{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,18]],"date-time":"2026-10-18T02:29:24Z","timestamp":1792290564575,"version":"4.4.0"},"reference-count":85,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2024,9,1]],"date-time":"2024-09-01T00:00:00Z","timestamp":1725148800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2024,9,1]],"date-time":"2024-09-01T00:00:00Z","timestamp":1725148800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2024,6,8]],"date-time":"2024-06-08T00:00:00Z","timestamp":1717804800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["International Journal of Cardiology Congenital Heart Disease"],"published-print":{"date-parts":[[2024,9]]},"DOI":"10.1016\/j.ijcchd.2024.100520","type":"journal-article","created":{"date-parts":[[2024,6,10]],"date-time":"2024-06-10T02:32:59Z","timestamp":1717986779000},"page":"100520","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":6,"special_numbering":"C","title":["Update on Eisenmenger syndrome \u2013 Review of pathophysiology and recent progress in risk assessment and management"],"prefix":"10.1016","volume":"17","author":[{"given":"Ranjan","family":"Banerjee","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6107-2281","authenticated-orcid":false,"given":"Alexander R.","family":"Opotowsky","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.ijcchd.2024.100520_bib1","doi-asserted-by":"crossref","first-page":"755","DOI":"10.1136\/bmj.2.5099.755","article-title":"The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt","volume":"2","author":"Wood","year":"1958","journal-title":"Br Med J"},{"key":"10.1016\/j.ijcchd.2024.100520_bib2","doi-asserted-by":"crossref","first-page":"1638","DOI":"10.1136\/heartjnl-2020-316665","article-title":"Eisenmenger syndrome: diagnosis, prognosis and clinical management","volume":"106","author":"Arvanitaki","year":"2020","journal-title":"Heart"},{"key":"10.1016\/j.ijcchd.2024.100520_bib3","doi-asserted-by":"crossref","first-page":"198","DOI":"10.1016\/j.ijcard.2006.09.017","article-title":"Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry","volume":"120","author":"Duffels","year":"2007","journal-title":"Int J Cardiol"},{"key":"10.1016\/j.ijcchd.2024.100520_bib4","doi-asserted-by":"crossref","first-page":"2325","DOI":"10.1093\/eurheartj\/ehi396","article-title":"The spectrum of adult congenital heart disease in Europe: morbidity and mortality in a 5 year follow-up period","volume":"26","author":"Engelfriet","year":"2005","journal-title":"Eur Heart J"},{"key":"10.1016\/j.ijcchd.2024.100520_bib5","doi-asserted-by":"crossref","first-page":"1664","DOI":"10.1016\/j.cjca.2019.10.002","article-title":"Eisenmenger syndrome: a multisystem disorder\u2014do not destabilize the balanced but fragile physiology","volume":"35","author":"Chaix","year":"2019","journal-title":"Can J Cardiol"},{"key":"10.1016\/j.ijcchd.2024.100520_bib6","doi-asserted-by":"crossref","first-page":"1183","DOI":"10.1016\/j.jacc.2022.01.022","article-title":"Eisenmenger syndrome","volume":"79","author":"Arvanitaki","year":"2022","journal-title":"J Am Coll Cardiol"},{"key":"10.1016\/j.ijcchd.2024.100520_bib7","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1136\/heartjnl-2012-301685","article-title":"Management of adults with cyanotic congenital heart disease","volume":"101","author":"Oechslin","year":"2015","journal-title":"Heart"},{"key":"10.1016\/j.ijcchd.2024.100520_bib8","doi-asserted-by":"crossref","first-page":"1737","DOI":"10.1093\/eurheartj\/ehl116","article-title":"Presentation, survival prospects, and predictors of death in Eisenmenger syndrome: a combined retrospective and case\u2013control study","volume":"27","author":"Diller","year":"2006","journal-title":"Eur Heart J"},{"key":"10.1016\/j.ijcchd.2024.100520_bib9","doi-asserted-by":"crossref","first-page":"1386","DOI":"10.1016\/j.ijcard.2012.12.021","article-title":"Worsening