{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T14:51:32Z","timestamp":1790088692950,"version":"4.0.1"},"reference-count":148,"publisher":"Georg Thieme Verlag KG","issue":"06","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Semin Respir Crit Care Med"],"published-print":{"date-parts":[[2023,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Pulmonary arterial hypertension (PAH) is a rare pulmonary vascular disease characterized by progressive pulmonary arterial remodeling, increased pulmonary vascular resistance, right ventricular dysfunction, and reduced survival. Effective therapies have been developed that target three pathobiologic pathways in PAH: nitric oxide, endothelin-1, and prostacyclin. Approved therapies for PAH include phosphodiesterase type-5 inhibitors, soluble guanylate cyclase stimulators, endothelin receptor antagonists, prostacyclin analogs, and prostacyclin receptor agonists. Management of PAH in the modern era incorporates multidimensional risk assessment to guide the use of these medications. For patients with PAH and without significant comorbidities, current guidelines recommend two oral medications (phosphodiesterase type-5 inhibitor and endothelin receptor antagonist) for low- and intermediate-risk patients, with triple therapy including a parenteral prostacyclin to be considered in those at high or intermediate-high risk. Combination therapy may be poorly tolerated and less effective in patients with PAH and cardiopulmonary comorbidities. Thus, a single-agent approach with individualized decisions to add-on other PAH therapies is recommended in older patients and those with significant comorbid conditions. Management of PAH is best performed in multidisciplinary teams located in experienced centers. Other core pillars of PAH management include supportive and adjunctive treatments including oxygen, diuretics, rehabilitation, and anticoagulation in certain patients. Patients with PAH who progress despite optimal treatment or who are refractory to best medical care should be referred for lung transplantation, if eligible. Despite considerable progress, PAH is often fatal and new therapies that reverse the disease and improve outcomes are desperately needed.<\/jats:p>","DOI":"10.1055\/s-0043-1770118","type":"journal-article","created":{"date-parts":[[2023,6,27]],"date-time":"2023-06-27T22:39:58Z","timestamp":1687905598000},"page":"746-761","source":"Crossref","is-referenced-by-count":7,"title":["Management of Pulmonary Arterial Hypertension"],"prefix":"10.1055","volume":"44","author":[{"given":"Jason","family":"Weatherald","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Medicine, Department of Medicine, University of Alberta, Edmonton, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rhea A.","family":"Varughese","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Medicine, Department of Medicine, University of Alberta, Edmonton, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan","family":"Liu","sequence":"additional","affiliation":[{"name":"Division of Respirology, Department of Medicine, University of Calgary, Calgary, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marc","family":"Humbert","sequence":"additional","affiliation":[{"name":"Facult\u00e9 de M\u00e9decine, Universit\u00e9 Paris-Saclay, Le Kremlin-Bic\u00eatre, France"},{"name":"Service de Pneumologie et Soins Intensifs Respiratoires, Assistance Publique H\u00f4pitaux de Paris, H\u00f4pital Bic\u00eatre, Le Kremlin-Bic\u00eatre, France"},{"name":"H\u00f4pital Marie Lannelongue, Le Plessis Robinson, INSERM UMR_S 999, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2023,6,27]]},"reference":[{"issue":"10","key":"ref1","doi-asserted-by":"crossref","first-page":"603","DOI":"10.1038\/nrcardio.2017.84","article-title":"Epidemiology and treatment of pulmonary arterial hypertension","volume":"14","author":"E MT Lau","year":"2017","journal-title":"Nat Rev Cardiol"},{"issue":"25","key":"ref2","doi-asserted-by":"crossref","first-page":"2361","DOI":"10.1056\/NEJMra2000348","article-title":"Pulmonary arterial hypertension","volume":"385","author":"P M Hassoun","year":"2021","journal-title":"N Engl J Med"},{"issue":"01","key":"ref3","doi-asserted-by":"crossref","first-page":"1801887","DOI":"10.1183\/13993003.01887-2018","article-title":"Pathology and pathobiology of pulmonary hypertension: state of the art and research perspectives","volume":"53","author":"M Humbert","year":"2019","journal-title":"Eur Respir J"},{"issue":"02","key":"ref4","doi-asserted-by":"crossref","first-page":"1700740","DOI":"10.1183\/13993003.00740-2017","article-title":"Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model","volume":"50","author":"M M Hoeper","year":"2017","journal-title":"Eur Respir J"},{"issue":"06","key":"ref5","doi-asserted-by":"crossref","first-page":"2102419","DOI":"10.1183\/13993003.02419-2021","article-title":"External validation of a refined 4-strata risk assessment score from the French Pulmonary Hypertension Registry","volume":"59","author":"A Boucly","year":"2021","journal-title":"Eur Respir J"},{"issue":"04","key":"ref6","doi-asserted-by":"crossref","first-page":"903","DOI":"10.1183\/13993003.01032-2015","article-title":"2015 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT)","volume":"46","author":"N Gali\u00e8","year":"2015","journal-title":"Eur Respir J"},{"issue":"01","key":"ref7","doi-asserted-by":"crossref","first-page":"2200879","DOI":"10.1183\/13993003.00879-2022","article-title":"2022 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension","volume":"61","author":"M Humbert","year":"2023","journal-title":"Eur Respir J"},{"issue":"38","key":"ref8","doi-asserted-by":"crossref","first-page":"3618","DOI":"10.1093\/eurheartj\/ehac237","article-title":"2022 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension","volume":"43","author":"M Humbert","year":"2022","journal-title":"Eur Heart J"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"1884","DOI":"10.1016\/S0140-6736(22)01601-4","article-title":"The evolving landscape of pulmonary arterial hypertension clinical trials","volume":"400","author":"J Weatherald","year":"2022","journal-title":"Lancet"},{"issue":"20","key":"ref10","doi-asserted-by":"crossref","first-page":"13729","DOI":"10.1074\/jbc.274.20.13729","article-title":"Cyclic