{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T04:22:58Z","timestamp":1792815778565,"version":"4.5.0"},"reference-count":112,"publisher":"European Respiratory Society (ERS)","issue":"6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2012,4,18]]},"published-print":{"date-parts":[[2012,12]]},"abstract":"<jats:p>Pulmonary arterial hypertension (PAH) is no longer an orphan disease. There are three different classes of drugs for the treatment of PAH that are currently being used and an increasing number of patients are being treated with a single drug or combination therapy. During the last 25 yrs, new insights into the pathobiology of PAH have been gained. The classical mechanical concepts of pressure, flow, shear stress, right ventricle wall stress and impedance have been complemented with the new concepts of cell injury and repair and interactions of complex multicellular systems. Integrating these concepts will become critical as we design new medical therapies in order to change the prognosis of patients with these fatal diseases. This review intends to summarise recent pathobiological concepts of PAH and right ventricle failure mainly derived from human studies, which reflect the progress made in the understanding of this complex group of pulmonary vascular diseases.<\/jats:p>","DOI":"10.1183\/09031936.00046612","type":"journal-article","created":{"date-parts":[[2012,6,28]],"date-time":"2012-06-28T04:54:49Z","timestamp":1340859289000},"page":"1555-1565","source":"Crossref","is-referenced-by-count":254,"title":["Pathobiology of pulmonary arterial hypertension and right ventricular failure"],"prefix":"10.1183","volume":"40","author":[{"given":"Norbert F.","family":"Voelkel","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jose","family":"Gomez-Arroyo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Abbate","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harm J.","family":"Bogaard","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark R.","family":"Nicolls","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"81","published-online":{"date-parts":[[2012,6,27]]},"reference":[{"key":"2024102021115565000_40.6.1555.1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00139009"},{"key":"2024102021115565000_40.6.1555.2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.10-0075"},{"key":"2024102021115565000_40.6.1555.3","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200510-1668OC"},{"key":"2024102021115565000_40.6.1555.4","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00092306"},{"key":"2024102021115565000_40.6.1555.5","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00101211"},{"key":"2024102021115565000_40.6.1555.6","doi-asserted-by":"publisher","DOI":"10.1378\/chest.07-0903"},{"key":"2024102021115565000_40.6.1555.7","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.109.898122"},{"key":"2024102021115565000_40.6.1555.8","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.109.911818"},{"key":"2024102021115565000_40.6.1555.9","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00057010"},{"key":"2024102021115565000_40.6.1555.10","first-page":"P9","article-title":"Pathology of lung-tissue and lung vessels in valvular heart-disease","volume":"13","author":"Wagenvoort","year":"1977","journal-title":"B Eur Physiopath Res"},{"key":"2024102021115565000_40.6.1555.11","doi-asserted-by":"publisher","DOI":"10.1172\/JCI33452"},{"key":"2024102021115565000_40.6.1555.12","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.102.22.2781"},{"key":"2024102021115565000_40.6.1555.13","first-page":"275","article-title":"Exuberant endothelial cell growth and elements of inflammation are present in plexiform lesions of pulmonary hypertension","volume":"144","author":"Tuder","year":"1994","journal-title":"Am J Pathol"},{"key":"2024102021115565000_40.6.1555.14","doi-asserted-by":"publisher","DOI":"10.1038\/nrcardio.2011.87"},{"key":"2024102021115565000_40.6.1555.15","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra040291"},{"key":"2024102021115565000_40.6.1555.16","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.106.632208"},{"key":"2024102021115565000_40.6.1555.17","doi-asserted-by":"publisher","DOI":"10.1378\/chest.08-0492"},{"key":"2024102021115565000_40.6.1555.18","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200108023450502"},{"key":"2024102021115565000_40.6.1555.19","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2009.04.015"},{"key":"2024102021115565000_40.6.1555.20","doi-asserted-by":"publisher","DOI":"10.1186\/1465-9921-12-99"},{"key":"2024102021115565000_40.6.1555.21","doi-asserted-by":"publisher","DOI":"10.1186\/1465-9921-11-73"},{"key":"2024102021115565000_40.6.1555.22","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.105.601930"},{"key":"2024102021115565000_40.6.1555.23","doi-asserted-by":"publisher","DOI":"10.1086\/316947"},{"key":"2024102021115565000_40.6.1555.24","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.0000012754.72951.3D"},{"key":"2024102021115565000_40.6.1555.25","doi-asserted-by":"publisher","DOI":"10.1186\/1465-9921-10-87"},{"key":"2024102021115565000_40.6.1555.26","doi-asserted-by":"publisher","DOI":"10.1513\/pats.200605-118SF"},{"key":"2024102021115565000_40.6.1555.27","doi-asserted-by":"publisher","DOI":"10.1161\/01.RES.0000200180.01710.e6"},{"key":"2024102021115565000_40.6.1555.28","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2011.09.044"},{"key":"2024102021115565000_40.6.1555.29","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200108023450503"},{"key":"2024102021115565000_40.6.1555.30","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200908-1284OC"},{"key":"2024102021115565000_40.6.1555.31","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200712-1807OC"},{"key":"2024102021115565000_40.6.1555.32","doi-asserted-by":"publisher","DOI":"10.1002\/humu.21605"},{"key":"