{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T12:32:45Z","timestamp":1792067565207,"version":"4.4.0"},"reference-count":176,"publisher":"Elsevier BV","issue":"4","license":[{"start":{"date-parts":[[2013,12,1]],"date-time":"2013-12-01T00:00:00Z","timestamp":1385856000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2013,12,1]],"date-time":"2013-12-01T00:00:00Z","timestamp":1385856000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","theclinics.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Clinics in Chest Medicine"],"published-print":{"date-parts":[[2013,12]]},"DOI":"10.1016\/j.ccm.2013.08.010","type":"journal-article","created":{"date-parts":[[2013,10,18]],"date-time":"2013-10-18T20:30:59Z","timestamp":1382128259000},"page":"619-637","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":49,"title":["Epidemiology of Pulmonary Arterial Hypertension"],"prefix":"10.1016","volume":"34","author":[{"given":"Darren B.","family":"Taichman","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jess","family":"Mandel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.ccm.2013.08.010_bib1","first-page":"197","article-title":"Uber Sklerose der Lungen Arterie","volume":"48","author":"Romberg","year":"1891","journal-title":"Dtsch Arch Klin Med"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib2","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1164\/rccm.200402-197OE","article-title":"A\u00a0century of pulmonary hemodynamics","volume":"170","author":"Fishman","year":"2004","journal-title":"Am J Respir Crit Care Med"},{"issue":"6","key":"10.1016\/j.ccm.2013.08.010_bib3","doi-asserted-by":"crossref","first-page":"686","DOI":"10.1016\/0002-9343(51)90020-4","article-title":"Primary pulmonary hypertension. I. Clinical and hemodynamic study","volume":"11","author":"Dresdale","year":"1951","journal-title":"Am J Med"},{"issue":"Suppl 1","key":"10.1016\/j.ccm.2013.08.010_bib4","doi-asserted-by":"crossref","first-page":"7S","DOI":"10.1378\/chest.126.1_suppl.7S","article-title":"Diagnosis and management of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines","volume":"126","author":"Rubin","year":"2004","journal-title":"Chest"},{"key":"10.1016\/j.ccm.2013.08.010_bib5","doi-asserted-by":"crossref","first-page":"1163","DOI":"10.1161\/01.CIR.42.6.1163","article-title":"Primary pulmonary hypertension: a pathologic study of the lung vessels in 156 classically diagnosed cases","volume":"42","author":"Wagenvoort","year":"1970","journal-title":"Circulation"},{"issue":"5","key":"10.1016\/j.ccm.2013.08.010_bib6","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1016\/S0002-9629(15)40640-8","article-title":"High prevalence of hypothyroidism in patients with primary pulmonary hypertension","volume":"318","author":"Curnock","year":"1999","journal-title":"Am J Med Sci"},{"issue":"6","key":"10.1016\/j.ccm.2013.08.010_bib7","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1097\/01.crd.0000137259.83169.e3","article-title":"Reversible pulmonary hypertension, tricuspid regurgitation and right-sided heart failure associated with hyperthyroidism: case report and review of the literature","volume":"12","author":"Lozano","year":"2004","journal-title":"Cardiol Rev"},{"issue":"6","key":"10.1016\/j.ccm.2013.08.010_bib8","doi-asserted-by":"crossref","first-page":"579","DOI":"10.1056\/NEJMoa052744","article-title":"Selective serotonin-reuptake inhibitors and risk of persistent pulmonary hypertension of the newborn","volume":"354","author":"Chambers","year":"2006","journal-title":"N\u00a0Engl J Med"},{"issue":"9","key":"10.1016\/j.ccm.2013.08.010_bib9","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1056\/NEJM199608293350901","article-title":"Appetite-suppressant drugs and the risk of primary pulmonary hypertension. International Primary Pulmonary Hypertension Study Group","volume":"335","author":"Abenhaim","year":"1996","journal-title":"N\u00a0Engl J Med"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib10","doi-asserted-by":"crossref","first-page":"37S","DOI":"10.1378\/chest.105.2_Supplement.37S","article-title":"The International Primary Pulmonary Hypertension Study","volume":"105","author":"Group IPPHS","year":"1994","journal-title":"Chest"},{"issue":"6","key":"10.1016\/j.ccm.2013.08.010_bib11","doi-asserted-by":"crossref","first-page":"1801","DOI":"10.1378\/chest.119.6.1801","article-title":"Primary pulmonary hypertension In Israel: a National Survey","volume":"119","author":"Appelbaum","year":"2001","journal-title":"Chest"},{"issue":"9","key":"10.1016\/j.ccm.2013.08.010_bib12","doi-asserted-by":"crossref","first-page":"1023","DOI":"10.1164\/rccm.200510-1668OC","article-title":"Pulmonary arterial hypertension in France: results from a national registry","volume":"173","author":"Humbert","year":"2006","journal-title":"Am J Respir Crit Care Med"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib13","doi-asserted-by":"crossref","first-page":"216","DOI":"10.7326\/0003-4819-107-2-216","article-title":"Primary pulmonary hypertension. A national prospective study","volume":"107","author":"Rich","year":"1987","journal-title":"Ann Intern Med"},{"issue":"4","key":"10.1016\/j.ccm.2013.08.010_bib14","doi-asserted-by":"crossref","first-page":"33S","DOI":"10.1378\/chest.105.2_Supplement.33S","article-title":"Primary pulmonary hypertension: case series from France","volume":"105","author":"Brenot","year":"1994","journal-title":"Chest"},{"issue":"4","key":"10.1016\/j.ccm.2013.08.010_bib15","doi-asserted-by":"crossref","first-page":"1733","DOI":"10.1161\/01.CIR.89.4.1733","article-title":"Survival in\u00a0primary pulmonary hypertension. Validation of a prognostic equation","volume":"89","author":"Sandoval","year":"1994","journal-title":"Circulation"},{"issue":"1","key":"10.1016\/j.ccm.2013.08.010_bib16","doi-asserted-by":"crossref","first-page":"12","DOI":"10.2169\/internalmedicine.38.12","article-title":"Prediction of life expectancy in patients with primary pulmonary hypertension. A retrospective nationwide survey from 1980-1990","volume":"38","author":"Okada","year":"1999","journal-title":"Intern Med"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib17","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1378\/chest.06-2913","article-title":"Registry and survival study in Chinese patients with idiopathic and familial pulmonary arterial hypertension","volume":"132","author":"Jing","year":"2007","journal-title":"Chest"},{"issue":"Suppl 1","key":"10.1016\/j.ccm.2013.08.010_bib18","doi-asserted-by":"crossref","first-page":"78S","DOI":"10.1378\/chest.126.1_suppl.78S","article-title":"Prognosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines","volume":"126","author":"McLaughlin","year":"2004","journal-title":"Chest"},{"issue":"4","key":"10.1016\/j.ccm.2013.08.010_bib19","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":"Fuster","year":"1984","journal-title":"Circulation"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib20","doi-asserted-by":"crossref","first-page":"354","DOI":"10.1378\/chest.11-0676","article-title":"The REVEAL Registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension","volume":"141","author":"Benza","year":"2012","journal-title":"Chest"},{"issue":"2 Pt 1","key":"10.1016\/j.ccm.2013.08.010_bib21","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1164\/ajrccm.161.2.9906015","article-title":"Clinical correlates and prognostic significance of six-minute walk test in patients with primary pulmonary hypertension. Comparison with cardiopulmonary exercise testing","volume":"161","author":"Miyamoto","year":"2000","journal-title":"Am J Respir Crit Care Med"},{"issue":"4","key":"10.1016\/j.ccm.2013.08.010_bib22","doi-asserted-by":"crossref","first-page":"780","DOI":"10.1016\/S0735-1097(02)02012-0","article-title":"Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival","volume":"40","author":"Sitbon","year":"2002","journal-title":"J\u00a0Am Coll Cardiol"},{"issue":"5","key":"10.1016\/j.ccm.2013.08.010_bib23","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1056\/NEJM199602013340504","article-title":"A\u00a0comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group","volume":"334","author":"Barst","year":"1996","journal-title":"N\u00a0Engl J Med"},{"issue":"3","key":"10.1016\/j.ccm.2013.08.010_bib24","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1161\/01.CIR.0000022687.18568.2A","article-title":"Assessment of survival in patients with primary pulmonary hypertension: importance of cardiopulmonary exercise testing","volume":"106","author":"Wensel","year":"2002","journal-title":"Circulation"},{"issue":"7","key":"10.1016\/j.ccm.2013.08.010_bib25","doi-asserted-by":"crossref","first-page":"1214","DOI":"10.1016\/S0735-1097(02)01744-8","article-title":"Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension","volume":"39","author":"Raymond","year":"2002","journal-title":"J\u00a0Am Coll Cardiol"},{"issue":"4","key":"10.1016\/j.ccm.2013.08.010_bib26","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1016\/S0002-9149(99)00342-2","article-title":"Frequency and prognostic significance of pericardial effusion in primary pulmonary hypertension. PPH Study Group. Primary pulmonary hypertension","volume":"84","author":"Hinderliter","year":"1999","journal-title":"Am J Cardiol"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib27","doi-asserted-by":"crossref","first-page":"353","DOI":"10.1161\/01.CIR.80.2.353","article-title":"Two-dimensional and Doppler-echocardiographic and cardiac catheterization correlates of survival in primary pulmonary hypertension","volume":"80","author":"Eysmann","year":"1989","journal-title":"Circulation"},{"issue":"9","key":"10.1016\/j.ccm.2013.08.010_bib28","doi-asserted-by":"crossref","first-page":"1157","DOI":"10.1016\/S0002-9149(98)00140-4","article-title":"Value of a Doppler-derived index combining systolic and diastolic time intervals in predicting outcome in primary pulmonary hypertension","volume":"81","author":"Yeo","year":"1998","journal-title":"Am J Cardiol"},{"issue":"9","key":"10.1016\/j.ccm.2013.08.010_bib29","doi-asserted-by":"crossref","first-page":"1034","DOI":"10.1164\/rccm.200604-547OC","article-title":"Tricuspid annular displacement predicts survival in pulmonary hypertension","volume":"174","author":"Forfia","year":"2006","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.ccm.2013.08.010_bib30","doi-asserted-by":"crossref","first-page":"628","DOI":"10.1016\/j.amjcard.2010.10.027","article-title":"Clinical and prognostic relevance of echocardiographic evaluation of right ventricular geometry in patients with idiopathic pulmonary arterial hypertension","volume":"107","author":"Ghio","year":"2011","journal-title":"Am J Cardiol"},{"key":"10.1016\/j.ccm.2013.08.010_bib31","doi-asserted-by":"crossref","first-page":"1299","DOI":"10.1378\/chest.10-2015","article-title":"Right ventricular strain for prediction of survival in patients with pulmonary arterial hypertension","volume":"139","author":"Sachdev","year":"2011","journal-title":"Chest"},{"issue":"5","key":"10.1016\/j.ccm.2013.08.010_bib32","doi-asserted-by":"crossref","first-page":"343","DOI":"10.7326\/0003-4819-115-5-343","article-title":"Survival in patients with primary pulmonary hypertension. Results from a national prospective registry","volume":"115","author":"D'Alonzo","year":"1991","journal-title":"Ann Intern Med"},{"issue":"3","key":"10.1016\/j.ccm.2013.08.010_bib33","first-page":"165","article-title":"Primary pulmonary hypertension: natural history and prognostic factors","volume":"46","author":"Rajasekhar","year":"1994","journal-title":"Indian Heart J"},{"key":"10.1016\/j.ccm.2013.08.010_bib34","series-title":"Pulmonary vascular disease","first-page":"66","article-title":"Classification and prognosis of pulmonary arterial hypertension","author":"Friedman","year":"2006"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib35","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1164\/ajrccm.160.2.9812078","article-title":"Serum uric acid levels correlate with the severity and the mortality of primary pulmonary hypertension","volume":"160","author":"Nagaya","year":"1999","journal-title":"Am J Respir Crit Care Med"},{"issue":"8","key":"10.1016\/j.ccm.2013.08.010_bib36","doi-asserted-by":"crossref","first-page":"865","DOI":"10.1161\/01.CIR.102.8.865","article-title":"Plasma brain natriuretic peptide as a prognostic indicator in patients with primary pulmonary hypertension","volume":"102","author":"Nagaya","year":"2000","journal-title":"Circulation"},{"issue":"5","key":"10.1016\/j.ccm.2013.08.010_bib37","doi-asserted-by":"crossref","first-page":"1562","DOI":"10.1378\/chest.120.5.1562","article-title":"Big endothelin-1 and endothelin-1 plasma levels are correlated with the severity of primary pulmonary hypertension","volume":"120","author":"Rubens","year":"2001","journal-title":"Chest"},{"issue":"7","key":"10.1016\/j.ccm.2013.08.010_bib38","doi-asserted-by":"crossref","first-page":"1581","DOI":"10.1016\/0735-1097(95)00399-1","article-title":"Neurohormonal activation in patients with right ventricular failure from pulmonary hypertension: relation to hemodynamic variables and endothelin levels","volume":"26","author":"Nootens","year":"1995","journal-title":"J\u00a0Am Coll Cardiol"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib39","first-page":"110","article-title":"Plasma brain natriuretic peptide as a prognostic indicator in patients with primary pulmonary hypertension","volume":"37","author":"Nagaya","year":"2001","journal-title":"J\u00a0Cardiol"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib40","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1016\/j.amjcard.2004.09.006","article-title":"New predictors of outcome in idiopathic pulmonary arterial hypertension","volume":"95","author":"Kawut","year":"2005","journal-title":"Am J Cardiol"},{"issue":"3","key":"10.1016\/j.ccm.2013.08.010_bib41","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1164\/ajrccm.165.3.200109-0130c","article-title":"Outcome after cardiopulmonary resuscitation in patients with pulmonary arterial hypertension","volume":"165","author":"Hoeper","year":"2002","journal-title":"Am J Respir Crit Care