GMP phosphodiesterase-5: target of sildenafil","volume":"274","author":"J D Corbin","year":"1999","journal-title":"J Biol Chem"},{"issue":"02","key":"ref11","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1007\/s00395-004-0504-5","article-title":"Antiproliferative effect of sildenafil on human pulmonary artery smooth muscle cells","volume":"100","author":"B Tantini","year":"2005","journal-title":"Basic Res Cardiol"},{"issue":"20","key":"ref12","doi-asserted-by":"crossref","first-page":"2148","DOI":"10.1056\/NEJMoa050010","article-title":"Sildenafil citrate therapy for pulmonary arterial hypertension","volume":"353","author":"N Gali\u00e8","year":"2005","journal-title":"N Engl J Med"},{"issue":"12","key":"ref13","first-page":"2417","article-title":"Sildenafil for pulmonary arterial hypertension associated with connective tissue disease","volume":"34","author":"D B Badesch","year":"2007","journal-title":"J Rheumatol"},{"issue":"22","key":"ref14","doi-asserted-by":"crossref","first-page":"2894","DOI":"10.1161\/CIRCULATIONAHA.108.839274","article-title":"Tadalafil therapy for pulmonary arterial hypertension","volume":"119","author":"N Gali\u00e8","year":"2009","journal-title":"Circulation"},{"issue":"01","key":"ref15","first-page":"CD012621","article-title":"Phosphodiesterase 5 inhibitors for pulmonary hypertension","volume":"1","author":"H Barnes","year":"2019","journal-title":"Cochrane Database Syst Rev"},{"issue":"06","key":"ref16","doi-asserted-by":"crossref","first-page":"679","DOI":"10.1586\/17476348.2015.1106316","article-title":"Riociguat for the treatment of pulmonary hypertension","volume":"9","author":"N Hambly","year":"2015","journal-title":"Expert Rev Respir Med"},{"issue":"04","key":"ref17","doi-asserted-by":"crossref","first-page":"881","DOI":"10.1183\/09031936.00114407","article-title":"Expression and function of soluble guanylate cyclase in pulmonary arterial hypertension","volume":"32","author":"R T Schermuly","year":"2008","journal-title":"Eur Respir J"},{"issue":"04","key":"ref18","doi-asserted-by":"crossref","first-page":"330","DOI":"10.1056\/NEJMoa1209655","article-title":"Riociguat for the treatment of pulmonary arterial hypertension","volume":"369","author":"H-A Ghofrani","year":"2013","journal-title":"N Engl J Med"},{"issue":"05","key":"ref19","doi-asserted-by":"crossref","first-page":"1303","DOI":"10.1183\/09031936.00090614","article-title":"Riociguat for the treatment of pulmonary arterial hypertension: a long-term extension study (PATENT-2)","volume":"45","author":"L J Rubin","year":"2015","journal-title":"Eur Respir J"},{"issue":"02","key":"ref20","doi-asserted-by":"crossref","first-page":"422","DOI":"10.1136\/annrheumdis-2015-209087","article-title":"Riociguat for the treatment of pulmonary arterial hypertension associated with connective tissue disease: results from PATENT-1 and PATENT-2","volume":"76","author":"M Humbert","year":"2017","journal-title":"Ann Rheum Dis"},{"issue":"22","key":"ref21","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":"S Rosenkranz","year":"2015","journal-title":"Heart"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"106241","DOI":"10.1016\/j.rmed.2020.106241","article-title":"Riociguat treatment in patients with pulmonary arterial hypertension: Final safety data from the EXPERT registry","volume":"177","author":"M M Hoeper","year":"2020","journal-title":"Respir Med"},{"issue":"05","key":"ref23","doi-asserted-by":"crossref","first-page":"1314","DOI":"10.1183\/09031936.00105914","article-title":"PATENT PLUS: a blinded, randomised and extension study of riociguat plus sildenafil in pulmonary arterial hypertension","volume":"45","author":"N Gali\u00e8","year":"2015","journal-title":"Eur Respir J"},{"issue":"24","key":"ref24","doi-asserted-by":"crossref","first-page":"1732","DOI":"10.1056\/NEJM199306173282402","article-title":"Expression of endothelin-1 in the lungs of patients with pulmonary hypertension","volume":"328","author":"A Giaid","year":"1993","journal-title":"N Engl J Med"},{"issue":"06","key":"ref25","doi-asserted-by":"crossref","first-page":"464","DOI":"10.7326\/0003-4819-114-6-464","article-title":"Increased plasma endothelin-1 in pulmonary hypertension: marker or mediator of disease?","volume":"114","author":"D J Stewart","year":"1991","journal-title":"Ann Intern Med"},{"issue":"03","key":"ref26","doi-asserted-by":"crossref","first-page":"398","DOI":"10.1164\/ajrccm.165.3.2104059","article-title":"ET(A) and ET(B) receptors modulate the proliferation of human pulmonary artery smooth muscle cells","volume":"165","author":"N Davie","year":"2002","journal-title":"Am J Respir Crit Care Med"},{"issue":"12","key":"ref27","doi-asserted-by":"crossref","first-page":"896","DOI":"10.1056\/NEJMoa012212","article-title":"Bosentan therapy for pulmonary arterial hypertension","volume":"346","author":"L J Rubin","year":"2002","journal-title":"N Engl J Med"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"2093","DOI":"10.1016\/S0140-6736(08)60919-8","article-title":"Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial","volume":"371","author":"N Gali\u00e8","year":"2008","journal-title":"Lancet"},{"issue":"02","key":"ref29","doi-asserted-by":"crossref","first-page":"338","DOI":"10.1183\/09031936.00138706","article-title":"Results of European post-marketing surveillance of bosentan in pulmonary hypertension","volume":"30","author":"M Humbert","year":"2007","journal-title":"Eur Respir J"},{"issue":"23","key":"ref30","doi-asserted-by":"crossref","first-page":"3010","DOI":"10.1161\/CIRCULATIONAHA.107.742510","article-title":"Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2","volume":"117","author":"N Gali\u00e8","year":"2008","journal-title":"Circulation"},{"issue":"21","key":"ref31","doi-asserted-by":"crossref","first-page":"1971","DOI":"10.1016\/j.jacc.2009.07.033","article-title":"Long-term ambrisentan therapy for the treatment of pulmonary arterial hypertension","volume":"54","author":"R J Oudiz","year":"2009","journal-title":"J Am Coll Cardiol"},{"issue":"03","key":"ref32","doi-asserted-by":"crossref","first-page":"736","DOI":"10.1124\/jpet.108.142976","article-title":"Pharmacology of macitentan, an orally active tissue-targeting dual endothelin receptor antagonist","volume":"327","author":"M Iglarz","year":"2008","journal-title":"J