in oxygen saturation and exercise capacity predict adverse outcome in patients with Eisenmenger syndrome","volume":"168","author":"Van De Bruaene","year":"2013","journal-title":"Int J Cardiol"},{"key":"10.1016\/j.ijcchd.2024.100520_bib10","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1016\/j.ijcard.2018.05.062","article-title":"Use of intravenous iron in cyanotic patients with congenital heart disease and\/or pulmonary hypertension","volume":"267","author":"Blanche","year":"2018","journal-title":"Int J Cardiol"},{"key":"10.1016\/j.ijcchd.2024.100520_bib11","series-title":"StatPearls. Treasure Island (FL): StatPearls publishing Copyright \u00a9 2024","article-title":"Hyperviscosity syndrome","author":"Perez Rogers","year":"2024"},{"key":"10.1016\/j.ijcchd.2024.100520_bib12","first-page":"139","article-title":"AHA\/ACC guideline for the management of adults with congenital heart disease: a report of the American college of cardiology\/American heart association task force on clinical practice guidelines","author":"Stout","year":"2018","journal-title":"Circulation"},{"key":"10.1016\/j.ijcchd.2024.100520_bib13","doi-asserted-by":"crossref","first-page":"308","DOI":"10.1001\/jamacardio.2018.0125","article-title":"Association of albuminuria with major adverse outcomes in adults with congenital heart disease: results from the Boston adult congenital heart biobank","volume":"3","author":"Rajpal","year":"2018","journal-title":"JAMA Cardiol"},{"key":"10.1016\/j.ijcchd.2024.100520_bib14","doi-asserted-by":"crossref","first-page":"403","DOI":"10.1016\/0002-9149(91)90842-9","article-title":"Incidence of renal dysfunction in adults with cyanotic congenital heart disease","volume":"68","author":"Flanagan","year":"1991","journal-title":"Am J Cardiol"},{"key":"10.1016\/j.ijcchd.2024.100520_bib15","doi-asserted-by":"crossref","first-page":"2320","DOI":"10.1161\/CIRCULATIONAHA.107.734921","article-title":"Prevalence, predictors, and prognostic value of renal dysfunction in adults with congenital heart disease","volume":"117","author":"Dimopoulos","year":"2008","journal-title":"Circulation"},{"key":"10.1016\/j.ijcchd.2024.100520_bib16","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1016\/j.ahj.2019.04.018","article-title":"Creatinine versus cystatin C to estimate glomerular filtration rate in adults with congenital heart disease: results of the Boston Adult Congenital Heart Disease Biobank","volume":"214","author":"Opotowsky","year":"2019","journal-title":"Am Heart J"},{"key":"10.1016\/j.ijcchd.2024.100520_bib17","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1136\/hrt.41.1.23","article-title":"Haemostatic defects in cyanotic congenital heart disease","volume":"41","author":"Henriksson","year":"1979","journal-title":"Heart"},{"key":"10.1016\/j.ijcchd.2024.100520_bib18","doi-asserted-by":"crossref","first-page":"2060","DOI":"10.1093\/eurheartj\/ehx201","article-title":"Past and current cause-specific mortality in Eisenmenger syndrome","volume":"38","author":"Hjortshoj","year":"2017","journal-title":"Eur Heart J"},{"key":"10.1016\/j.ijcchd.2024.100520_bib19","doi-asserted-by":"crossref","first-page":"634","DOI":"10.1016\/j.jacc.2007.04.056","article-title":"Pulmonary arterial thrombosis in eisenmenger syndrome is associated with biventricular dysfunction and decreased pulmonary flow velocity","volume":"50","author":"Broberg","year":"2007","journal-title":"J Am Coll Cardiol"},{"key":"10.1016\/j.ijcchd.2024.100520_bib20","doi-asserted-by":"crossref","first-page":"1540","DOI":"10.1136\/heartjnl-2015-307657","article-title":"Prevalence of cerebral and pulmonary thrombosis in patients with cyanotic congenital heart disease","volume":"101","author":"Jensen","year":"2015","journal-title":"Heart"},{"key":"10.1016\/j.ijcchd.2024.100520_bib21","doi-asserted-by":"crossref","first-page":"1386","DOI":"10.1093\/eurheartj\/ehr461","article-title":"Reference