{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T11:22:01Z","timestamp":1790767321991,"version":"4.1.0"},"reference-count":30,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"15","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Circulation"],"published-print":{"date-parts":[[2017,4,11]]},"abstract":"<jats:sec>\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>Eisenmenger syndrome is associated with substantial morbidity and mortality. There is no consensus, however, on mortality risk stratification. We aimed to investigate survival and predictors of death in a large, contemporary cohort of Eisenmenger syndrome patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>In a multicenter approach, we identified adults with Eisenmenger syndrome under follow-up between 2000 and 2015. We examined survival and its association with clinical, electrocardiographic, echocardiographic, and laboratory parameters.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>\n                      We studied 1098 patients (median age, 34.4 years; range, 16.1\u201384.4 years; 65.1% female; 31.9% with Down syndrome). The majority had a posttricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pretricuspid lesion (n=140, 12.7%). Over a median follow-up of 3.1 years (interquartile range, 1.4\u20135.9), allowing for 4361.6 patient-years observation, 278 patients died and 6 underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis, only age (hazard ratio [HR], 1.41\/10 years; 95% confidence interval [CI], 1.24\u20131.59;\n                      <jats:italic>P<\/jats:italic>\n                      &lt;0.001), pretricuspid shunt (HR, 1.56; 95% CI, 1.02\u20132.39;\n                      <jats:italic>P<\/jats:italic>\n                      =0.041), oxygen saturation at rest (HR, 0.53\/10%; 95% CI, 0.43\u20130.65;\n                      <jats:italic>P<\/jats:italic>\n                      &lt;0.001), presence of sinus rhythm (HR, 0.53; 95% CI, 0.32\u20130.88;\n                      <jats:italic>P<\/jats:italic>\n                      =0.013), and presence of pericardial effusion (HR, 2.41; 95% CI, 1.59\u20133.66;\n                      <jats:italic>P<\/jats:italic>\n                      &lt;0.001) remained significant predictors of death.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions:<\/jats:title>\n                    <jats:p>There is significant premature mortality among contemporary adults with Eisenmenger syndrome. We report, herewith, a multivariable mortality risk stratification model based on 5 simple, noninvasive predictors of death in this population.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1161\/circulationaha.116.023033","type":"journal-article","created":{"date-parts":[[2016,12,15]],"date-time":"2016-12-15T22:15:43Z","timestamp":1481840143000},"page":"1432-1440","update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":152,"title":["Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome"],"prefix":"10.1161","volume":"135","author":[{"given":"Aleksander","family":"Kempny","sequence":"first","affiliation":[{"name":"From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children\u2019s Hospital and Brigham and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristel S\u00f8rensen","family":"Hjortsh\u00f8j","sequence":"additional","affiliation":[{"name":"From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children\u2019s Hospital and Brigham and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hong","family":"Gu","sequence":"additional","affiliation":[{"name":"From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children\u2019s Hospital and Brigham and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei","family":"Li","sequence":"additional","affiliation":[{"name":"From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children\u2019s Hospital and Brigham and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexander R.","family":"Opotowsky","sequence":"additional","affiliation":[{"name":"From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children\u2019s Hospital and Brigham and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael J.","family":"Landzberg","sequence":"additional","affiliation":[{"name":"From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children\u2019s Hospital and Brigham and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annette Schophuus","family":"Jensen","sequence":"additional","affiliation":[{"name":"From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children\u2019s Hospital and Brigham and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lars","family":"S\u00f8ndergaard","sequence":"additional","affiliation":[{"name":"From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children\u2019s Hospital and Brigham and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mette-Elise","family":"Estensen","sequence":"additional","affiliation":[{"name":"From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children\u2019s Hospital and Brigham and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulf","family":"Thil\u00e9n","sequence":"additional","affiliation":[{"name":"From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children\u2019s Hospital and Brigham and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Werner","family":"Budts","sequence":"additional","affiliation":[{"name":"From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children\u2019s Hospital and Brigham and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara J.","family":"Mulder","sequence":"additional","affiliation":[{"name":"From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children\u2019s Hospital and Brigham and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilja","family":"Blok","sequence":"additional","affiliation":[{"name":"From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children\u2019s Hospital and Brigham and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lidia","family":"Tomkiewicz-Paj\u0105k","sequence":"additional","affiliation":[{"name":"From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children\u2019s Hospital and Brigham and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kamil","family":"Szostek","sequence":"additional","affiliation":[{"name":"From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children\u2019s Hospital and Brigham and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michele","family":"D\u2019Alto","sequence":"additional","affiliation":[{"name":"From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children\u2019s Hospital and Brigham and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giancarlo","family":"Scognamiglio","sequence":"additional","affiliation":[{"name":"From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children\u2019s Hospital and Brigham and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katja","family":"Prok\u0161elj","sequence":"additional","affiliation":[{"name":"From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children\u2019s Hospital and Brigham and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerhard-Paul","family":"Diller","sequence":"additional","affiliation":[{"name":"From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children\u2019s Hospital and Brigham and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Konstantinos","family":"Dimopoulos","sequence":"additional","affiliation":[{"name":"From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children\u2019s Hospital and Brigham and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen J.","family":"Wort","sequence":"additional","affiliation":[{"name":"From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children\u2019s Hospital and Brigham and..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael A.","family":"Gatzoulis","sequence":"additional","affiliation":[{"name":"From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children\u2019s Hospital and Brigham and..."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_4_2_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.115.017202"},{"key":"e_1_3_4_3_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehq249"},{"key":"e_1_3_4_4_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.108.190690"},{"key":"e_1_3_4_5_2","doi-asserted-by":"publisher","DOI":"10.1136\/heartjnl-2014-305690"},{"key":"e_1_3_4_6_2","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.2.5099.755"},{"key":"e_1_3_4_7_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.105.592386"},{"key":"e_1_3_4_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0735-1097(99)00153-9"},{"key":"e_1_3_4_9_2","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.166.1.at1102"},{"key":"e_1_3_4_10_2","doi-asserted-by":"publisher","DOI":"10.1136\/heartjnl-2014-305494"},{"key":"e_1_3_4_11_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.109.883876"},{"key":"e_1_3_4_12_2","unstructured":"R developement Core Team. 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