Pharmacol Exp Ther"},{"issue":"09","key":"ref33","doi-asserted-by":"crossref","first-page":"809","DOI":"10.1056\/NEJMoa1213917","article-title":"Macitentan and morbidity and mortality in pulmonary arterial hypertension","volume":"369","author":"T Pulido","year":"2013","journal-title":"N Engl J Med"},{"issue":"04","key":"ref34","doi-asserted-by":"crossref","first-page":"287","DOI":"10.1007\/s40264-015-0365-x","article-title":"Comparative safety and tolerability of prostacyclins in pulmonary hypertension","volume":"39","author":"C O'Connell","year":"2016","journal-title":"Drug Saf"},{"issue":"05","key":"ref35","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":"R J Barst","year":"1996","journal-title":"N Engl J Med"},{"issue":"02","key":"ref36","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1016\/j.ahj.2013.08.008","article-title":"Two formulations of epoprostenol sodium in the treatment of pulmonary arterial hypertension: EPITOME-1 (epoprostenol for injection in pulmonary arterial hypertension), a phase IV, open-label, randomized study","volume":"167","author":"K M Chin","year":"2014","journal-title":"Am Heart J"},{"issue":"06","key":"ref37","doi-asserted-by":"crossref","first-page":"800","DOI":"10.1164\/ajrccm.165.6.2106079","article-title":"Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial","volume":"165","author":"G Simonneau","year":"2002","journal-title":"Am J Respir Crit Care Med"},{"issue":"02","key":"ref38","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1016\/j.healun.2009.09.005","article-title":"Exercise improvement and plasma biomarker changes with intravenous treprostinil therapy for pulmonary arterial hypertension: a placebo-controlled trial","volume":"29","author":"J Hiremath","year":"2010","journal-title":"J Heart Lung Transplant"},{"issue":"03","key":"ref39","doi-asserted-by":"crossref","first-page":"683","DOI":"10.1378\/chest.129.3.683","article-title":"Safety and efficacy of IV treprostinil for pulmonary arterial hypertension: a prospective, multicenter, open-label, 12-week trial","volume":"129","author":"V F Tapson","year":"2006","journal-title":"Chest"},{"issue":"18","key":"ref40","doi-asserted-by":"crossref","first-page":"1915","DOI":"10.1016\/j.jacc.2010.01.027","article-title":"Addition of inhaled treprostinil to oral therapy for pulmonary arterial hypertension: a randomized controlled clinical trial","volume":"55","author":"V V McLaughlin","year":"2010","journal-title":"J Am Coll Cardiol"},{"issue":"03","key":"ref41","doi-asserted-by":"crossref","first-page":"e12119","DOI":"10.1002\/pul2.12119","article-title":"INSPIRE: Safety and tolerability of inhaled Yutrepia (treprostinil) in pulmonary arterial hypertension (PAH)","volume":"12","author":"N S Hill","year":"2022","journal-title":"Pulm Circ"},{"issue":"06","key":"ref42","doi-asserted-by":"crossref","first-page":"1383","DOI":"10.1378\/chest.11-2212","article-title":"Oral treprostinil for the treatment of pulmonary arterial hypertension in patients on background endothelin receptor antagonist and\/or phosphodiesterase type 5 inhibitor therapy (the FREEDOM-C study): a randomized controlled trial","volume":"142","author":"V F Tapson","year":"2012","journal-title":"Chest"},{"issue":"03","key":"ref43","doi-asserted-by":"crossref","first-page":"952","DOI":"10.1378\/chest.12-2875","article-title":"Oral treprostinil for the treatment of pulmonary arterial hypertension in patients receiving background endothelin receptor antagonist and phosphodiesterase type 5 inhibitor therapy (the FREEDOM-C2 study): a randomized controlled trial","volume":"144","author":"V F Tapson","year":"2013","journal-title":"Chest"},{"issue":"05","key":"ref44","doi-asserted-by":"crossref","first-page":"624","DOI":"10.1161\/CIRCULATIONAHA.112.124388","article-title":"Efficacy and safety of oral treprostinil monotherapy for the treatment of pulmonary arterial hypertension: a randomized, controlled trial","volume":"127","author":"Z-C Jing","year":"2013","journal-title":"Circulation"},{"issue":"06","key":"ref45","doi-asserted-by":"crossref","first-page":"707","DOI":"10.1164\/rccm.201908-1640OC","article-title":"Combination therapy with oral treprostinil for pulmonary arterial hypertension. A double-blind placebo-controlled clinical trial","volume":"201","author":"R J White","year":"2020","journal-title":"Am J Respir Crit Care Med"},{"issue":"05","key":"ref46","doi-asserted-by":"crossref","first-page":"322","DOI":"10.1056\/NEJMoa020204","article-title":"Inhaled iloprost for severe pulmonary hypertension","volume":"347","author":"H Olschewski","year":"2002","journal-title":"N Engl J Med"},{"issue":"11","key":"ref47","doi-asserted-by":"crossref","first-page":"1257","DOI":"10.1164\/rccm.200603-358OC","article-title":"Randomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertension","volume":"174","author":"V V McLaughlin","year":"2006","journal-title":"Am J Respir Crit Care Med"},{"issue":"04","key":"ref48","doi-asserted-by":"crossref","first-page":"691","DOI":"10.1183\/09031936.06.00057906","article-title":"Combining inhaled iloprost with bosentan in patients with idiopathic pulmonary arterial hypertension","volume":"28","author":"M M Hoeper","year":"2006","journal-title":"Eur Respir J"},{"issue":"03","key":"ref49","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1007\/s40256-015-0117-4","article-title":"Pharmacokinetics and tolerability of the novel oral prostacyclin IP receptor agonist selexipag","volume":"15","author":"P Kaufmann","year":"2015","journal-title":"Am J Cardiovasc Drugs"},{"issue":"02","key":"ref50","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1111\/bcp.12963","article-title":"Pharmacokinetics of the novel oral prostacyclin receptor agonist selexipag in subjects with hepatic or renal impairment","volume":"82","author":"P Kaufmann","year":"2016","journal-title":"Br J Clin Pharmacol"},{"issue":"26","key":"ref51","doi-asserted-by":"crossref","first-page":"2522","DOI":"10.1056\/NEJMoa1503184","article-title":"Selexipag for the treatment of pulmonary arterial hypertension","volume":"373","author":"O Sitbon","year":"2015","journal-title":"N Engl J Med"},{"issue":"03","key":"ref52","doi-asserted-by":"crossref","first-page":"2045894020931324","DOI":"10.1177\/2045894020931324","article-title":"Transitions between infused