values for exercise limitations among adults with congenital heart disease. Relation to activities of daily life\u2014single centre experience and review of published data","volume":"33","author":"Kempny","year":"2011","journal-title":"Eur Heart J"},{"key":"10.1016\/j.ijcchd.2024.100520_bib22","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1093\/eurheartj\/ehaa554","article-title":"2020 ESC Guidelines for the management of adult congenital heart disease","volume":"42","author":"Baumgartner","year":"2021","journal-title":"Eur Heart J"},{"key":"10.1016\/j.ijcchd.2024.100520_bib23","doi-asserted-by":"crossref","first-page":"268","DOI":"10.1111\/j.1747-0803.2012.00633.x","article-title":"Does anticoagulation in eisenmenger syndrome impact long-term survival?","volume":"7","author":"Sandoval","year":"2012","journal-title":"Congenit Heart Dis"},{"key":"10.1016\/j.ijcchd.2024.100520_bib24","doi-asserted-by":"crossref","first-page":"1449","DOI":"10.1093\/eurheartj\/ehv743","article-title":"German competence network for congenital heart defects I. Current therapy and outcome of eisenmenger syndrome: data of the German national register for congenital heart defects","volume":"37","author":"Diller","year":"2016","journal-title":"Eur Heart J"},{"key":"10.1016\/j.ijcchd.2024.100520_bib25","doi-asserted-by":"crossref","first-page":"745","DOI":"10.7326\/0003-4819-128-9-199805010-00008","article-title":"The Eisenmenger syndrome in adults","volume":"128","author":"Vongpatanasin","year":"1998","journal-title":"Ann Intern Med"},{"key":"10.1016\/j.ijcchd.2024.100520_bib26","doi-asserted-by":"crossref","first-page":"974","DOI":"10.1016\/j.healun.2023.02.1494","article-title":"Characteristics, treatments and survival of pulmonary arterial hypertension associated with congenital heart disease in China: insights from a national multicenter prospective registry","volume":"42","author":"Xie","year":"2023","journal-title":"J Heart Lung Transplant"},{"key":"10.1016\/j.ijcchd.2024.100520_bib27","doi-asserted-by":"crossref","first-page":"1366","DOI":"10.1136\/heartjnl-2014-305690","article-title":"Survival prospects of treatment na\u00efve patients with Eisenmenger: a systematic review of the literature and report of own experience","volume":"100","author":"Diller","year":"2014","journal-title":"Heart"},{"key":"10.1016\/j.ijcchd.2024.100520_bib28","first-page":"100","article-title":"Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome","volume":"15","author":"Hopkins","year":"1996","journal-title":"J Heart Lung Transplant"},{"key":"10.1016\/j.ijcchd.2024.100520_bib29","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1016\/j.amjcard.2013.09.032","article-title":"Four- and seven-year outcomes of patients with congenital heart disease-associated pulmonary arterial hypertension (from the REVEAL Registry)","volume":"113","author":"Barst","year":"2014","journal-title":"Am J Cardiol"},{"key":"10.1016\/j.ijcchd.2024.100520_bib30","doi-asserted-by":"crossref","first-page":"842","DOI":"10.1164\/rccm.202009-3698OC","article-title":"Association between initial treatment strategy and long-term survival in pulmonary arterial hypertension","volume":"204","author":"Boucly","year":"2021","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.ijcchd.2024.100520_bib31","doi-asserted-by":"crossref","first-page":"1393","DOI":"10.1016\/j.jacc.2021.07.057","article-title":"Three- versus two-drug therapy for patients with newly diagnosed pulmonary arterial hypertension","volume":"78","author":"Chin","year":"2021","journal-title":"J Am Coll Cardiol"},{"key":"10.1016\/j.ijcchd.2024.100520_bib32","doi-asserted-by":"crossref","first-page":"1520","DOI":"10.1016\/j.cjca.2016.03.004","article-title":"Combination