Med"},{"issue":"3","key":"10.1016\/j.ccm.2013.08.010_bib42","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1378\/chest.99.3.551","article-title":"Hypoxic pulmonary vasoconstriction in systemic sclerosis and primary pulmonary hypertension","volume":"99","author":"Morgan","year":"1991","journal-title":"Chest"},{"issue":"7","key":"10.1016\/j.ccm.2013.08.010_bib43","doi-asserted-by":"crossref","first-page":"2204","DOI":"10.1016\/S0735-1097(00)00994-3","article-title":"Inhaled nitric oxide selectively dilates pulmonary vasculature in adult patients with pulmonary hypertension, irrespective of etiology","volume":"36","author":"Krasuski","year":"2000","journal-title":"J\u00a0Am Coll Cardiol"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib44","doi-asserted-by":"crossref","first-page":"334","DOI":"10.1161\/01.CIR.66.2.334","article-title":"Prostacyclin-induced acute pulmonary vasodilation in primary pulmonary hypertension","volume":"66","author":"Rubin","year":"1982","journal-title":"Circulation"},{"issue":"2 Pt 1","key":"10.1016\/j.ccm.2013.08.010_bib45","doi-asserted-by":"crossref","first-page":"384","DOI":"10.1164\/ajrccm.151.2.7842196","article-title":"Inhaled nitric oxide as a screening vasodilator agent in primary pulmonary hypertension. A dose-response study and comparison with prostacyclin","volume":"151","author":"Sitbon","year":"1995","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.ccm.2013.08.010_bib46","doi-asserted-by":"crossref","first-page":"55A","DOI":"10.1016\/S0002-9149(99)80384-1","article-title":"Role of pharmacologic tests in the treatment of primary pulmonary hypertension","volume":"75","author":"Galie","year":"1995","journal-title":"Am J Cardiol"},{"issue":"1","key":"10.1016\/j.ccm.2013.08.010_bib47","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1378\/chest.107.1.54","article-title":"Comparative acute effects of adenosine and prostacyclin in primary pulmonary hypertension","volume":"107","author":"Nootens","year":"1995","journal-title":"Chest"},{"issue":"6","key":"10.1016\/j.ccm.2013.08.010_bib48","doi-asserted-by":"crossref","first-page":"2018","DOI":"10.1161\/01.CIR.82.6.2018","article-title":"Prostacyclin and acetylcholine as screening agents for acute pulmonary vasodilator responsiveness in primary pulmonary hypertension","volume":"82","author":"Palevsky","year":"1990","journal-title":"Circulation"},{"issue":"12 Suppl S","key":"10.1016\/j.ccm.2013.08.010_bib49","doi-asserted-by":"crossref","first-page":"40S","DOI":"10.1016\/j.jacc.2004.02.032","article-title":"Diagnosis and differential assessment of pulmonary arterial hypertension","volume":"43","author":"Barst","year":"2004","journal-title":"J\u00a0Am Coll Cardiol"},{"issue":"Suppl 1","key":"10.1016\/j.ccm.2013.08.010_bib50","doi-asserted-by":"crossref","first-page":"35S","DOI":"10.1378\/chest.126.1_suppl.35S","article-title":"Medical therapy for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines","volume":"126","author":"Badesch","year":"2004","journal-title":"Chest"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib51","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":"Rich","year":"1992","journal-title":"N\u00a0Engl J Med"},{"issue":"23","key":"10.1016\/j.ccm.2013.08.010_bib52","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":"Sitbon","year":"2005","journal-title":"Circulation"},{"issue":"12","key":"10.1016\/j.ccm.2013.08.010_bib53","doi-asserted-by":"crossref","first-page":"1477","DOI":"10.1161\/01.CIR.0000029100.82385.58","article-title":"Survival in primary pulmonary hypertension: the impact of epoprostenol therapy","volume":"106","author":"McLaughlin","year":"2002","journal-title":"Circulation"},{"issue":"1","key":"10.1016\/j.ccm.2013.08.010_bib54","first-page":"194","article-title":"Familial primary pulmonary hypertension: clinical patterns","volume":"129","author":"Loyd","year":"1984","journal-title":"Am Rev Respir Dis"},{"issue":"1","key":"10.1016\/j.ccm.2013.08.010_bib55","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1164\/ajrccm.152.1.7599869","article-title":"Genetic anticipation and abnormal gender ratio at birth in familial primary pulmonary hypertension","volume":"152","author":"Loyd","year":"1995","journal-title":"Am J Respir Crit Care Med"},{"issue":"9","key":"10.1016\/j.ccm.2013.08.010_bib56","doi-asserted-by":"crossref","first-page":"892","DOI":"10.1164\/rccm.201205-0886OC","article-title":"Longitudinal analysis casts doubt on the presence of genetic anticipation in heritable pulmonary arterial hypertension","volume":"186","author":"Larkin","year":"2012","journal-title":"Am J Respir Crit Care Med"},{"issue":"3","key":"10.1016\/j.ccm.2013.08.010_bib57","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1038\/ng0397-277","article-title":"Localization of the gene for familial primary pulmonary hypertension to chromosome 2q31-32","volume":"15","author":"Nichols","year":"1997","journal-title":"Nat Genet"},{"issue":"12","key":"10.1016\/j.ccm.2013.08.010_bib58","doi-asserted-by":"crossref","first-page":"2603","DOI":"10.1161\/01.CIR.95.12.2603","article-title":"Mapping of familial primary pulmonary hypertension locus (PPH1) to chromosome 2q31-q32","volume":"95","author":"Morse","year":"1997","journal-title":"Circulation"},{"issue":"3","key":"10.1016\/j.ccm.2013.08.010_bib59","doi-asserted-by":"crossref","first-page":"737","DOI":"10.1086\/303059","article-title":"Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene","volume":"67","author":"Deng","year":"2000","journal-title":"Am J Hum Genet"},{"issue":"1","key":"10.1016\/j.ccm.2013.08.010_bib60","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1038\/79226","article-title":"Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension. The International PPH Consortium","volume":"26","author":"Lane","year":"2000","journal-title":"Nat Genet"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib61","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1002\/humu.20285","article-title":"Mutations of the TGF-beta type II receptor BMPR2 in pulmonary arterial hypertension","volume":"27","author":"Machado","year":"2006","journal-title":"Hum Mutat"},{"key":"10.1016\/j.ccm.2013.08.010_bib62","series-title":"Pulmonary vascular disease","first-page":"50","article-title":"Genetics of pulmonary arterial hypertension","author":"Elliott","year":"2006"},{"issue":"5","key":"10.1016\/j.ccm.2013.08.010_bib63","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1056\/NEJM200108023450503","article-title":"Clinical and molecular genetic features of pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia","volume":"345","author":"Trembath","year":"2001","journal-title":"N\u00a0Engl J Med"},{"issue":"5","key":"10.1016\/j.ccm.2013.08.010_bib64","doi-asserted-by":"crossref","first-page":"446","DOI":"10.1136\/thx.2003.11890","article-title":"Endoglin germline mutation in a patient with hereditary haemorrhagic telangiectasia and dexfenfluramine associated pulmonary arterial hypertension","volume":"59","author":"Chaouat","year":"2004","journal-title":"Thorax"},{"issue":"12","key":"10.1016\/j.ccm.2013.08.010_bib65","doi-asserted-by":"crossref","first-page":"865","DOI":"10.1136\/jmg.40.12.865","article-title":"Molecular