and oral prostacyclin pathway agents in pulmonary arterial hypertension: key considerations","volume":"10","author":"T Sargent","year":"2020","journal-title":"Pulm Circ"},{"issue":"03","key":"ref53","doi-asserted-by":"crossref","first-page":"2045894019862167","DOI":"10.1177\/2045894019862167","article-title":"EXPRESS: transition from parental prostacyclin to selexipag: a case series of five pulmonary arterial hypertension patients","volume":"9","author":"N Holthaus","year":"2019","journal-title":"Pulm Circ"},{"issue":"23","key":"ref54","doi-asserted-by":"crossref","first-page":"1877","DOI":"10.2146\/ajhp170814","article-title":"Transition from treprostinil to selexipag in patients with pulmonary arterial hypertension: case series","volume":"75","author":"S M Fanous","year":"2018","journal-title":"Am J Health Syst Pharm"},{"issue":"04","key":"ref55","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1097\/FJC.0000000000000800","article-title":"Experience in transitioning from parenteral prostacyclins to selexipag in pulmonary arterial hypertension","volume":"75","author":"K S Parikh","year":"2020","journal-title":"J Cardiovasc Pharmacol"},{"issue":"01","key":"ref56","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1016\/j.healun.2018.09.003","article-title":"Safety and tolerability of transition from inhaled treprostinil to oral selexipag in pulmonary arterial hypertension: Results from the TRANSIT-1 study","volume":"38","author":"A Frost","year":"2019","journal-title":"J Heart Lung Transplant"},{"issue":"06","key":"ref57","doi-asserted-by":"crossref","first-page":"1902418","DOI":"10.1183\/13993003.02418-2019","article-title":"Transition from intravenous epoprostenol to selexipag in pulmonary arterial hypertension: a word of caution","volume":"55","author":"K Yanaka","year":"2020","journal-title":"Eur Respir J"},{"issue":"01","key":"ref58","doi-asserted-by":"crossref","first-page":"1801913","DOI":"10.1183\/13993003.01913-2018","article-title":"Haemodynamic definitions and updated clinical classification of pulmonary hypertension","volume":"53","author":"G Simonneau","year":"2019","journal-title":"Eur Respir J"},{"issue":"23","key":"ref59","doi-asserted-by":"crossref","first-page":"3105","DOI":"10.1161\/CIRCULATIONAHA.104.488486","article-title":"Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension","volume":"111","author":"O Sitbon","year":"2005","journal-title":"Circulation"},{"issue":"04","key":"ref60","doi-asserted-by":"crossref","first-page":"464","DOI":"10.1164\/rccm.201508-1678OC","article-title":"Critical genomic networks and vasoreactive variants in idiopathic pulmonary arterial hypertension","volume":"194","author":"A R Hemnes","year":"2016","journal-title":"Am J Respir Crit Care Med"},{"issue":"04","key":"ref61","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1161\/CIRCULATIONAHA.114.013317","article-title":"Peripheral blood signature of vasodilator-responsive pulmonary arterial hypertension","volume":"131","author":"A R Hemnes","year":"2015","journal-title":"Circulation"},{"issue":"02","key":"ref62","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1056\/NEJM199207093270203","article-title":"The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension","volume":"327","author":"S Rich","year":"1992","journal-title":"N Engl J Med"},{"issue":"15","key":"ref63","doi-asserted-by":"crossref","first-page":"1898","DOI":"10.1093\/eurheartj\/ehq170","article-title":"Long-term response to calcium-channel blockers in non-idiopathic pulmonary arterial hypertension","volume":"31","author":"D Montani","year":"2010","journal-title":"Eur Heart J"},{"issue":"13","key":"ref64","doi-asserted-by":"crossref","first-page":"1204","DOI":"10.1056\/NEJMoa2024277","article-title":"Sotatercept for the treatment of pulmonary arterial hypertension","volume":"384","author":"M Humbert","year":"2021","journal-title":"N Engl J Med"},{"issue":"01","key":"ref65","doi-asserted-by":"crossref","first-page":"2201347","DOI":"10.1183\/13993003.01347-2022","article-title":"Sotatercept for the treatment of pulmonary arterial hypertension: PULSAR open-label extension","volume":"61","author":"M Humbert","year":"2023","journal-title":"Eur Respir J"},{"issue":"16","key":"ref66","doi-asserted-by":"crossref","first-page":"1478","DOI":"10.1056\/NEJMoa2213558","article-title":"Phase 3 trial of sotatercept for treatment of pulmonary arterial hypertension","volume":"388","author":"M M Hoeper","year":"2023","journal-title":"N Engl J Med"},{"issue":"02","key":"ref67","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1016\/j.chest.2019.02.004","article-title":"Predicting survival in patients with pulmonary arterial hypertension: the REVEAL Risk Score Calculator 2.0 and comparison with ESC\/ERS-based risk assessment strategies","volume":"156","author":"R L Benza","year":"2019","journal-title":"Chest"},{"issue":"01","key":"ref68","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1016\/j.chest.2020.08.2069","article-title":"Development and validation of an abridged version of the REVEAL 2.0 risk score calculator, REVEAL Lite 2, for use in patients with pulmonary arterial hypertension","volume":"159","author":"R L Benza","year":"2021","journal-title":"Chest"},{"issue":"47","key":"ref69","doi-asserted-by":"crossref","first-page":"4175","DOI":"10.1093\/eurheartj\/ehx257","article-title":"A comprehensive risk stratification at early follow-up determines prognosis in pulmonary arterial hypertension","volume":"39","author":"D Kylhammar","year":"2018","journal-title":"Eur Heart J"},{"issue":"01","key":"ref70","doi-asserted-by":"crossref","first-page":"2102311","DOI":"10.1183\/13993003.02311-2021","article-title":"COMPERA 2.0: a refined 4-strata risk assessment model for pulmonary arterial hypertension","volume":"60","author":"M M Hoeper","year":"2021","journal-title":"Eur Respir J"},{"issue":"02","key":"ref71","doi-asserted-by":"crossref","first-page":"1700889","DOI":"10.1183\/13993003.00889-2017","article-title":"Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension","volume":"50","author":"A Boucly","year":"2017","journal-title":"Eur Respir J"},{"issue":"04","key":"ref72","doi-asserted-by":"crossref","first-page":"1800678","DOI":"10.1183\/13993003.00678-2018","article-title":"Haemodynamics and serial risk assessment in systemic