therapy for pulmonary arterial hypertension: a systematic review and meta-analysis","volume":"32","author":"Fox","year":"2016","journal-title":"Can J Cardiol"},{"key":"10.1016\/j.ijcchd.2024.100520_bib33","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1016\/S2213-2600(16)00027-8","article-title":"Combination therapy versus monotherapy for pulmonary arterial hypertension: a meta-analysis","volume":"4","author":"Lajoie","year":"2016","journal-title":"Lancet Respir Med"},{"key":"10.1016\/j.ijcchd.2024.100520_bib34","doi-asserted-by":"crossref","DOI":"10.1183\/13993003.01552-2021","article-title":"Mortality trends in pulmonary arterial hypertension in Canada: a temporal analysis of survival per ESC\/ERS guideline era","volume":"59","author":"Zelt","year":"2022","journal-title":"Eur Respir J"},{"key":"10.1016\/j.ijcchd.2024.100520_bib35","doi-asserted-by":"crossref","DOI":"10.1161\/JAHA.121.024969","article-title":"Mortality in pulmonary arterial hypertension in the modern era: early insights from the pulmonary hypertension association registry","volume":"11","author":"Chang","year":"2022","journal-title":"J Am Heart Assoc"},{"key":"10.1016\/j.ijcchd.2024.100520_bib36","doi-asserted-by":"crossref","first-page":"736","DOI":"10.1136\/heartjnl-2011-301522","article-title":"B-type natriuretic peptide concentrations in contemporary Eisenmenger syndrome patients: predictive value and response to disease targeting therapy","volume":"98","author":"Diller","year":"2012","journal-title":"Heart"},{"key":"10.1016\/j.ijcchd.2024.100520_bib37","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1136\/heartjnl-2018-313613","article-title":"Hospitalisations for heart failure predict mortality in pulmonary hypertension related to congenital heart disease","volume":"105","author":"Ntiloudi","year":"2019","journal-title":"Heart"},{"key":"10.1016\/j.ijcchd.2024.100520_bib38","article-title":"One-minute sit-to-stand test in patients with pulmonary arterial hypertension associated with congenital heart disease: a single-center prospective study","author":"Pavsic","year":"2023","journal-title":"Hellenic J Cardiol"},{"key":"10.1016\/j.ijcchd.2024.100520_bib39","article-title":"Exercise ventilatory reserve predicts survival in adult congenital heart disease associated pulmonary arterial hypertension with Eisenmenger physiology","volume":"7","author":"Chinthaka","year":"2022","journal-title":"International Journal of Cardiology Congenital Heart Disease"},{"key":"10.1016\/j.ijcchd.2024.100520_bib40","doi-asserted-by":"crossref","first-page":"1461","DOI":"10.1161\/CIRCULATIONAHA.112.091421","article-title":"Echocardiographic predictors of outcome in Eisenmenger syndrome","volume":"126","author":"Moceri","year":"2012","journal-title":"Circulation"},{"key":"10.1016\/j.ijcchd.2024.100520_bib41","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1186\/s12968-022-00880-2","article-title":"Detection and evaluation of myocardial fibrosis in Eisenmenger syndrome using cardiovascular magnetic resonance late gadolinium enhancement and T1 mapping","volume":"24","author":"Gong","year":"2022","journal-title":"J Cardiovasc Magn Reson"},{"key":"10.1016\/j.ijcchd.2024.100520_bib42","doi-asserted-by":"crossref","first-page":"1478","DOI":"10.1002\/jmri.27791","article-title":"Diverse right ventricular remodeling evaluated by MRI and prognosis in eisenmenger syndrome with different shunt locations","volume":"55","author":"Gong","year":"2022","journal-title":"J Magn Reson Imag"},{"key":"10.1016\/j.ijcchd.2024.100520_bib43","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1016\/j.acvd.2017.01.006","article-title":"Outcome of adults with Eisenmenger syndrome treated with drugs specific to pulmonary arterial hypertension: a French multicentre study","volume":"110","author":"Hascoet","year":"2017","journal-title":"Arch