and functional analysis identifies ALK-1 as the predominant cause of pulmonary hypertension related to hereditary haemorrhagic telangiectasia","volume":"40","author":"Harrison","year":"2003","journal-title":"J\u00a0Med Genet"},{"issue":"3","key":"10.1016\/j.ccm.2013.08.010_bib66","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1183\/09031936.04.00085504","article-title":"Primary pulmonary hypertension in families with hereditary haemorrhagic telangiectasia","volume":"23","author":"Abdalla","year":"2004","journal-title":"Eur Respir J"},{"issue":"5","key":"10.1016\/j.ccm.2013.08.010_bib67","doi-asserted-by":"crossref","first-page":"518","DOI":"10.1038\/ng.2581","article-title":"Genome-wide association analysis identifies a susceptibility locus for pulmonary arterial hypertension","volume":"45","author":"Germain","year":"2013","journal-title":"Nat Genet"},{"issue":"5","key":"10.1016\/j.ccm.2013.08.010_bib68","doi-asserted-by":"crossref","first-page":"590","DOI":"10.1164\/rccm.200602-165OC","article-title":"High frequency of BMPR2 exonic deletions\/duplications in familial pulmonary arterial hypertension","volume":"174","author":"Cogan","year":"2006","journal-title":"Am J Respir Crit Care Med"},{"issue":"10","key":"10.1016\/j.ccm.2013.08.010_bib69","doi-asserted-by":"crossref","first-page":"741","DOI":"10.1136\/jmg.37.10.741","article-title":"Sporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR-II, a receptor member of the TGF-beta family","volume":"37","author":"Thomson","year":"2000","journal-title":"J\u00a0Med Genet"},{"issue":"12 Suppl S","key":"10.1016\/j.ccm.2013.08.010_bib70","doi-asserted-by":"crossref","first-page":"33S","DOI":"10.1016\/j.jacc.2004.02.028","article-title":"Genetic basis of pulmonary arterial hypertension: current understanding and future directions","volume":"43","author":"Newman","year":"2004","journal-title":"J\u00a0Am Coll Cardiol"},{"issue":"3","key":"10.1016\/j.ccm.2013.08.010_bib71","doi-asserted-by":"crossref","first-page":"518","DOI":"10.1183\/09031936.02.01762002","article-title":"BMPR2 germline mutations in pulmonary hypertension associated with fenfluramine derivatives","volume":"20","author":"Humbert","year":"2002","journal-title":"Eur Respir J"},{"issue":"3","key":"10.1016\/j.ccm.2013.08.010_bib72","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1183\/09031936.04.00018604","article-title":"BMPR2 mutations in pulmonary arterial hypertension with congenital heart disease","volume":"24","author":"Roberts","year":"2004","journal-title":"Eur Respir J"},{"issue":"6","key":"10.1016\/j.ccm.2013.08.010_bib73","doi-asserted-by":"crossref","first-page":"889","DOI":"10.1164\/rccm.200208-861OC","article-title":"Pulmonary veno-occlusive disease caused by an inherited mutation in bone morphogenetic protein receptor II","volume":"167","author":"Runo","year":"2003","journal-title":"Am J Respir Crit Care Med"},{"issue":"8","key":"10.1016\/j.ccm.2013.08.010_bib74","doi-asserted-by":"crossref","first-page":"851","DOI":"10.1164\/rccm.200908-1284OC","article-title":"Clinical outcomes of pulmonary arterial hypertension in patients carrying an ACVRL1 (ALK1) mutation","volume":"181","author":"Girerd","year":"2010","journal-title":"Am J Respir Crit Care Med"},{"issue":"5","key":"10.1016\/j.ccm.2013.08.010_bib75","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1136\/jmg.2008.062703","article-title":"A\u00a0new nonsense mutation of SMAD8 associated with pulmonary arterial hypertension","volume":"46","author":"Shintani","year":"2009","journal-title":"J\u00a0Med Genet"},{"issue":"5","key":"10.1016\/j.ccm.2013.08.010_bib76","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1056\/NEJM200108023450502","article-title":"Mutation in the gene for bone morphogenetic protein receptor II as a cause of primary pulmonary hypertension in a large kindred","volume":"345","author":"Newman","year":"2001","journal-title":"N\u00a0Engl J Med"},{"issue":"4","key":"10.1016\/j.ccm.2013.08.010_bib77","doi-asserted-by":"crossref","first-page":"973","DOI":"10.1378\/chest.108.4.973","article-title":"Coancestry in apparently sporadic primary pulmonary hypertension","volume":"108","author":"Elliott","year":"1995","journal-title":"Chest"},{"issue":"10","key":"10.1016\/j.ccm.2013.08.010_bib78","doi-asserted-by":"crossref","first-page":"1145","DOI":"10.1161\/01.CIR.102.10.1145","article-title":"Abnormal pulmonary artery pressure response in asymptomatic carriers of primary pulmonary hypertension gene","volume":"102","author":"Grunig","year":"2000","journal-title":"Circulation"},{"issue":"11","key":"10.1016\/j.ccm.2013.08.010_bib79","doi-asserted-by":"crossref","first-page":"1088","DOI":"10.1136\/ard.62.11.1088","article-title":"Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach","volume":"62","author":"Mukerjee","year":"2003","journal-title":"Ann Rheum Dis"},{"issue":"11","key":"10.1016\/j.ccm.2013.08.010_bib80","doi-asserted-by":"crossref","first-page":"2290","DOI":"10.3899\/jrheum.100245","article-title":"Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and metaanalysis of 5 studies","volume":"37","author":"Avouac","year":"2010","journal-title":"J\u00a0Rheumatol"},{"issue":"4","key":"10.1016\/j.ccm.2013.08.010_bib81","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1002\/art.1780290409","article-title":"Pulmonary hypertension in the CREST syndrome variant of systemic sclerosis","volume":"29","author":"Stupi","year":"1986","journal-title":"Arthritis Rheum"},{"issue":"4","key":"10.1016\/j.ccm.2013.08.010_bib82","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1093\/rheumatology\/40.4.453","article-title":"Pulmonary hypertension in systemic sclerosis: risk factors for progression and consequences for survival","volume":"40","author":"MacGregor","year":"2001","journal-title":"Rheumatology (Oxford)"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib83","doi-asserted-by":"crossref","first-page":"344","DOI":"10.1378\/chest.123.2.344","article-title":"Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis","volume":"123","author":"Kawut","year":"2003","journal-title":"Chest"},{"issue":"11","key":"10.1016\/j.ccm.2013.08.010_bib84","doi-asserted-by":"crossref","first-page":"2437","DOI":"10.1002\/1529-0131(200011)43:11<2437::AID-ANR10>3.0.CO;2-U","article-title":"Severe organ involvement in systemic sclerosis with diffuse scleroderma","volume":"43","author":"Steen","year":"2000","journal-title":"Arthritis Rheum"},{"issue":"122","key":"10.1016\/j.ccm.2013.08.010_bib85","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1183\/09059180.00003811","article-title":"Pulmonary hypertension in systemic sclerosis and systemic lupus erythematosus","volume":"20","author":"Johnson","year":"2011","journal-title":"Eur Respir Rev"},{"issue":"12","key":"10.1016\/j.ccm.2013.08.010_bib86","doi-asserted-by":"crossref","first-page":"896","DOI":"10.1056\/NEJMoa012212","article-title":"Bosentan therapy for pulmonary arterial hypertension","volume":"346","author":"Rubin","year":"2002","journal-title":"N\u00a0Engl J