sclerosis associated pulmonary arterial hypertension","volume":"52","author":"J Weatherald","year":"2018","journal-title":"Eur Respir J"},{"issue":"01","key":"ref73","doi-asserted-by":"crossref","first-page":"1801889","DOI":"10.1183\/13993003.01889-2018","article-title":"Risk stratification and medical therapy of pulmonary arterial hypertension","volume":"53","author":"N Gali\u00e8","year":"2019","journal-title":"Eur Respir J"},{"issue":"09","key":"ref74","doi-asserted-by":"crossref","first-page":"834","DOI":"10.1056\/NEJMoa1413687","article-title":"Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension","volume":"373","author":"N Gali\u00e8","year":"2015","journal-title":"N Engl J Med"},{"issue":"03","key":"ref75","doi-asserted-by":"crossref","first-page":"2000673","DOI":"10.1183\/13993003.00673-2020","article-title":"Initial combination therapy of macitentan and tadalafil in pulmonary arterial hypertension","volume":"56","author":"O Sitbon","year":"2020","journal-title":"Eur Respir J"},{"issue":"06","key":"ref76","doi-asserted-by":"crossref","first-page":"1727","DOI":"10.1183\/13993003.02043-2015","article-title":"Initial dual oral combination therapy in pulmonary arterial hypertension","volume":"47","author":"O Sitbon","year":"2016","journal-title":"Eur Respir J"},{"issue":"06","key":"ref77","doi-asserted-by":"crossref","first-page":"1700007","DOI":"10.1183\/13993003.00007-2017","article-title":"Upfront combination therapy reduces right ventricular volumes in pulmonary arterial hypertension","volume":"49","author":"M C van de Veerdonk","year":"2017","journal-title":"Eur Respir J"},{"issue":"09","key":"ref79","doi-asserted-by":"crossref","first-page":"1102","DOI":"10.1164\/rccm.201507-1398OC","article-title":"Ambrisentan and tadalafil up-front combination therapy in scleroderma-associated pulmonary arterial hypertension","volume":"192","author":"P M Hassoun","year":"2015","journal-title":"Am J Respir Crit Care Med"},{"issue":"05","key":"ref80","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1016\/j.healun.2022.01.002","article-title":"Upfront riociguat and ambrisentan combination therapy for newly diagnosed pulmonary arterial hypertension: a prospective open-label trial","volume":"41","author":"J Weatherald","year":"2022","journal-title":"J Heart Lung Transplant"},{"issue":"01","key":"ref81","doi-asserted-by":"crossref","first-page":"2045894019826944","DOI":"10.1177\/2045894019826944","article-title":"Clinical and hemodynamic benefit of macitentan and riociguat upfront combination in patients with pulmonary arterial hypertension","volume":"9","author":"R Sulica","year":"2019","journal-title":"Pulm Circ"},{"issue":"06","key":"ref82","doi-asserted-by":"crossref","first-page":"1691","DOI":"10.1183\/09031936.00116313","article-title":"Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study","volume":"43","author":"O Sitbon","year":"2014","journal-title":"Eur Respir J"},{"issue":"02","key":"ref83","doi-asserted-by":"crossref","first-page":"376","DOI":"10.1016\/j.chest.2019.09.009","article-title":"Risk reduction and right heart reverse remodeling by upfront triple combination therapy in pulmonary arterial hypertension","volume":"157","author":"M D'Alto","year":"2020","journal-title":"Chest"},{"issue":"07","key":"ref84","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":"A Boucly","year":"2021","journal-title":"Am J Respir Crit Care Med"},{"issue":"14","key":"ref85","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":"K M Chin","year":"2021","journal-title":"J Am Coll Cardiol"},{"issue":"06","key":"ref86","doi-asserted-by":"crossref","first-page":"573","DOI":"10.1016\/S2213-2600(20)30532-4","article-title":"Switching to riociguat versus maintenance therapy with phosphodiesterase-5 inhibitors in patients with pulmonary arterial hypertension (REPLACE): a multicentre, open-label, randomised controlled trial","volume":"9","author":"M M Hoeper","year":"2021","journal-title":"Lancet Respir Med"},{"issue":"01","key":"ref87","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1007\/s40256-017-0262-z","article-title":"Targeting the prostacyclin pathway with selexipag in patients with pulmonary arterial hypertension receiving double combination therapy: insights from the randomized controlled GRIPHON study","volume":"18","author":"J G Coghlan","year":"2018","journal-title":"Am J Cardiovasc Drugs"},{"issue":"05","key":"ref88","doi-asserted-by":"crossref","first-page":"1800629","DOI":"10.1183\/13993003.00629-2018","article-title":"Age, risk and outcomes in idiopathic pulmonary arterial hypertension","volume":"51","author":"M M Hoeper","year":"2018","journal-title":"Eur Respir J"},{"issue":"12","key":"ref89","doi-asserted-by":"crossref","first-page":"1286","DOI":"10.1016\/j.healun.2019.09.010","article-title":"Patients with pulmonary arterial hypertension with and without cardiovascular risk factors: Results from the AMBITION trial","volume":"38","author":"V V McLaughlin","year":"2019","journal-title":"J Heart Lung Transplant"},{"issue":"12","key":"ref90","doi-asserted-by":"crossref","first-page":"1435","DOI":"10.1016\/j.healun.2020.09.011","article-title":"Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry","volume":"39","author":"M M Hoeper","year":"2020","journal-title":"J Heart Lung Transplant"},{"issue":"10","key":"ref91","doi-asserted-by":"crossref","first-page":"937","DOI":"10.1016\/S2213-2600(22)00097-2","article-title":"Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis","volume":"10","author":"M M Hoeper","year":"2022","journal-title":"Lancet Respir Med"},{"issue":"05","key":"ref92","doi-asserted-by":"crossref","first-page":"1702310","DOI":"10.1183\/13993003.02310-2017","article-title":"Impact of age and comorbidity on risk stratification in idiopathic pulmonary arterial hypertension","volume":"51","author":"C Hjalmarsson","year":"2018","journal-title":"Eur Respir J"},{"issue":"11","key":"ref93","doi-asserted-by":"crossref","first-page":"1349","DOI":"10.1016\/j.healun.2021.07.005","article-title":"Consensus document for the selection of lung transplant candidates: an update from the International Society for Heart and Lung Transplantation","volume":"40","author":"L E Leard","year":"2021","journal-title":"J Heart Lung