{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T18:47:39Z","timestamp":1792608459846,"version":"4.5.0"},"reference-count":34,"publisher":"Oxford University Press (OUP)","issue":"26","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2017,7,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Aims<\/jats:title>\n                  <jats:p>Eisenmenger syndrome (ES) is associated with considerable morbidity and mortality. Therapeutic strategies have changed during the 2000s in conjunction with an emphasis on specialist follow-up. The aim of this study was to determine the cause-specific mortality in ES and evaluate any relevant changes between 1977 and 2015.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods and results<\/jats:title>\n                  <jats:p>This is a retrospective, descriptive multicentre study. A total of 1546 patients (mean age 38.7\u2009\u00b1\u200915.4 years; 36% male) from 13 countries were included. Cause-specific mortality was examined before and after July 2006, \u2018early\u2019 and \u2018late\u2019, respectively. Over a median follow-up of 6.1 years (interquartile range 2.1\u201321.5 years) 558 deaths were recorded; cause-specific mortality was identified in 411 (74%) cases. Leading causes of death were heart failure (34%), infection (26%), sudden cardiac death (10%), thromboembolism (8%), haemorrhage (7%), and peri-procedural (7%). Heart failure deaths increased in the \u2018late\u2019 relative to the \u2018early\u2019 era (P\u2009=\u20090.032), whereas death from thromboembolic events and death in relation to cardiac and non-cardiac procedures decreased (P\u2009=\u20090.014, P\u2009=\u20090.014, P\u2009=\u20090.004, respectively). There was an increase in longevity in the \u2018late\u2019 vs. \u2018early\u2019 era (median survival 52.3 vs. 35.2 years, P\u2009&amp;lt;\u20090.001).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>The study shows that despite changes in therapy, care, and follow-up of ES in tertiary care centres, all-cause mortality including cardiac remains high. Patients from the \u2018late\u2019 era, however, die later and from chronic rather than acute cardiac causes, primarily heart failure, whereas peri-procedural and deaths due to haemoptysis have become less common. Lifelong vigilance in tertiary centres and further research for ES are clearly needed.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/eurheartj\/ehx201","type":"journal-article","created":{"date-parts":[[2017,3,29]],"date-time":"2017-03-29T19:22:36Z","timestamp":1490815356000},"page":"2060-2067","source":"Crossref","is-referenced-by-count":88,"title":["Past and current cause-specific mortality in Eisenmenger syndrome"],"prefix":"10.1093","volume":"38","author":[{"given":"Cristel M. S\u00f8rensen","family":"Hjortsh\u00f8j","sequence":"first","affiliation":[{"name":"1Department of Cardiology, Rigshospitalet, Copenhagen, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aleksander","family":"Kempny","sequence":"additional","affiliation":[{"name":"2Biomedical Research Unit, Adult Congenital Heart Centre, National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Royal Brompton Hospital, Imperial College London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annette Schophuus","family":"Jensen","sequence":"additional","affiliation":[{"name":"1Department of Cardiology, Rigshospitalet, Copenhagen, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keld","family":"S\u00f8rensen","sequence":"additional","affiliation":[{"name":"3Department of Internal Medicine, Aalborg University Hospital, Farsoe, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edit","family":"Nagy","sequence":"additional","affiliation":[{"name":"4Department of Medicine, Karolinska Institutet, Stockholm, Sweden"},{"name":"5Department of Cardiology, Karolinska University Hospital, Stockholm, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mikael","family":"Dellborg","sequence":"additional","affiliation":[{"name":"6Department of Cardiology, Sahlgrenska Academy, University of G\u00f6teborg, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bengt","family":"Johansson","sequence":"additional","affiliation":[{"name":"7Department of Cardiology, Norrland University Hospital, Ume\u00e5, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Virginija","family":"Rudiene","sequence":"additional","affiliation":[{"name":"8Department of Cardiology, Vilnius University Hospital, Vilnius, Lithuania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gu","family":"Hong","sequence":"additional","affiliation":[{"name":"9Department of Paediatric Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexander R.","family":"Opotowsky","sequence":"additional","affiliation":[{"name":"10Boston Adult Congenital Heart (BACH), Pulmonary Hypertension Service, Boston Children\u2019s Hospital and Brigham and Women\u2019s Hospital, Harvard Medical School, Boston, MA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Werner","family":"Budts","sequence":"additional","affiliation":[{"name":"11Department of Cardiology, University Hospitals Leuven, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara J.","family":"Mulder","sequence":"additional","affiliation":[{"name":"12Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lidia","family":"Tomkiewicz-Paj\u0105k","sequence":"additional","affiliation":[{"name":"13Department of Cardiac and Vascular Diseases, Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michele","family":"D\u2019Alto","sequence":"additional","affiliation":[{"name":"14Department of Cardiology, Second University of Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katja","family":"Prok\u0161elj","sequence":"additional","affiliation":[{"name":"15Department of Cardiology, University Medical Center Ljubljana, Slovenia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerhard-Paul","family":"Diller","sequence":"additional","affiliation":[{"name":"2Biomedical Research Unit, Adult Congenital Heart Centre, National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Royal Brompton Hospital, Imperial College London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Konstantinos","family":"Dimopoulos","sequence":"additional","affiliation":[{"name":"2Biomedical Research Unit, Adult Congenital Heart Centre, National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Royal Brompton Hospital, Imperial College London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mette-Elise","family":"Estensen","sequence":"additional","affiliation":[{"name":"16Department of Cardiology, Rikshospitalet, Oslo, Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henrik","family":"Holmstr\u00f8m","sequence":"additional","affiliation":[{"name":"17Department of Paediatric Cardiology, Rikshospitalet, Oslo, Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maila","family":"Turanlahti","sequence":"additional","affiliation":[{"name":"18Pediatric Cardiology, Hospital for Children and Adolescents, Helsinki University Central Hospital, Helsinki, Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulf","family":"Thil\u00e9n","sequence":"additional","affiliation":[{"name":"19Department of Cardiology, Lund University Hospital, Lund, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael A.","family":"Gatzoulis","sequence":"additional","affiliation":[{"name":"2Biomedical Research Unit, Adult Congenital Heart Centre, National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Royal Brompton Hospital, Imperial College London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lars","family":"S\u00f8ndergaard","sequence":"additional","affiliation":[{"name":"1Department of Cardiology, Rigshospitalet, Copenhagen, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2017,4,18]]},"reference":[{"key":"2022102517271362200_ehx201-B1","first-page":"1","article-title":"Die angeborenens Defekte der Kammerscheidewand des Herzen","volume":"32","author":"Eisenmenger","year":"1897","journal-title":"Z Klin Med Suppl"},{"key":"2022102517271362200_ehx201-B2","doi-asserted-by":"crossref","first-page":"755","DOI":"10.1136\/bmj.2.5099.755","article-title":"The Eisenmenger syndrome","volume":"2","author":"Wood","year":"1958","journal-title":"Br Med J"},{"key":"2022102517271362200_ehx201-B3","doi-asserted-by":"crossref","first-page":"1845","DOI":"10.1053\/euhj.1998.1046","article-title":"Eisenmenger syndrome. Factors relating to deterioration and death","volume":"19","author":"Daliento","year":"1998","journal-title":"Eur Heart J"},{"key":"2022102517271362200_ehx201-B4","doi-asserted-by":"crossref","first-page":"658","DOI":"10.1016\/0002-9149(71)90054-3","article-title":"Fate of the patient with the Eisenmenger syndrome","volume":"28","author":"Young","year":"1971","journal-title":"Am J Cardiol"},{"key":"2022102517271362200_ehx201-B5","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1016\/0167-5273(94)90166-X","article-title":"Prognosis for patients with Eisenmenger syndrome of various aetiology","volume":"45","author":"Saha","year":"1994","journal-title":"Int J Cardiol"},{"key":"2022102517271362200_ehx201-B6","doi-asserted-by":"crossref","first-page":"677","DOI":"10.1016\/S0002-9149(99)00415-4","article-title":"Determinants of survival and length of survival in adults with Eisenmenger syndrome","volume":"84","author":"Cantor","year":"1999","journal-title":"Am J 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