Med"},{"issue":"4","key":"10.1016\/j.ccm.2013.08.010_bib87","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1007\/s002960050074","article-title":"Pulmonary hypertension in systemic lupus erythematosus","volume":"18","author":"Shen","year":"1999","journal-title":"Rheumatol Int"},{"issue":"7","key":"10.1016\/j.ccm.2013.08.010_bib88","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1177\/000331978503600705","article-title":"Cardiovascular manifestations in systemic lupus erythematosus. Prospective study of 100 patients","volume":"36","author":"Badui","year":"1985","journal-title":"Angiology"},{"issue":"9","key":"10.1016\/j.ccm.2013.08.010_bib89","first-page":"1923","article-title":"Pulmonary hypertension in systemic lupus erythematosus: clinical association and survival in 18 patients","volume":"26","author":"Li","year":"1999","journal-title":"J\u00a0Rheumatol"},{"issue":"7","key":"10.1016\/j.ccm.2013.08.010_bib90","first-page":"918","article-title":"Pulmonary hypertension in systemic lupus erythematosus","volume":"16","author":"Simonson","year":"1989","journal-title":"J\u00a0Rheumatol"},{"issue":"3","key":"10.1016\/j.ccm.2013.08.010_bib91","doi-asserted-by":"crossref","first-page":"510","DOI":"10.1016\/0002-8703(95)90278-3","article-title":"Five-year follow-up study of the prevalence and progression of pulmonary hypertension in systemic lupus erythematosus","volume":"129","author":"Winslow","year":"1995","journal-title":"Am Heart J"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib92","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1016\/j.pcad.2012.07.006","article-title":"Immune and inflammatory mechanisms in pulmonary arterial hypertension","volume":"55","author":"El Chami","year":"2012","journal-title":"Prog Cardiovasc Dis"},{"issue":"5","key":"10.1016\/j.ccm.2013.08.010_bib93","doi-asserted-by":"crossref","first-page":"1268","DOI":"10.1378\/chest.100.5.1268","article-title":"Primary pulmonary hypertension in HIV infection","volume":"100","author":"Speich","year":"1991","journal-title":"Chest"},{"key":"10.1016\/j.ccm.2013.08.010_bib94","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1164\/rccm.200704-541OC","article-title":"Prevalence of HIV-related pulmonary arterial hypertension in the current antiretroviral therapy era","volume":"177","author":"Sitbon","year":"2008","journal-title":"Am J Respir Crit Care Med"},{"issue":"1","key":"10.1016\/j.ccm.2013.08.010_bib95","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1097\/QAD.0b013e328331c65e","article-title":"HIV-associated pulmonary arterial hypertension: survival and prognostic factors in the modern therapeutic era","volume":"24","author":"Degano","year":"2010","journal-title":"AIDS"},{"issue":"6","key":"10.1016\/j.ccm.2013.08.010_bib96","doi-asserted-by":"crossref","first-page":"2722","DOI":"10.1161\/01.CIR.89.6.2722","article-title":"Pulmonary hypertension in patients with human immunodeficiency virus infection. Comparison with primary pulmonary hypertension","volume":"89","author":"Petitpretz","year":"1994","journal-title":"Circulation"},{"key":"10.1016\/j.ccm.2013.08.010_bib97","first-page":"282","article-title":"Impact of highly active antiretroviral therapy in HIV-positive patients with cardiac involvement","volume":"40","author":"Pugliese","year":"2000","journal-title":"J\u00a0Infect"},{"issue":"10","key":"10.1016\/j.ccm.2013.08.010_bib98","doi-asserted-by":"crossref","first-page":"1433","DOI":"10.1164\/rccm.200204-330OC","article-title":"Prognostic factors for survival in human immunodeficiency virus-associated pulmonary arterial hypertension","volume":"167","author":"Nunes","year":"2003","journal-title":"Am J Respir Crit Care Med"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib99","doi-asserted-by":"crossref","first-page":"448","DOI":"10.1378\/chest.11-1460","article-title":"An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry","volume":"142","author":"Benza","year":"2012","journal-title":"Chest"},{"key":"10.1016\/j.ccm.2013.08.010_bib100","series-title":"Pulmonary vascular disease","first-page":"132","article-title":"Portopulmonary hypertension","author":"Swanson","year":"2006"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib101","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1164\/rccm.201209-1583CI","article-title":"Pulmonary vascular complications of liver disease","volume":"187","author":"Fritz","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.ccm.2013.08.010_bib102","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1002\/lt.500030503","article-title":"Severe pulmonary hypertension in liver transplant candidates","volume":"3","author":"Ramsay","year":"1997","journal-title":"Liver Transpl Surg"},{"key":"10.1016\/j.ccm.2013.08.010_bib103","doi-asserted-by":"crossref","first-page":"1233","DOI":"10.1016\/S0735-1097(87)80123-7","article-title":"Coexistent pulmonary and portal hypertension: morphologic and clinical features","volume":"10","author":"Edwards","year":"1987","journal-title":"J\u00a0Am Coll Cardiol"},{"key":"10.1016\/j.ccm.2013.08.010_bib104","doi-asserted-by":"crossref","first-page":"1461","DOI":"10.1016\/S0140-6736(04)16107-2","article-title":"Portopulmonary hypertension and hepatopulmonary syndrome","volume":"363","author":"Hoeper","year":"2004","journal-title":"Lancet"},{"issue":"9","key":"10.1016\/j.ccm.2013.08.010_bib105","doi-asserted-by":"crossref","first-page":"1355","DOI":"10.1136\/gut.52.9.1355","article-title":"Portopulmonary hypertension in decompensated cirrhosis with refractory ascites","volume":"52","author":"Benjaminov","year":"2003","journal-title":"Gut"},{"issue":"6","key":"10.1016\/j.ccm.2013.08.010_bib106","doi-asserted-by":"crossref","DOI":"10.1164\/ajrccm.159.6.9804054","article-title":"Prostacyclin synthase expression is decreased in lungs from patients with severe pulmonary hypertension","volume":"159","author":"Tuder","year":"1999","journal-title":"Am J Respir Crit Care Med"},{"issue":"1","key":"10.1016\/j.ccm.2013.08.010_bib107","doi-asserted-by":"crossref","DOI":"10.1002\/hep.22275","article-title":"Clinical risk factors for portopulmonary hypertension","volume":"48","author":"Kawut","year":"2008","journal-title":"Hepatology"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib108","doi-asserted-by":"crossref","DOI":"10.1016\/0016-5085(91)90225-A","article-title":"Pulmonary hypertension complicating portal hypertension: prevalence and relation to splanchnic hemodynamics","volume":"100","author":"Hadengue","year":"1991","journal-title":"Gastroenterology"},{"issue":"9","key":"10.1016\/j.ccm.2013.08.010_bib109","doi-asserted-by":"crossref","DOI":"10.1164\/rccm.200809-1472OC","article-title":"Genetic risk factors for portopulmonary hypertension in patients with advanced liver disease","volume":"179","author":"Roberts","year":"2009","journal-title":"Am J Respir Crit Care Med"},{"issue":"4","key":"10.1016\/j.ccm.2013.08.010_bib110","doi-asserted-by":"crossref","first-page":"437","DOI":"10.1164\/arrd.1983.127.4.437","article-title":"Primary pulmonary hypertension and cirrhosis: are they related?","volume":"127","author":"McDonnell","year":"1983","journal-title":"Am Rev Respir