstructing the data from\n                    published Kaplan-Meier survival curves","volume":"12","author":"P Guyot","year":"2012","journal-title":"BMC Med Res Methodol"},{"issue":"04","key":"ref11","doi-asserted-by":"crossref","first-page":"786","DOI":"10.1177\/1536867X1801700402","article-title":"Reconstructing time-to-event data from published Kaplan-Meier curves","volume":"17","author":"Y Wei","year":"2017","journal-title":"Stata J"},{"issue":"03","key":"ref12","doi-asserted-by":"crossref","first-page":"444","DOI":"10.1093\/ejcts\/ezu002","article-title":"Outcome of aortic surgery in patients with Loeys-Dietz syndrome primarily\n                    treated as having Marfan syndrome","volume":"46","author":"F S Schoenhoff","year":"2014","journal-title":"Eur J Cardiothorac Surg"},{"issue":"12","key":"ref13","doi-asserted-by":"crossref","first-page":"8","DOI":"10.3390\/jcm8122079","article-title":"Case-matched comparison of cardiovascular outcome in Loeys-Dietz syndrome versus\n                    Marfan syndrome","volume":"8","author":"K M\u00fchlst\u00e4dt","year":"2019","journal-title":"J Clin Med"},{"issue":"01","key":"ref14","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1016\/j.jtcvs.2020.07.089","article-title":"The differences in surgical long-term outcomes between Marfan syndrome and\n                    Loeys-Dietz syndrome","volume":"164","author":"Y Seike","year":"2022","journal-title":"J Thorac Cardiovasc Surg"},{"key":"ref15","doi-asserted-by":"crossref","DOI":"10.3389\/fcvm.2022.1009947","article-title":"Long-term outcomes in heritable thoracic aortic disease","volume":"9","author":"E N Robertson","year":"2022","journal-title":"Front Cardiovasc Med"},{"issue":"05","key":"ref16","doi-asserted-by":"crossref","first-page":"1166","DOI":"10.1016\/j.jtcvs.2019.07.130","article-title":"Management of the aortic arch in patients with Loeys-Dietz syndrome","volume":"160","author":"F S Schoenhoff","year":"2020","journal-title":"J Thorac Cardiovasc Surg"},{"key":"ref17","doi-asserted-by":"crossref","DOI":"10.3389\/fcvm.2024.1429222","article-title":"Impact of early diagnosis on surgical outcomes in patients with Loeys-Dietz\n                    syndrome","volume":"11","author":"H Kim","year":"2024","journal-title":"Front Cardiovasc Med"}],"container-title":["AORTA"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/a-2965-6400.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T23:10:00Z","timestamp":1791501000000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/a-2965-6400"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,10,8]]},"references-count":17,"journal-issue":{"issue":"CP","published-online":{"date-parts":[[2026,3,26]]},"published-print":{"date-parts":[[2026]]}},"alternative-id":["a29656400"],"URL":"https:\/\/doi.org\/10.1055\/a-2965-6400","archive":["Portico","CLOCKSS"],"relation":{},"ISSN":["2325-4637"],"issn-type":[{"value":"2325-4637","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,10,8]]}}}