Transplant"},{"issue":"03","key":"ref94","doi-asserted-by":"crossref","first-page":"300","DOI":"10.1164\/rccm.202201-0217OC","article-title":"The lung allocation score remains inequitable for patients with PAH, even after the 2015 revision","volume":"207","author":"N A Kolaitis","year":"2022","journal-title":"Am J Respir Crit Care Med"},{"issue":"10","key":"ref95","doi-asserted-by":"crossref","first-page":"1056","DOI":"10.1016\/j.healun.2019.08.004","article-title":"The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: Thirty-sixth adult heart transplantation report - 2019; focus theme: donor and recipient size match","volume":"38","author":"K K Khush","year":"2019","journal-title":"J Heart Lung Transplant"},{"issue":"05","key":"ref96","doi-asserted-by":"crossref","first-page":"527","DOI":"10.1164\/rccm.201210-1865OC","article-title":"Clinical risk factors for primary graft dysfunction after lung transplantation","volume":"187","author":"J M Diamond","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"issue":"10","key":"ref97","doi-asserted-by":"crossref","first-page":"1514","DOI":"10.1513\/AnnalsATS.201610-810OC","article-title":"Clinical risk factors and prognostic model for primary graft dysfunction after lung transplantation in patients with pulmonary hypertension","volume":"14","author":"M K Porteous","year":"2017","journal-title":"Ann Am Thorac Soc"},{"issue":"10","key":"ref98","doi-asserted-by":"crossref","first-page":"1454","DOI":"10.1016\/j.healun.2004.11.049","article-title":"Report of the ISHLT Working Group on Primary Lung Graft Dysfunction part II: definition. A consensus statement of the International Society for Heart and Lung Transplantation","volume":"24","author":"J D Christie","year":"2005","journal-title":"J Heart Lung Transplant"},{"issue":"03","key":"ref99","doi-asserted-by":"crossref","first-page":"1316","DOI":"10.1016\/j.jtcvs.2017.09.161","article-title":"Extracorporeal life support as a bridge to lung transplantation-experience of a high-volume transplant center","volume":"155","author":"K Hoetzenecker","year":"2018","journal-title":"J Thorac Cardiovasc Surg"},{"issue":"12","key":"ref100","doi-asserted-by":"crossref","first-page":"1275","DOI":"10.1016\/j.healun.2019.09.004","article-title":"Extracorporeal life support bridge for pulmonary hypertension: a high-volume single-center experience","volume":"38","author":"E B Rosenzweig","year":"2019","journal-title":"J Heart Lung Transplant"},{"issue":"01","key":"ref101","doi-asserted-by":"crossref","first-page":"1801906","DOI":"10.1183\/13993003.01906-2018","article-title":"Intensive care, right ventricular support and lung transplantation in patients with pulmonary hypertension","volume":"53","author":"M M Hoeper","year":"2019","journal-title":"Eur Respir J"},{"issue":"15","key":"ref102","doi-asserted-by":"crossref","first-page":"1159","DOI":"10.1093\/eurheartj\/ehx099","article-title":"Effect of breathing oxygen-enriched air on exercise performance in patients with precapillary pulmonary hypertension: randomized, sham-controlled cross-over trial","volume":"38","author":"S Ulrich","year":"2017","journal-title":"Eur Heart J"},{"issue":"02","key":"ref103","doi-asserted-by":"crossref","first-page":"1900276","DOI":"10.1183\/13993003.002762019","article-title":"Effect of domiciliary oxygen therapy on exercise capacity and quality of life in patients with pulmonary arterial or chronic thromboembolic pulmonary hypertension: a randomised, placebo-controlled trial","volume":"54","author":"S Ulrich","year":"2019","journal-title":"Eur Respir J"},{"issue":"04","key":"ref104","doi-asserted-by":"crossref","first-page":"580","DOI":"10.1161\/01.CIR.70.4.580","article-title":"Primary pulmonary hypertension: natural history and the importance of thrombosis","volume":"70","author":"V Fuster","year":"1984","journal-title":"Circulation"},{"issue":"09","key":"ref105","doi-asserted-by":"crossref","first-page":"e004757","DOI":"10.1161\/CIRCOUTCOMES.118.004757","article-title":"Is anticoagulation beneficial in pulmonary arterial hypertension?","volume":"11","author":"M S Khan","year":"2018","journal-title":"Circ Cardiovasc Qual Outcomes"},{"issue":"08","key":"ref106","doi-asserted-by":"crossref","first-page":"879","DOI":"10.1016\/j.cjca.2014.04.016","article-title":"Oral anticoagulation for pulmonary arterial hypertension: systematic review and meta-analysis","volume":"30","author":"D Caldeira","year":"2014","journal-title":"Can J Cardiol"},{"key":"ref107","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1016\/j.thromres.2020.08.024","article-title":"Can anticoagulants improve the survival rate for patients with idiopathic pulmonary arterial hypertension? A systematic review and meta-analysis","volume":"196","author":"P Wang","year":"2020","journal-title":"Thromb Res"},{"issue":"02","key":"ref108","doi-asserted-by":"crossref","first-page":"276","DOI":"10.3899\/jrheum.110765","article-title":"Warfarin in systemic sclerosis-associated and idiopathic pulmonary arterial hypertension. A Bayesian approach to evaluating treatment for uncommon disease","volume":"39","author":"S R Johnson","year":"2012","journal-title":"J Rheumatol"},{"issue":"01","key":"ref109","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1161\/CIRCULATIONAHA.113.004526","article-title":"Anticoagulation and survival in pulmonary arterial hypertension: results from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA)","volume":"129","author":"K M Olsson","year":"2014","journal-title":"Circulation"},{"issue":"25","key":"ref110","doi-asserted-by":"crossref","first-page":"2403","DOI":"10.1161\/CIRCULATIONAHA.115.018435","article-title":"Effect of warfarin treatment on survival of patients with pulmonary arterial hypertension (PAH) in the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL)","volume":"132","author":"I R Preston","year":"2015","journal-title":"Circulation"},{"key":"ref111","doi-asserted-by":"crossref","first-page":"100579","DOI":"10.1016\/j.blre.2019.05.003","article-title":"Indications and potential pitfalls of anticoagulants in pulmonary hypertension: Would DOACs become a better option than VKAs?","volume":"37","author":"V Margelidon-Cozzolino","year":"2019","journal-title":"Blood Rev"},{"issue":"05","key":"ref112","doi-asserted-by":"crossref","first-page":"882","DOI":"10.1111\/j.1532-5415.2009.02223.x","article-title":"Risk factors