Dis"},{"key":"10.1016\/j.ccm.2013.08.010_bib111","doi-asserted-by":"crossref","first-page":"1502","DOI":"10.1002\/hep.21431","article-title":"Portopulmonary hypertension: results from a 10-year screening algorithm","volume":"44","author":"Krowka","year":"2006","journal-title":"Hepatology"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib112","doi-asserted-by":"crossref","first-page":"376","DOI":"10.1378\/chest.09-1140","article-title":"Pulmonary arterial hypertension: baseline characteristics from the REVEAL registry","volume":"137","author":"Badesch","year":"2010","journal-title":"Chest"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib113","doi-asserted-by":"crossref","first-page":"492","DOI":"10.1016\/S0735-1097(10)80121-4","article-title":"Association between primary pulmonary hypertension and portal hypertension: analysis of its pathophysiology and clinical, laboratory and hemodynamic manifestations","volume":"17","author":"Robalino","year":"1991","journal-title":"J\u00a0Am Coll Cardiol"},{"key":"10.1016\/j.ccm.2013.08.010_bib114","doi-asserted-by":"crossref","first-page":"2445","DOI":"10.1111\/j.1600-6143.2008.02384.x","article-title":"Survival in portopulmonary hypertension: Mayo Clinic experience categorized by treatment subgroups","volume":"8","author":"Swanson","year":"2008","journal-title":"Am J Transplant"},{"issue":"9","key":"10.1016\/j.ccm.2013.08.010_bib115","doi-asserted-by":"crossref","first-page":"1107","DOI":"10.1002\/lt.20459","article-title":"Hemodynamics and survival of patients with portopulmonary hypertension","volume":"11","author":"Kawut","year":"2005","journal-title":"Liver Transpl"},{"issue":"4","key":"10.1016\/j.ccm.2013.08.010_bib116","doi-asserted-by":"crossref","DOI":"10.1378\/chest.11-0160","article-title":"Portopulmonary hypertension: a report from the US-based REVEAL Registry","volume":"141","author":"Krowka","year":"2012","journal-title":"Chest"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib117","doi-asserted-by":"crossref","first-page":"174","DOI":"10.1002\/lt.20016","article-title":"Hepatopulmonary syndrome and portopulmonary hypertension: a report of the multicenter liver transplant database","volume":"10","author":"Krowka","year":"2004","journal-title":"Liver Transpl"},{"issue":"4","key":"10.1016\/j.ccm.2013.08.010_bib118","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1053\/jlts.2000.6356","article-title":"Pulmonary hemodynamics and perioperative cardiopulmonary-related mortality in patients with portopulmonary hypertension undergoing liver transplantation","volume":"6","author":"Krowka","year":"2000","journal-title":"Liver Transpl"},{"key":"10.1016\/j.ccm.2013.08.010_bib119","doi-asserted-by":"crossref","first-page":"604","DOI":"10.1097\/00007890-199702270-00020","article-title":"Continuous intravenous infusion of epoprostenol for the treatment of portopulmonary hypertension","volume":"63","author":"Kuo","year":"1997","journal-title":"Transplantation"},{"issue":"5","key":"10.1016\/j.ccm.2013.08.010_bib120","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1002\/lt.500050515","article-title":"Progressive splenomegaly after epoprostenol therapy in portopulmonary hypertension","volume":"5","author":"Findlay","year":"1999","journal-title":"Liver Transpl Surg"},{"issue":"3","key":"10.1016\/j.ccm.2013.08.010_bib121","doi-asserted-by":"crossref","first-page":"641","DOI":"10.1002\/hep.510300307","article-title":"Improvement in pulmonary hemodynamics during intravenous epoprostenol (prostacyclin): a study of 15 patients with moderate to severe portopulmonary hypertension","volume":"30","author":"Krowka","year":"1999","journal-title":"Hepatology"},{"issue":"14","key":"10.1016\/j.ccm.2013.08.010_bib122","first-page":"637","article-title":"Successful use of continuous intravenous prostacyclin in a patient with severe portopulmonary hypertension","volume":"112","author":"Kahler","year":"2000","journal-title":"Wien Klin Wochenschr"},{"issue":"7","key":"10.1016\/j.ccm.2013.08.010_bib123","doi-asserted-by":"crossref","first-page":"945","DOI":"10.1002\/lt.20153","article-title":"Sildenafil for portopulmonary hypertension in a patient undergoing liver transplantation","volume":"10","author":"Makisalo","year":"2004","journal-title":"Liver Transpl"},{"issue":"4","key":"10.1016\/j.ccm.2013.08.010_bib124","doi-asserted-by":"crossref","first-page":"703","DOI":"10.1183\/09031936.04.00133203","article-title":"Intravenous iloprost bridging to orthotopic liver transplantation in portopulmonary hypertension","volume":"24","author":"Minder","year":"2004","journal-title":"Eur Respir J"},{"key":"10.1016\/j.ccm.2013.08.010_bib125","doi-asserted-by":"crossref","first-page":"861","DOI":"10.1183\/09031936.04.00010904","article-title":"Pulmonary-Hepatic Vascular Disorders Scientific Committee ERS Task Force","volume":"24","author":"Rodriguez-Roisin","year":"2004","journal-title":"Eur Respir J"},{"key":"10.1016\/j.ccm.2013.08.010_bib126","series-title":"The pulmonary circulation: normal and abnormal","first-page":"397","article-title":"Aminorex pulmonary hypertension","author":"Gurtner","year":"1990"},{"key":"10.1016\/j.ccm.2013.08.010_bib127","series-title":"Primary pulmonary hypertension","first-page":"131","article-title":"Risk factors for primary pulmonary hypertension","author":"Brenot","year":"1996"},{"issue":"6","key":"10.1016\/j.ccm.2013.08.010_bib128","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1136\/hrt.70.6.537","article-title":"Primary pulmonary hypertension and fenfluramine use","volume":"70","author":"Brenot","year":"1993","journal-title":"Br Heart J"},{"issue":"3","key":"10.1016\/j.ccm.2013.08.010_bib129","doi-asserted-by":"crossref","first-page":"870","DOI":"10.1378\/chest.117.3.870","article-title":"Anorexigens and pulmonary hypertension in the United States: results from the surveillance of North American pulmonary hypertension","volume":"117","author":"Rich","year":"2000","journal-title":"Chest"},{"issue":"Suppl 3","key":"10.1016\/j.ccm.2013.08.010_bib130","doi-asserted-by":"crossref","first-page":"195S","DOI":"10.1378\/chest.114.3_Supplement.195S","article-title":"Primary pulmonary hypertension associated with the use of fenfluramine derivatives","volume":"114","author":"Simonneau","year":"1998","journal-title":"Chest"},{"issue":"3","key":"10.1016\/j.ccm.2013.08.010_bib131","doi-asserted-by":"crossref","first-page":"714","DOI":"10.1378\/chest.112.3.714","article-title":"The effect of anticoagulant therapy in primary and anorectic drug-induced pulmonary hypertension","volume":"112","author":"Frank","year":"1997","journal-title":"Chest"},{"issue":"11","key":"10.1016\/j.ccm.2013.08.010_bib132","doi-asserted-by":"crossref","first-page":"1366","DOI":"10.1016\/j.amjcard.2003.08.034","article-title":"Comparison of survival in patients with pulmonary hypertension associated with fenfluramine to patients with primary pulmonary hypertension","volume":"92","author":"Rich","year":"2003","journal-title":"Am J Cardiol"},{"issue":"3","key":"10.1016\/j.ccm.2013.08.010_bib133","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1183\/09031936.00057010","article-title":"Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension","volume":"36","author":"Humbert","year":"2010","journal-title":"Eur