Cardiovasc Dis"},{"key":"10.1016\/j.ijcchd.2024.100520_bib44","doi-asserted-by":"crossref","first-page":"1432","DOI":"10.1161\/CIRCULATIONAHA.116.023033","article-title":"Predictors of death in contemporary adult patients with eisenmenger syndrome: a multicenter study","volume":"135","author":"Kempny","year":"2017","journal-title":"Circulation"},{"key":"10.1016\/j.ijcchd.2024.100520_bib45","doi-asserted-by":"crossref","DOI":"10.1016\/j.ijcchd.2020.100070","article-title":"Mortality in adult patients with Eisenmenger Syndrome: 5-Years perspective","volume":"2","author":"Kempny","year":"2021","journal-title":"International Journal of Cardiology Congenital Heart Disease"},{"key":"10.1016\/j.ijcchd.2024.100520_bib46","doi-asserted-by":"crossref","first-page":"1562","DOI":"10.1164\/rccm.201311-2025LE","article-title":"Treatment of group I pulmonary arterial hypertension with carvedilol is safe","volume":"189","author":"Grinnan","year":"2014","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.ijcchd.2024.100520_bib47","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1183\/13993003.00090-2016","article-title":"Bisoprolol in idiopathic pulmonary arterial hypertension: an explorative study","volume":"48","author":"Van Campen","year":"2016","journal-title":"Eur Respir J"},{"key":"10.1016\/j.ijcchd.2024.100520_bib48","series-title":"American thoracic society international conference","article-title":"Beta-blocker therapy in patients with idiopathic pulmonary arterial hypertension: a randomized controlled trial","author":"Van Campen Kdb","year":"2014"},{"key":"10.1016\/j.ijcchd.2024.100520_bib49","doi-asserted-by":"crossref","first-page":"1128","DOI":"10.3390\/medicina58081128","article-title":"Effects of sodium-glucose Co-Transporter-2 inhibition on pulmonary arterial stiffness and right ventricular function in heart failure with reduced ejection fraction","volume":"58","author":"\u00c7amc\u0131","year":"2022","journal-title":"Medicina"},{"key":"10.1016\/j.ijcchd.2024.100520_bib50","doi-asserted-by":"crossref","first-page":"42","DOI":"10.3390\/jcm12010042","article-title":"Impact of SGLT2 inhibitor therapy on right ventricular function in patients with heart failure and reduced ejection fraction","volume":"12","author":"Mustapic","year":"2022","journal-title":"J Clin Med"},{"key":"10.1016\/j.ijcchd.2024.100520_bib51","doi-asserted-by":"crossref","DOI":"10.1161\/JAHA.121.024449","article-title":"Effect of sacubitril\/valsartan on the right ventricular function and pulmonary hypertension in patients with heart failure with reduced ejection fraction: a systematic review and meta\u2010analysis of observational studies","volume":"11","author":"Zhang","year":"2022","journal-title":"J Am Heart Assoc"},{"key":"10.1016\/j.ijcchd.2024.100520_bib52","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1016\/j.ijcard.2013.08.102","article-title":"A cornerstone of heart failure treatment is not effective in experimental right ventricular failure","volume":"169","author":"Borgdorff","year":"2013","journal-title":"Int J Cardiol"},{"key":"10.1016\/j.ijcchd.2024.100520_bib53","doi-asserted-by":"crossref","DOI":"10.1161\/JAHA.119.015708","article-title":"Angiotensin receptor\u2010neprilysin inhibition attenuates right ventricular remodeling in pulmonary hypertension","volume":"9","author":"Sharifi Kia","year":"2020","journal-title":"J Am Heart Assoc"},{"key":"10.1016\/j.ijcchd.2024.100520_bib54","doi-asserted-by":"crossref","first-page":"716","DOI":"10.1093\/eurheartj\/eht072","article-title":"Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: a comparison between clinical subgroups","volume":"35","author":"Manes","year":"2014","journal-title":"Eur Heart J"},{"key":"10.1016\/j.ijcchd.2024.100520_bib55","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1161\/CIRCULATIONAHA.109.883876","article-title":"Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension","volume":"121","author":"Dimopoulos","year":"2010","journal-title":"Circulation"},{"key":"10.1016\/j.ijcchd.2024.100520_bib56","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1161\/CIRCULATIONAHA.106.630715","article-title":"Bosentan Randomized Trial of Endothelin Antagonist Therapy I. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study","volume":"114","author":"Galie","year":"2006","journal-title":"Circulation"},{"key":"10.1016\/j.ijcchd.2024.100520_bib57","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1016\/j.ijcard.2007.04.078","article-title":"Longer-term bosentan therapy improves functional capacity in Eisenmenger syndrome: results of the BREATHE-5 open-label extension study","volume":"127","author":"Gatzoulis","year":"2008","journal-title":"Int J Cardiol"},{"key":"10.1016\/j.ijcchd.2024.100520_bib58","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1161\/CIRCULATIONAHA.118.033575","article-title":"Evaluation of macitentan in patients with eisenmenger syndrome","volume":"139","author":"Gatzoulis","year":"2019","journal-title":"Circulation"},{"key":"10.1016\/j.ijcchd.2024.100520_bib59","doi-asserted-by":"crossref","first-page":"424","DOI":"10.1111\/j.1747-0803.2011.00561.x","article-title":"Clinical efficacy of phosphodiesterase-5 inhibitor tadalafil in Eisenmenger syndrome--a randomized, placebo-controlled, double-blind crossover study","volume":"6","author":"Mukhopadhyay","year":"2011","journal-title":"Congenit Heart Dis"},{"key":"10.1016\/j.ijcchd.2024.100520_bib60","doi-asserted-by":"crossref","first-page":"851 e851","DOI":"10.1016\/j.ahj.2005.09.006","article-title":"A randomized, placebo-controlled, double-blind, crossover study to evaluate the efficacy of oral sildenafil therapy in severe pulmonary artery hypertension","volume":"151","author":"Singh","year":"2006","journal-title":"Am Heart J"},{"key":"10.1016\/j.ijcchd.2024.100520_bib61","doi-asserted-by":"crossref","first-page":"1834","DOI":"10.1016\/j.amjcard.2013.08.003","article-title":"Effects of inhaled iloprost on exercise capacity, quality of life, and cardiac function in patients with pulmonary arterial hypertension secondary to congenital heart disease (the Eisenmenger syndrome) (from the EIGER Study)","volume":"112","author":"Cha","year":"2013","journal-title":"Am J Cardiol"},{"key":"10.1016\/j.ijcchd.2024.100520_bib62","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1016\/j.ijcard.2019.07.004","article-title":"A single-centre, placebo-controlled, double-blind randomised cross-over study of nebulised iloprost in patients with Eisenmenger syndrome: a pilot study","volume":"299","author":"Nashat","year":"2020","journal-title":"Int J Cardiol"},{"key":"10.1016\/j.ijcchd.2024.100520_bib63","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1056\/NEJM199602013340504","article-title":"A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension","volume":"334","author":"Barst","year":"1996","journal-title":"N Engl J Med"},{"key":"10.1016\/j.ijcchd.2024.100520_bib64","doi-asserted-by":"crossref","first-page":"4117","DOI":"10.1016\/j.ijcard.2013.07.072","article-title":"Long-term effects of continuous prostacyclin therapy in adults with pulmonary hypertension associated with congenital heart disease","volume":"168","author":"Thomas","year":"2013","journal-title":"Int J Cardiol"},{"key":"10.1016\/j.ijcchd.2024.100520_bib65","doi-asserted-by":"crossref","DOI":"10.1183\/13993003.01401-2019","article-title":"The effects of parenteral prostacyclin therapy as add-on treatment to oral compounds in Eisenmenger syndrome","volume":"54","author":"D'Alto","year":"2019","journal-title":"Eur Respir J"},{"key":"10.1016\/j.ijcchd.2024.100520_bib66","doi-asserted-by":"crossref","first-page":"1195","DOI":"10.1136\/heartjnl-2017-312143","article-title":"Subcutaneous