for community-acquired pneumonia in immunocompetent seniors","volume":"57","author":"M L Jackson","year":"2009","journal-title":"J Am Geriatr Soc"},{"issue":"10","key":"ref113","doi-asserted-by":"crossref","first-page":"984","DOI":"10.1136\/thoraxjnl-2015-206780","article-title":"Which individuals are at increased risk of pneumococcal disease and why? Impact of COPD, asthma, smoking, diabetes, and\/or chronic heart disease on community-acquired pneumonia and invasive pneumococcal disease","volume":"70","author":"A Torres","year":"2015","journal-title":"Thorax"},{"issue":"02","key":"ref114","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1016\/j.vaccine.2017.11.078","article-title":"Cardioprotective effect of influenza and pneumococcal vaccination in patients with cardiovascular diseases","volume":"36","author":"A Ciszewski","year":"2018","journal-title":"Vaccine"},{"issue":"04","key":"ref115","doi-asserted-by":"crossref","first-page":"2102276","DOI":"10.1183\/13993003.02276-2021","article-title":"The paradox of pulmonary arterial hypertension in Italy in the COVID-19 era: is risk of disease progression around the corner?","volume":"60","author":"R Badagliacca","year":"2022","journal-title":"Eur Respir J"},{"issue":"05","key":"ref116","doi-asserted-by":"crossref","first-page":"573","DOI":"10.1164\/rccm.202112-2761OC","article-title":"COVID-19 in patients with pulmonary hypertension: a national prospective cohort study","volume":"206","author":"D Montani","year":"2022","journal-title":"Am J Respir Crit Care Med"},{"issue":"12","key":"ref117","doi-asserted-by":"crossref","first-page":"1395","DOI":"10.3390\/vaccines9121395","article-title":"COVID-19 vaccination in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: safety profile and reasons for opting against vaccination","volume":"9","author":"M Wieteska-Mi\u0142ek","year":"2021","journal-title":"Vaccines (Basel)"},{"issue":"08","key":"ref118","doi-asserted-by":"crossref","first-page":"800","DOI":"10.1016\/j.healun.2014.04.003","article-title":"Emotional symptoms and quality of life in patients with pulmonary arterial hypertension","volume":"33","author":"J MM Vanhoof","year":"2014","journal-title":"J Heart Lung Transplant"},{"key":"ref119","doi-asserted-by":"crossref","first-page":"349","DOI":"10.2147\/VHRM.S147173","article-title":"Anxiety and depression in patients with pulmonary hypertension: impact and management challenges","volume":"14","author":"M Bussotti","year":"2018","journal-title":"Vasc Health Risk Manag"},{"issue":"130","key":"ref120","doi-asserted-by":"crossref","first-page":"535","DOI":"10.1183\/09059180.00005713","article-title":"Understanding the impact of pulmonary arterial hypertension on patients' and carers' lives","volume":"22","author":"L Guillevin","year":"2013","journal-title":"Eur Respir Rev"},{"issue":"01","key":"ref121","doi-asserted-by":"crossref","first-page":"e000876","DOI":"10.1136\/bmjresp-2021-000876","article-title":"Depression, anxiety and psychological distress in patients with pulmonary hypertension: a mixed-methods study","volume":"8","author":"Y Takita","year":"2021","journal-title":"BMJ Open Respir Res"},{"key":"ref122","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1016\/j.jpsychores.2017.12.003","article-title":"Impact of psychological factors on the health-related quality of life of patients treated for pulmonary arterial hypertension","volume":"105","author":"L Halimi","year":"2018","journal-title":"J Psychosom Res"},{"issue":"01","key":"ref123","doi-asserted-by":"crossref","first-page":"104","DOI":"10.1186\/1465-9921-14-104","article-title":"Anxiety and depression disorders in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension","volume":"14","author":"D Harzheim","year":"2013","journal-title":"Respir Res"},{"issue":"06","key":"ref124","doi-asserted-by":"crossref","first-page":"831","DOI":"10.1097\/01.psy.0000145593.37594.39","article-title":"Anxiety and depression in patients with pulmonary hypertension","volume":"66","author":"B L\u00f6we","year":"2004","journal-title":"Psychosom Med"},{"key":"ref125","doi-asserted-by":"crossref","first-page":"667602","DOI":"10.3389\/fpsyt.2021.667602","article-title":"Prevalence of mental disorders and impact on quality of life in patients with pulmonary arterial hypertension","volume":"12","author":"K M Olsson","year":"2021","journal-title":"Front Psychiatry"},{"issue":"08","key":"ref126","doi-asserted-by":"crossref","first-page":"1404","DOI":"10.1513\/AnnalsATS.202103-325OC","article-title":"A systematic evaluation of the quality, accuracy, and reliability of internet websites about pulmonary arterial hypertension","volume":"19","author":"D Saleh","year":"2022","journal-title":"Ann Am Thorac Soc"},{"issue":"01","key":"ref127","doi-asserted-by":"crossref","first-page":"1801899","DOI":"10.1183\/13993003.01899-2018","article-title":"Genetics and genomics of pulmonary arterial hypertension","volume":"53","author":"N W Morrell","year":"2019","journal-title":"Eur Respir J"},{"issue":"01","key":"ref128","doi-asserted-by":"crossref","first-page":"2004229","DOI":"10.1183\/13993003.04229-2020","article-title":"Screening for pulmonary arterial hypertension in adults carrying a BMPR2 mutation","volume":"58","author":"D Montani","year":"2021","journal-title":"Eur Respir J"},{"issue":"01","key":"ref129","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1093\/eurheartj\/ehv337","article-title":"Exercise training improves peak oxygen consumption and haemodynamics in patients with severe pulmonary arterial hypertension and inoperable chronic thrombo-embolic pulmonary hypertension: a prospective, randomized, controlled trial","volume":"37","author":"N Ehlken","year":"2016","journal-title":"Eur Heart J"},{"issue":"23","key":"ref130","doi-asserted-by":"crossref","first-page":"2284","DOI":"10.1093\/eurheartj\/ehaa696","article-title":"Standardized exercise training is feasible, safe, and effective in pulmonary arterial and chronic thromboembolic pulmonary hypertension: results from a large European multicentre randomized controlled trial","volume":"42","author":"E Gr\u00fcnig","year":"2021","journal-title":"Eur Heart J"},{"issue":"04","key":"ref131","first-page":"2045894020968023","article-title":"\u2018Tired, afraid, breathless\u2026.