Respir J"},{"key":"10.1016\/j.ccm.2013.08.010_bib134","series-title":"Pulmonary vascular disease","first-page":"170","article-title":"Hemolytic anemia associated pulmonary hypertension","author":"Machado","year":"2006"},{"issue":"1","key":"10.1016\/j.ccm.2013.08.010_bib135","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1001\/jama.294.1.81","article-title":"Dysregulated arginine metabolism, hemolysis-associated pulmonary hypertension, and mortality in sickle cell disease","volume":"294","author":"Morris","year":"2005","journal-title":"JAMA"},{"key":"10.1016\/j.ccm.2013.08.010_bib136","series-title":"Pulmonary circulation: diseases and their treatment","first-page":"237","article-title":"Pulmonary hypertension in sickle cell disease and thalassemia","author":"Castro","year":"2004"},{"issue":"10","key":"10.1016\/j.ccm.2013.08.010_bib137","doi-asserted-by":"crossref","first-page":"1037","DOI":"10.1053\/hupa.2002.128059","article-title":"Pulmonary hypertension in sickle cell hemoglobinopathy: a clinicopathologic study of 20 cases","volume":"33","author":"Haque","year":"2002","journal-title":"Hum Pathol"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib138","first-page":"269","article-title":"Cardiac involvement in beta-thalassemia\/hemoglobin E disease: clinical and hemodynamic findings","volume":"21","author":"Jootar","year":"1990","journal-title":"Southeast Asian J Trop Med Public Health"},{"issue":"3","key":"10.1016\/j.ccm.2013.08.010_bib139","doi-asserted-by":"crossref","first-page":"532","DOI":"10.1016\/S0002-8703(97)70091-7","article-title":"Pulmonary hypertension in patients with thalassemia major","volume":"134","author":"Du","year":"1997","journal-title":"Am Heart J"},{"issue":"5","key":"10.1016\/j.ccm.2013.08.010_bib140","doi-asserted-by":"crossref","first-page":"1523","DOI":"10.1378\/chest.127.5.1523","article-title":"Thalassemia heart disease: a comparative evaluation of thalassemia major and thalassemia intermedia","volume":"127","author":"Aessopos","year":"2005","journal-title":"Chest"},{"key":"10.1016\/j.ccm.2013.08.010_bib141","doi-asserted-by":"crossref","first-page":"342","DOI":"10.1196\/annals.1345.041","article-title":"Pulmonary hypertension in \u03b2-thalassemia","volume":"1054","author":"Aessopos","year":"2005","journal-title":"Ann N Y Acad Sci"},{"issue":"5","key":"10.1016\/j.ccm.2013.08.010_bib142","doi-asserted-by":"crossref","first-page":"629","DOI":"10.1183\/09031936.93.04050629","article-title":"Spherocytosis and pulmonary hypertension coincidental occurrence or causal relationship?","volume":"4","author":"Verresen","year":"1991","journal-title":"Eur Respir J"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib143","doi-asserted-by":"crossref","first-page":"642","DOI":"10.1378\/chest.102.2.642","article-title":"Pulmonary hypertension in paroxysmal nocturnal hemoglobinuria","volume":"102","author":"Heller","year":"1992","journal-title":"Chest"},{"issue":"4","key":"10.1016\/j.ccm.2013.08.010_bib144","doi-asserted-by":"crossref","first-page":"1257","DOI":"10.1182\/blood-2002-03-0948","article-title":"Pulmonary hypertension in sickle cell disease: cardiac catheterization results and survival","volume":"101","author":"Castro","year":"2003","journal-title":"Blood"},{"issue":"6","key":"10.1016\/j.ccm.2013.08.010_bib145","doi-asserted-by":"crossref","first-page":"300","DOI":"10.1097\/SMJ.0b013e318256b55b","article-title":"Misclassification of pulmonary hypertension in adults with sickle hemoglobinopathies using Doppler echocardiography","volume":"105","author":"Fitzgerald","year":"2012","journal-title":"South Med J"},{"issue":"1","key":"10.1016\/j.ccm.2013.08.010_bib146","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1183\/09031936.00119811","article-title":"Pulmonary hypertension in patients with sickle cell disease: not so frequent but so different","volume":"39","author":"Simonneau","year":"2012","journal-title":"Eur Respir J"},{"issue":"1","key":"10.1016\/j.ccm.2013.08.010_bib147","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1056\/NEJMoa1005565","article-title":"A\u00a0hemodynamic study of pulmonary hypertension in sickle cell disease","volume":"365","author":"Parent","year":"2011","journal-title":"N\u00a0Engl J Med"},{"issue":"1","key":"10.1016\/j.ccm.2013.08.010_bib148","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1183\/09031936.00134410","article-title":"Pulmonary hypertension diagnosed by right heart catheterisation in sickle cell disease","volume":"39","author":"Fonseca","year":"2012","journal-title":"Eur Respir J"},{"issue":"1","key":"10.1016\/j.ccm.2013.08.010_bib149","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1097\/00005792-198801000-00005","article-title":"Sickle cell chronic lung disease: prior morbidity and the risk of pulmonary failure","volume":"67","author":"Powars","year":"1988","journal-title":"Medicine (Baltimore)"},{"issue":"6","key":"10.1016\/j.ccm.2013.08.010_bib150","doi-asserted-by":"crossref","first-page":"626","DOI":"10.1016\/0002-9149(94)90760-9","article-title":"Pulmonary hypertension in sickle cell disease","volume":"74","author":"Sutton","year":"1994","journal-title":"Am J Cardiol"},{"issue":"9","key":"10.1016\/j.ccm.2013.08.010_bib151","doi-asserted-by":"crossref","first-page":"886","DOI":"10.1056\/NEJMoa035477","article-title":"Pulmonary hypertension as a risk factor for death in patients with sickle cell disease","volume":"350","author":"Gladwin","year":"2004","journal-title":"N\u00a0Engl J Med"},{"issue":"4","key":"10.1016\/j.ccm.2013.08.010_bib152","doi-asserted-by":"crossref","first-page":"855","DOI":"10.1182\/blood-2010-09-306167","article-title":"Hospitalization for pain in patients with sickle cell disease treated with sildenafil for elevated TRV and low exercise capacity","volume":"118","author":"Machado","year":"2011","journal-title":"Blood"},{"key":"10.1016\/j.ccm.2013.08.010_bib153","series-title":"Pulmonary vascular disease","article-title":"Pulmonary veno-occlusive disease","author":"Mandel","year":"2006"},{"issue":"5","key":"10.1016\/j.ccm.2013.08.010_bib154","doi-asserted-by":"crossref","first-page":"1964","DOI":"10.1164\/ajrccm.162.5.9912045","article-title":"Pulmonary veno-occlusive disease","volume":"162","author":"Mandel","year":"2000","journal-title":"Am J Respir Crit Care Med"},{"issue":"12 Suppl S","key":"10.1016\/j.ccm.2013.08.010_bib155","doi-asserted-by":"crossref","first-page":"5S","DOI":"10.1016\/j.jacc.2004.02.037","article-title":"Clinical classification of pulmonary hypertension","volume":"43","author":"Simonneau","year":"2004","journal-title":"J\u00a0Am Coll Cardiol"},{"issue":"6","key":"10.1016\/j.ccm.2013.08.010_bib156","first-page":"1671","article-title":"Pulmonary veno-occlusive disease: a case series and new observations","volume":"118","author":"Holcomb","year":"2000","journal-title":"Chest"},{"issue":"4","key":"10.1016\/j.ccm.2013.08.010_bib157","doi-asserted-by":"crossref","first-page":"937","DOI":"10.2214\/ajr.167.4.8819387","article-title":"Pulmonary