{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,19]],"date-time":"2026-03-19T01:05:32Z","timestamp":1773882332151,"version":"3.50.1"},"reference-count":38,"publisher":"BMJ","issue":"4","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["Heart"],"accepted":{"date-parts":[[1999,4,13]]},"published-print":{"date-parts":[[1999,10,1]]},"abstract":"<jats:sec>\n                  <jats:title>OBJECTIVE<\/jats:title>\n                  <jats:p>To evaluate survival and complication free survival in patients with Marfan\u2019s syndrome and to assess the possible influence of recently revised guidelines for prophylactic aortic root replacement in these patients.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec sec-type=\"methods\">\n                  <jats:title>METHODS<\/jats:title>\n                  <jats:p>130 patients who had been attending one institution over 14 years were evaluated. Kaplan\u2013Meier analysis was performed in 125 patients who did not present with aortic root dissection as the first sign of Marfan\u2019s syndrome, with the end points: death, aortic root dissection, and prophylactic aortic root replacement after diagnosis. In the patients developing aortic root dissection, current guidelines for prophylactic aortic root replacement were retrospectively applied to investigate the number of dissections that could theoretically have been prevented. The guidelines were: (1) aortic root diameter \u2a7e\u200955 mm, (2) positive family history of aortic dissections and aortic root diameter \u2a7e\u200950 mm, and (3) aortic root growth \u2a7e\u20092 mm\/year. Outcomes following emergency surgery (15 patients) and prophylactic surgery of the aortic root (30 patients) were compared.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>RESULTS<\/jats:title>\n                  <jats:p>Five and 10 year survival after diagnosis was 95% and 88%, and the five and 10 year complication free survival was 78% and 66%, respectively. Thirteen patients developed dissection, 30 underwent prophylactic repair, and 82 had an uncomplicated course. Eleven dissections could theoretically have been prevented by application of the current guidelines. Five year survival following emergency and prophylactic repair of the aortic root was 51%, and 97%, respectively.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>CONCLUSIONS<\/jats:title>\n                  <jats:p>Survival in the Marfan\u2019s syndrome in the past 14 years seems satisfactory; with application of current guidelines, it has probably even improved. However, because of the high fatality rate in Marfan patients developing aortic root dissection, more extensive screening for Marfan\u2019s syndrome and a search for additional risk factors are desirable.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1136\/hrt.82.4.499","type":"journal-article","created":{"date-parts":[[2014,10,22]],"date-time":"2014-10-22T15:31:45Z","timestamp":1413991905000},"page":"499-504","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":127,"title":["Survival and complication free survival in Marfan\u2019s syndrome: implications of current guidelines"],"prefix":"10.1136","volume":"82","author":[{"given":"M","family":"Groenink","sequence":"first","affiliation":[{"name":"aDepartment of Cardiology, Academic Medical Centre, Meibergdreef 9, 1105 AZ Amsterdam, Netherlands, \n\nbDepartment of Epidemiology and Biostatistics, Academic Medical Centre, \n\ncDepartment of Paediatric Cardiology, Academic Medical Centre, \n\ndDepartment of Paediatrics and Institute for Human Genetics, Academic Medical Centre, \n\neDepartment of Cardiology, Leiden University Medical Centre, Leiden, Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"T A J","family":"Lohuis","sequence":"additional","affiliation":[{"name":"aDepartment of Cardiology, Academic Medical Centre, Meibergdreef 9, 1105 AZ Amsterdam, Netherlands, \n\nbDepartment of Epidemiology and Biostatistics, Academic Medical Centre, \n\ncDepartment of Paediatric Cardiology, Academic Medical Centre, \n\ndDepartment of Paediatrics and Institute for Human Genetics, Academic Medical Centre, \n\neDepartment of Cardiology, Leiden University Medical Centre, Leiden, Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J G P","family":"Tijssen","sequence":"additional","affiliation":[{"name":"aDepartment of Cardiology, Academic Medical Centre, Meibergdreef 9, 1105 AZ Amsterdam, Netherlands, \n\nbDepartment of Epidemiology and Biostatistics, Academic Medical Centre, \n\ncDepartment of Paediatric Cardiology, Academic Medical Centre, \n\ndDepartment of Paediatrics and Institute for Human Genetics, Academic Medical Centre, \n\neDepartment of Cardiology, Leiden University Medical Centre, Leiden, Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M S J","family":"Naeff","sequence":"additional","affiliation":[{"name":"aDepartment of Cardiology, Academic Medical Centre, Meibergdreef 9, 1105 AZ Amsterdam, Netherlands, \n\nbDepartment of Epidemiology and Biostatistics, Academic Medical Centre, \n\ncDepartment of Paediatric Cardiology, Academic Medical Centre, \n\ndDepartment of Paediatrics and Institute for Human Genetics, Academic Medical Centre, \n\neDepartment of Cardiology, Leiden University Medical Centre, Leiden, Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R C M","family":"Hennekam","sequence":"additional","affiliation":[{"name":"aDepartment of Cardiology, Academic Medical Centre, Meibergdreef 9, 1105 AZ Amsterdam, Netherlands, \n\nbDepartment of Epidemiology and Biostatistics, Academic Medical Centre, \n\ncDepartment of Paediatric Cardiology, Academic Medical Centre, \n\ndDepartment of Paediatrics and Institute for Human Genetics, Academic Medical Centre, \n\neDepartment of Cardiology, Leiden University Medical Centre, Leiden, Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E E","family":"van der Wall","sequence":"additional","affiliation":[{"name":"aDepartment of Cardiology, Academic Medical Centre, Meibergdreef 9, 1105 AZ Amsterdam, Netherlands, \n\nbDepartment of Epidemiology and Biostatistics, Academic Medical Centre, \n\ncDepartment of Paediatric Cardiology, Academic Medical Centre, \n\ndDepartment of Paediatrics and Institute for Human Genetics, Academic Medical Centre, \n\neDepartment of Cardiology, Leiden University Medical Centre, Leiden, Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"B J M","family":"Mulder","sequence":"additional","affiliation":[{"name":"aDepartment of Cardiology, Academic Medical Centre, Meibergdreef 9, 1105 AZ Amsterdam, Netherlands, \n\nbDepartment of Epidemiology and Biostatistics, Academic Medical Centre, \n\ncDepartment of Paediatric Cardiology, Academic Medical Centre, \n\ndDepartment of Paediatrics and Institute for Human Genetics, Academic Medical Centre, \n\neDepartment of Cardiology, Leiden University Medical Centre, Leiden, Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[1999,10,1]]},"reference":[{"key":"2025101713152413000_82.4.499.1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197904053001406"},{"key":"2025101713152413000_82.4.499.2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.11.3.321"},{"key":"2025101713152413000_82.4.499.3","doi-asserted-by":"crossref","first-page":"804","DOI":"10.1056\/NEJM197204132861502","article-title":"Life expectancy and causes of death in the Marfan syndrome.","volume":"286","author":"Murdoch","year":"1972","journal-title":"N Engl J 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