\u2009\u2019 An international survey of the exercise experience for people living with pulmonary hypertension","volume":"10","author":"K SW Chia","year":"2020","journal-title":"Pulm Circ"},{"issue":"07","key":"ref132","doi-asserted-by":"crossref","first-page":"1650","DOI":"10.1016\/S0735-1097(98)00162-4","article-title":"Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996","volume":"31","author":"B M Weiss","year":"1998","journal-title":"J Am Coll Cardiol"},{"key":"ref133","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1016\/j.ejogrb.2020.08.028","article-title":"Pulmonary hypertension and pregnancy outcomes: systematic review and meta-analysis","volume":"253","author":"N Jha","year":"2020","journal-title":"Eur J Obstet Gynecol Reprod Biol"},{"issue":"09","key":"ref134","doi-asserted-by":"crossref","first-page":"1119","DOI":"10.1002\/ejhf.594","article-title":"Pulmonary hypertension and pregnancy outcomes: data from the Registry Of Pregnancy and Cardiac Disease (ROPAC) of the European Society of Cardiology","volume":"18","author":"K Sliwa","year":"2016","journal-title":"Eur J Heart Fail"},{"issue":"05","key":"ref135","doi-asserted-by":"crossref","first-page":"1330","DOI":"10.1378\/chest.12-0528","article-title":"Management of pulmonary arterial hypertension during pregnancy: a retrospective, multicenter experience","volume":"143","author":"A G Duarte","year":"2013","journal-title":"Chest"},{"issue":"04","key":"ref136","doi-asserted-by":"crossref","first-page":"881","DOI":"10.1183\/09031936.00141211","article-title":"Pregnancy outcomes in pulmonary arterial hypertension in the modern management era","volume":"40","author":"X Ja\u00efs","year":"2012","journal-title":"Eur Respir J"},{"issue":"05","key":"ref137","doi-asserted-by":"crossref","first-page":"565","DOI":"10.1111\/j.1471-0528.2009.02492.x","article-title":"Improved survival in pregnancy and pulmonary hypertension using a multiprofessional approach","volume":"117","author":"D G Kiely","year":"2010","journal-title":"BJOG"},{"issue":"23","key":"ref138","doi-asserted-by":"crossref","first-page":"e20285","DOI":"10.1097\/MD.0000000000020285","article-title":"Pregnancy outcomes in patients with pulmonary arterial hypertension: a retrospective study","volume":"99","author":"J Luo","year":"2020","journal-title":"Medicine (Baltimore)"},{"issue":"03","key":"ref139","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1093\/eurheartj\/ehn597","article-title":"Has there been any progress made on pregnancy outcomes among women with pulmonary arterial hypertension?","volume":"30","author":"E B\u00e9dard","year":"2009","journal-title":"Eur Heart J"},{"issue":"03","key":"ref140","doi-asserted-by":"crossref","first-page":"435","DOI":"10.1086\/682230","article-title":"Statement on pregnancy in pulmonary hypertension from the Pulmonary Vascular Research Institute","volume":"5","author":"A R Hemnes","year":"2015","journal-title":"Pulm Circ"},{"issue":"02","key":"ref141","doi-asserted-by":"crossref","first-page":"391","DOI":"10.1016\/j.suc.2007.12.005","article-title":"Physiologic changes in pregnancy","volume":"88","author":"C C Hill","year":"2008","journal-title":"Surg Clin North Am"},{"issue":"04","key":"ref142","doi-asserted-by":"crossref","first-page":"101348","DOI":"10.1016\/j.siny.2022.101348","article-title":"Pulmonary hypertension in pregnancy and its effects on the fetus","volume":"27","author":"B A Muller","year":"2022","journal-title":"Semin Fetal Neonatal Med"},{"key":"ref143","doi-asserted-by":"crossref","first-page":"689764","DOI":"10.3389\/fmed.2021.689764","article-title":"Favorable pregnancy outcomes in women with well-controlled pulmonary arterial hypertension","volume":"8","author":"N Corbach","year":"2021","journal-title":"Front Med (Lausanne)"},{"issue":"06","key":"ref144","doi-asserted-by":"crossref","first-page":"1701141","DOI":"10.1183\/13993003.01141-2017","article-title":"The potential effects of pregnancy in a patient with idiopathic pulmonary arterial hypertension responding to calcium channel blockade","volume":"50","author":"S Bostock","year":"2017","journal-title":"Eur Respir J"},{"issue":"162","key":"ref145","doi-asserted-by":"crossref","first-page":"200330","DOI":"10.1183\/16000617.0330-2020","article-title":"Sex and gender in pulmonary arterial hypertension","volume":"30","author":"C Cheron","year":"2021","journal-title":"Eur Respir Rev"},{"issue":"03","key":"ref146","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1016\/j.healun.2020.12.002","article-title":"Pregnancy in pulmonary arterial hypertension: Midterm outcomes of mothers and offspring","volume":"40","author":"J C Kamp","year":"2021","journal-title":"J Heart Lung Transplant"},{"issue":"02","key":"ref147","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1159\/000453062","article-title":"Sildenafil in pregnancy: a systematic review of maternal tolerance and obstetric and perinatal outcomes","volume":"41","author":"L Dunn","year":"2017","journal-title":"Fetal Diagn Ther"},{"issue":"142","key":"ref148","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1183\/16000617.0079-2016","article-title":"Pregnancy in pulmonary arterial hypertension","volume":"25","author":"K M Olsson","year":"2016","journal-title":"Eur Respir Rev"},{"key":"ref149","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1111\/1471-0528.15681","article-title":"Outcome of pregnancies in women with pulmonary hypertension: a single-centre experience from South India","volume":"126","author":"A Keepanasseril","year":"2019","journal-title":"BJOG"}],"container-title":["Seminars in Respiratory and Critical Care Medicine"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/s-0043-1770118.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,30]],"date-time":"2023-10-30T23:41:57Z","timestamp":1698709317000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/s-0043-1770118"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,6,27]]},"references-count":148,"journal-issue":{"issue":"06","published-online":{"date-parts":[[2023,10,30]]},"published-print":{"date-parts":[[2023,12]]}},"URL":"https:\/\/doi.org\/10.1055\/s-0043-1770118","archive":["Portico","CLOCKSS"],"relation":{},"ISSN":["1069-3424","1098-9048"],"issn-type":[{"value":"1069-3424","type":"print"},{"value":"1098-9048","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,6,27]]}}}