venoocclusive disease: CT findings in eight patients","volume":"167","author":"Swensen","year":"1996","journal-title":"AJR Am J Roentgenol"},{"key":"10.1016\/j.ccm.2013.08.010_bib158","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1378\/chest.113.1.237","article-title":"Massive pulmonary edema and death after prostacyclin infusion in a patient with pulmonary veno-occlusive disease","volume":"113","author":"Palmer","year":"1998","journal-title":"Chest"},{"issue":"3","key":"10.1016\/j.ccm.2013.08.010_bib159","doi-asserted-by":"crossref","first-page":"1096","DOI":"10.1378\/chest.122.3.1096","article-title":"Effects of continuous IV prostacyclin in a patient with pulmonary veno-occlusive disease","volume":"122","author":"Okumura","year":"2002","journal-title":"Chest"},{"issue":"5","key":"10.1016\/j.ccm.2013.08.010_bib160","doi-asserted-by":"crossref","first-page":"635","DOI":"10.1016\/j.healun.2004.02.008","article-title":"Recurrence of pulmonary veno-occlusive disease after heart-lung transplantation","volume":"24","author":"Izbicki","year":"2005","journal-title":"J\u00a0Heart Lung Transplant"},{"issue":"173","key":"10.1016\/j.ccm.2013.08.010_bib161","first-page":"133","article-title":"Pulmonary veno-occlusive disease","volume":"44","author":"Thadani","year":"1975","journal-title":"Q\u00a0J Med"},{"issue":"1","key":"10.1016\/j.ccm.2013.08.010_bib162","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1378\/chest.69.1.82","article-title":"Pulmonary veno-occlusive disease. Entity or syndrome?","volume":"69","author":"Wagenvoort","year":"1976","journal-title":"Chest"},{"issue":"4","key":"10.1016\/j.ccm.2013.08.010_bib163","doi-asserted-by":"crossref","DOI":"10.1097\/MD.0b013e31818193bb","article-title":"Pulmonary veno-occlusive disease: clinical, functional, radiologic, and hemodynamic characteristics and outcome of 24 cases confirmed by histology","volume":"87","author":"Montani","year":"2008","journal-title":"Medicine (Baltimore)"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib164","doi-asserted-by":"crossref","DOI":"10.1164\/rccm.201208-1364OC","article-title":"Cytotoxic cells and granulysin in pulmonary arterial hypertension and pulmonary veno-occlusive disease","volume":"187","author":"Perros","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"issue":"1","key":"10.1016\/j.ccm.2013.08.010_bib165","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1016\/S0025-6196(12)65277-X","article-title":"Primary pulmonary hypertension: a histopathologic study of 80 cases","volume":"60","author":"Bjornsson","year":"1985","journal-title":"Mayo Clin Proc"},{"issue":"10","key":"10.1016\/j.ccm.2013.08.010_bib166","doi-asserted-by":"crossref","first-page":"911","DOI":"10.1016\/S0046-8177(82)80051-8","article-title":"Pulmonary veno-occlusive disease in siblings: case reports and morphometric study","volume":"13","author":"Davies","year":"1982","journal-title":"Hum Pathol"},{"issue":"8","key":"10.1016\/j.ccm.2013.08.010_bib167","doi-asserted-by":"crossref","first-page":"1089","DOI":"10.1016\/S0954-6111(98)90363-8","article-title":"Pulmonary veno-occlusive disease complicating Felty's syndrome","volume":"92","author":"Devereux","year":"1998","journal-title":"Respir Med"},{"issue":"6","key":"10.1016\/j.ccm.2013.08.010_bib168","first-page":"472","article-title":"Pulmonary veno-occlusive disease in a patient with HIV infection. A case report with autopsy findings","volume":"22","author":"Hourseau","year":"2002","journal-title":"Ann Pathol"},{"issue":"1","key":"10.1016\/j.ccm.2013.08.010_bib169","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1016\/S0046-8177(83)80052-5","article-title":"Pulmonary veno-occlusive disease after chemotherapy","volume":"14","author":"Joselson","year":"1983","journal-title":"Hum Pathol"},{"issue":"11","key":"10.1016\/j.ccm.2013.08.010_bib170","doi-asserted-by":"crossref","first-page":"874","DOI":"10.1136\/thx.40.11.874","article-title":"Pulmonary veno-occlusive disease after chemotherapy","volume":"40","author":"Knight","year":"1985","journal-title":"Thorax"},{"issue":"6","key":"10.1016\/j.ccm.2013.08.010_bib171","doi-asserted-by":"crossref","first-page":"1643","DOI":"10.1016\/0002-8703(92)90092-A","article-title":"Fatal pulmonary venoocclusive disease after use of oral contraceptives","volume":"124","author":"Townend","year":"1992","journal-title":"Am Heart J"},{"issue":"6","key":"10.1016\/j.ccm.2013.08.010_bib172","doi-asserted-by":"crossref","first-page":"763","DOI":"10.1136\/thx.32.6.763","article-title":"Familial pulmonary veno-occlusive disease: a case report","volume":"32","author":"Voordes","year":"1977","journal-title":"Thorax"},{"issue":"5","key":"10.1016\/j.ccm.2013.08.010_bib173","doi-asserted-by":"crossref","first-page":"1388","DOI":"10.1378\/chest.109.5.1388","article-title":"Pulmonary veno-occlusive disease in an adult following bone marrow transplantation. Case report and review of the literature","volume":"109","author":"Williams","year":"1996","journal-title":"Chest"},{"issue":"2","key":"10.1016\/j.ccm.2013.08.010_bib174","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1183\/09031936.00106008","article-title":"Pulmonary arterial hypertension in limited cutaneous systemic sclerosis: a distinctive vasculopathy","volume":"34","author":"Overbeek","year":"2009","journal-title":"Eur Respir J"},{"issue":"17","key":"10.1016\/j.ccm.2013.08.010_bib175","doi-asserted-by":"crossref","first-page":"2128","DOI":"10.1161\/CIRCULATIONAHA.111.079921","article-title":"Pulmonary arterial hypertension in patients treated by dasatinib","volume":"125","author":"Montani","year":"2012","journal-title":"Circulation"},{"issue":"6","key":"10.1016\/j.ccm.2013.08.010_bib176","doi-asserted-by":"crossref","first-page":"1785","DOI":"10.1007\/s10620-010-1220-7","article-title":"Irreversible pulmonary hypertension associated with the use of interferon alpha for chronic hepatitis C","volume":"55","author":"Dhillon","year":"2010","journal-title":"Dig Dis Sci"}],"container-title":["Clinics in Chest Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S027252311300110X?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S027252311300110X?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,3]],"date-time":"2025-10-03T00:50:18Z","timestamp":1759452618000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S027252311300110X"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,12]]},"references-count":176,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2013,12]]}},"alternative-id":["S027252311300110X"],"URL":"https:\/\/doi.org\/10.1016\/j.ccm.2013.08.010","relation":{},"ISSN":["0272-5231"],"issn-type":[{"value":"0272-5231","type":"print"}],"subject":[],"published":{"date-parts":[[2013,12]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Epidemiology of Pulmonary Arterial Hypertension","name":"articletitle","label":"Article Title"},{"value":"Clinics in Chest Medicine","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ccm.2013.08.010","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2013 Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}