{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,20]],"date-time":"2026-08-20T11:41:35Z","timestamp":1787226095723,"version":"build-2736575974"},"reference-count":22,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2018,11,4]],"date-time":"2018-11-04T00:00:00Z","timestamp":1541289600000},"content-version":"vor","delay-in-days":3,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100005366","name":"Universitetet i Oslo","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005366","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Molec Gen &amp; Gen Med"],"published-print":{"date-parts":[[2018,11]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>To explore survival, causes of death, and the prevalence of cardiovascular events in a Norwegian Marfan syndrome (MFS) cohort. MFS is a heritable connective tissue disorder associated with reduced life expectancy\u2013primarily due to aortic pathology.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A follow\u2010up study of 84 MFS adults, initially investigated in 2003\u20132004. In 2014\u20132015, 16 were deceased, 47 of 68 survivors consented to new clinical investigations. Analyses of events were performed for 47 survivors and 16 deceased at follow\u2010up. Standardized mortality ratios (SMR), using the mortality rate of the Norwegian population as reference, were calculated for all 84 and calculated for men and women separately. Causes of death and information on cardiovascular events were retrieved from death certificates and medical records.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Standardized mortality ratios (95% confidence interval): for the whole cohort: 5.24 (3.00\u20138.51); for men: 8.20 (3.54\u201316.16); for women: 3.85 (1.66\u20137.58). Cardiovascular causes were found in 11 of 16 deceased, eight of these related to aortic pathology. Cancer was the cause of death in three patients. At follow\u2010up, 51% had new cardiovascular events; 59% had undergone aortic surgery. Men experienced aortic events at younger age than women. 32% of the survivors were not followed\u2010up as recommended.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Life expectancy is reduced in this MFS cohort compared to the Norwegian population. Cardiovascular complications develop throughout life, particularly aortic pathology, the major cause of death in MFS. Death and aortic pathology seem to occur earlier in men. There is a need to improve follow\u2010up according to guidelines.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/mgg3.489","type":"journal-article","created":{"date-parts":[[2018,11,4]],"date-time":"2018-11-04T23:35:37Z","timestamp":1541374537000},"page":"1114-1123","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":54,"title":["Survival, causes of death, and cardiovascular events in patients with Marfan syndrome"],"prefix":"10.1002","volume":"6","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2792-8864","authenticated-orcid":false,"given":"Thy Thy","family":"Vanem","sequence":"first","affiliation":[{"name":"Institute of Clinical Medicine, Faculty of Medicine University of Oslo Oslo Norway"},{"name":"Department of Cardiothoracic Surgery Oslo University Hospital Oslo Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Odd Ragnar","family":"Geiran","sequence":"additional","affiliation":[{"name":"Institute of Clinical Medicine, Faculty of Medicine University of Oslo Oslo Norway"},{"name":"Department of Cardiothoracic Surgery Oslo University Hospital Oslo Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kirsten","family":"Krohg\u2010S\u00f8rensen","sequence":"additional","affiliation":[{"name":"Institute of Clinical Medicine, Faculty of Medicine University of Oslo Oslo Norway"},{"name":"Department of Cardiothoracic Surgery Oslo University Hospital Oslo Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cecilie","family":"R\u00f8e","sequence":"additional","affiliation":[{"name":"Institute of Clinical Medicine, Faculty of Medicine University of Oslo Oslo Norway"},{"name":"Department of Physical Medicine and Rehabilitation Oslo University Hospital Oslo Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benedicte","family":"Paus","sequence":"additional","affiliation":[{"name":"Institute of Clinical Medicine, Faculty of Medicine University of Oslo Oslo Norway"},{"name":"Department of Medical Genetics Oslo University Hospital Oslo Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Svend","family":"Rand\u2010Hendriksen","sequence":"additional","affiliation":[{"name":"TRS, National Resource Centre for Rare Disorders Sunnaas Rehabilitation Hospital Nesoddtangen Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,11,4]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320290316"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19960424)62:4<417::AID-AJMG15>3.0.CO;2-R"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehq258"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehu281"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1086\/520125"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.1997.tb15176.x"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.1998.tb03714.x"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00392-016-1028-3"},{"key":"e_1_2_8_10_1","doi-asserted-by":"crossref","unstructured":"Guidelines for the management of grown\u2010up congenital heart disease (new version 2010)(2011).Giornale Italiano di Cardiologia (2006) 12(7\u20138) 505\u2013550.https:\/\/doi.org\/10.1714\/915.10078","DOI":"10.1714\/915.10078"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-4471-5442-6_25"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.111.054676"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2009.072785"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197204132861502"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1093\/ejcts\/ezy065"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197904053001406"},{"key":"e_1_2_8_17_1","volume-title":"Marfan syndrome \u2010 a diagnostic challenge: Aspects of a Norwegian cohort study","author":"Rand\u2010Hendriksen S.","year":"2010"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1038\/ejhg.2009.30"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.31759"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9149(00)80066-1"},{"key":"e_1_2_8_21_1","unstructured":"The Norwegian Tax Administration(2018).This is the National Registry. 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