treprostinil in congenital heart disease-related pulmonary arterial hypertension","volume":"104","author":"Skoro-Sajer","year":"2018","journal-title":"Heart"},{"key":"10.1016\/j.ijcchd.2024.100520_bib67","doi-asserted-by":"crossref","first-page":"2522","DOI":"10.1056\/NEJMoa1503184","article-title":"Selexipag for the treatment of pulmonary arterial hypertension","volume":"373","author":"Sitbon","year":"2015","journal-title":"N Engl J Med"},{"key":"10.1016\/j.ijcchd.2024.100520_bib68","doi-asserted-by":"crossref","first-page":"1286","DOI":"10.1016\/j.cjca.2021.01.023","article-title":"Use of the prostacyclin receptor agonist selexipag in patients with pulmonary arterial hypertension associated with eisenmenger syndrome","volume":"37","author":"Demerouti","year":"2021","journal-title":"Can J Cardiol"},{"key":"10.1016\/j.ijcchd.2024.100520_bib69","doi-asserted-by":"crossref","DOI":"10.1016\/j.ijcchd.2021.100144","article-title":"Selexipag for pulmonary arterial hypertension in a wide range of adult congenital heart disease","volume":"4","author":"van Dissel","year":"2021","journal-title":"International Journal of Cardiology Congenital Heart Disease"},{"key":"10.1016\/j.ijcchd.2024.100520_bib70","doi-asserted-by":"crossref","first-page":"1792","DOI":"10.1136\/heartjnl-2015-307832","article-title":"Riociguat for pulmonary arterial hypertension associated with congenital heart disease","volume":"101","author":"Rosenkranz","year":"2015","journal-title":"Heart"},{"key":"10.1016\/j.ijcchd.2024.100520_bib71","doi-asserted-by":"crossref","first-page":"378","DOI":"10.1016\/j.ijcard.2010.10.051","article-title":"Bosentan-sildenafil association in patients with congenital heart disease-related pulmonary arterial hypertension and Eisenmenger physiology","volume":"155","author":"D'Alto","year":"2012","journal-title":"Int J Cardiol"},{"key":"10.1016\/j.ijcchd.2024.100520_bib72","doi-asserted-by":"crossref","first-page":"633","DOI":"10.1016\/j.ihj.2021.07.007","article-title":"A randomized, double-blind, placebo-controlled study to evaluate sildenafil, ambrisentan combination therapy in pulmonary hypertension, particularly of Eisenmenger syndrome","volume":"73","author":"Mohammed","year":"2021","journal-title":"Indian Heart J"},{"key":"10.1016\/j.ijcchd.2024.100520_bib73","doi-asserted-by":"crossref","first-page":"1423","DOI":"10.1016\/j.apmr.2009.02.015","article-title":"Six-minute walk test in patients with down syndrome: validity and reproducibility","volume":"90","author":"Vis","year":"2009","journal-title":"Arch Phys Med Rehabil"},{"key":"10.1016\/j.ijcchd.2024.100520_bib74","series-title":"Dictionary of cancer terms","year":"2024"},{"key":"10.1016\/j.ijcchd.2024.100520_bib75","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1016\/j.ijcard.2008.02.004","article-title":"Evaluating operability in adults with congenital heart disease and the role of pretreatment with targeted pulmonary arterial hypertension therapy","volume":"129","author":"Dimopoulos","year":"2008","journal-title":"Int J Cardiol"},{"key":"10.1016\/j.ijcchd.2024.100520_bib76","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1111\/jocs.12325","article-title":"Repair of ventricular septal defect with eisenmenger syndrome after bosentan treatment","volume":"29","author":"Hu","year":"2014","journal-title":"J Card Surg"},{"key":"10.1016\/j.ijcchd.2024.100520_bib77","doi-asserted-by":"crossref","first-page":"760","DOI":"10.1016\/j.jtcvs.2008.03.064","article-title":"Atrial septal defect repair after a 10-month treatment with bosentan in a patient with severe pulmonary arterial hypertension: a case report","volume":"137","author":"Hoetzenecker","year":"2009","journal-title":"J Thorac Cardiovasc Surg"},{"key":"10.1016\/j.ijcchd.2024.100520_bib78","doi-asserted-by":"crossref","first-page":"501","DOI":"10.1016\/j.healun.2004.02.004","article-title":"Reversal of pulmonary hypertension and subsequent repair of atrial septal defect after treatment with continuous intravenous epoprostenol","volume":"24","author":"Frost","year":"2005","journal-title":"J Heart Lung Transplant"},{"key":"10.1016\/j.ijcchd.2024.100520_bib79","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1136\/hrt.2008.150086","article-title":"Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001-2006","volume":"95","author":"Haworth","year":"2009","journal-title":"Heart"},{"key":"10.1016\/j.ijcchd.2024.100520_bib80","doi-asserted-by":"crossref","first-page":"2097","DOI":"10.1016\/j.chest.2020.05.597","article-title":"Not all septal defects are equal: outcomes of bilateral lung transplant with cardiac defect repair vs combined heart-lung transplant in patients with eisenmenger syndrome in the United States","volume":"158","author":"Sertic","year":"2020","journal-title":"Chest"},{"key":"10.1016\/j.ijcchd.2024.100520_bib81","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1177\/02184923231151551","article-title":"Outcomes of heart-lung transplantation in Eisenmenger syndrome compared to primary pulmonary hypertension","volume":"31","author":"Sainathan","year":"2023","journal-title":"Asian Cardiovasc Thorac Ann"},{"key":"10.1016\/j.ijcchd.2024.100520_bib82","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1016\/j.ijcard.2012.09.036","article-title":"Efficacy of exercise training in pulmonary arterial hypertension associated with congenital heart disease","volume":"168","author":"Becker-Gr\u00fcnig","year":"2013","journal-title":"Int J Cardiol"},{"key":"10.1016\/j.ijcchd.2024.100520_bib83","first-page":"195","article-title":"Effects of home-based exercise training in Eisenmenger's syndrome: sub-group analysis of a randomized controlled trial","volume":"1","author":"Babu","year":"2023","journal-title":"Heart Failure Journal of India"},{"key":"10.1016\/j.ijcchd.2024.100520_bib84","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1111\/j.1747-0803.2009.00370.x","article-title":"Rehabilitation program in adult congenital heart disease patients with pulmonary hypertension","volume":"5","author":"Mart\u00ednez-Quintana","year":"2010","journal-title":"Congenit Heart Dis"},{"key":"10.1016\/j.ijcchd.2024.100520_bib85","doi-asserted-by":"crossref","first-page":"2219","DOI":"10.1016\/j.jacc.2021.03.012","article-title":"Lifespan perspective on congenital heart disease research: JACC state-of-the-art review","volume":"77","author":"Diller","year":"2021","journal-title":"J Am Coll Cardiol"}],"container-title":["International Journal of Cardiology Congenital Heart Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2666668524000296?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2666668524000296?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2024,9,15]],"date-time":"2024-09-15T19:49:42Z","timestamp":1726429782000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S2666668524000296"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,9]]},"references-count":85,"alternative-id":["S2666668524000296"],"URL":"https:\/\/doi.org\/10.1016\/j.ijcchd.2024.100520","relation":{},"ISSN":["2666-6685"],"issn-type":[{"value":"2666-6685","type":"print"}],"subject":[],"published":{"date-parts":[[2024,9]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Update on Eisenmenger syndrome \u2013 Review of pathophysiology and recent progress in risk assessment and management","name":"articletitle","label":"Article Title"},{"value":"International Journal of Cardiology Congenital Heart Disease","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ijcchd.2024.100520","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2024 Published by Elsevier B.V.","name":"copyright","label":"Copyright"}],"article-number":"100520"}}