2024102021115565000_40.6.1555.33","doi-asserted-by":"publisher","DOI":"10.4161\/epi.1.2.2762"},{"key":"2024102021115565000_40.6.1555.34","doi-asserted-by":"publisher","DOI":"10.4103\/2045-8932.87300"},{"key":"2024102021115565000_40.6.1555.35","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCRESAHA.109.197491"},{"key":"2024102021115565000_40.6.1555.36","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCRESAHA.108.182014"},{"key":"2024102021115565000_40.6.1555.37","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00362.2011"},{"key":"2024102021115565000_40.6.1555.38","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201106-1093OC"},{"key":"2024102021115565000_40.6.1555.39","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20101812"},{"key":"2024102021115565000_40.6.1555.40","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200709-1369PP"},{"key":"2024102021115565000_40.6.1555.41","doi-asserted-by":"crossref","first-page":"517","DOI":"10.1152\/jappl.1979.46.3.517","article-title":"Vascular hypertrophy in cattle susceptible to hypoxic pulmonary hypertension","volume":"46","author":"Weir","year":"1979","journal-title":"J Appl Physiol"},{"key":"2024102021115565000_40.6.1555.42","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1161\/01.RES.48.2.207","article-title":"Release of vasodilator prostaglandin, PGI2, from isolated rat lung during vasoconstriction","volume":"48","author":"Voelkel","year":"1981","journal-title":"Circ Res"},{"key":"2024102021115565000_40.6.1555.43","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199602013340504"},{"key":"2024102021115565000_40.6.1555.44","doi-asserted-by":"publisher","DOI":"10.1172\/JCI1910"},{"key":"2024102021115565000_40.6.1555.45","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201006-0905OC"},{"key":"2024102021115565000_40.6.1555.46","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)65137-1"},{"key":"2024102021115565000_40.6.1555.47","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00094706"},{"key":"2024102021115565000_40.6.1555.48","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201105-0927OC"},{"key":"2024102021115565000_40.6.1555.49","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0605080104"},{"key":"2024102021115565000_40.6.1555.50","doi-asserted-by":"publisher","DOI":"10.1096\/fj.07-8432com"},{"key":"2024102021115565000_40.6.1555.51","doi-asserted-by":"publisher","DOI":"10.1161\/01.RES.0000073585.50092.14"},{"key":"2024102021115565000_40.6.1555.52","doi-asserted-by":"publisher","DOI":"10.1172\/JCI23203"},{"key":"2024102021115565000_40.6.1555.53","doi-asserted-by":"crossref","first-page":"3","DOI":"10.4103\/2045-8932.78095","article-title":"Progenitor cells in pulmonary vascular remodeling","volume":"1","author":"Yeager","year":"2011","journal-title":"Pulm Circ"},{"key":"2024102021115565000_40.6.1555.54","doi-asserted-by":"publisher","DOI":"10.1089\/ars.2010.3507"},{"key":"2024102021115565000_40.6.1555.55","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00314.2007"},{"key":"2024102021115565000_40.6.1555.56","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2006.12.037"},{"key":"2024102021115565000_40.6.1555.57","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1111\/j.1742-1241.2009.02232.x","article-title":"Endothelial progenitor cells in pulmonary hypertension \u2013 dawn of cell-based therapy","volume":"165","author":"Toshner","year":"2010","journal-title":"Int J Clin Pract Suppl"},{"key":"2024102021115565000_40.6.1555.58","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMc051946"},{"key":"2024102021115565000_40.6.1555.59","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200707-1037OC"},{"key":"2024102021115565000_40.6.1555.60","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201001-0123OC"},{"key":"2024102021115565000_40.6.1555.61","doi-asserted-by":"publisher","DOI":"10.1038\/nrcardio.2011.104"},{"key":"2024102021115565000_40.6.1555.62","doi-asserted-by":"publisher","DOI":"10.1038\/clpt.2009.217"},{"key":"2024102021115565000_40.6.1555.63","doi-asserted-by":"publisher","DOI":"10.1165\/rcmb.2010-0371OC"},{"key":"2024102021115565000_40.6.1555.64","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00212.2011"},{"key":"2024102021115565000_40.6.1555.65","doi-asserted-by":"publisher","DOI":"10.1186\/1471-2466-11-30"},{"key":"2024102021115565000_40.6.1555.66","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.111.079921"},{"key":"2024102021115565000_40.6.1555.67","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.05.00045705"},{"key":"2024102021115565000_40.6.1555.68","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.151.5.7735624"},{"key":"2024102021115565000_40.6.1555.69","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.109.933762"},{"key":"2024102021115565000_40.6.1555.70","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.immunol.21.120601.141122"},{"key":"2024102021115565000_40.6.1555.71","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCRESAHA.110.236927"},{"key":"2024102021115565000_40.6.1555.72","doi-asserted-by":"publisher","DOI":"10.1378\/chest.100.5.1268"},{"key":"2024102021115565000_40.6.1555.73","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0005981"},{"key":"2024102021115565000_40.6.1555.74","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.182.3.1689"},{"key":"2024102021115565000_40.6.1555.75","first-page":"131","article-title":"Peripheral blood T-lymphocyte subsets in autoimmune thyroid disease","volume":"2","author":"Covas","year":"1992","journal-title":"J Investig Allergol Clin Immunol"},{"key":"2024102021115565000_40.6.1555.76","first-page":"726","article-title":"CD4+ T-lymphocytopenia \u2013 a frequent finding in anti-SSA antibody seropositive patients with primary Sjogren's syndrome","volume":"31","author":"Mandl","year":"2004","journal-title":"J Rheumatol"},{"key":"2024102021115565000_40.6.1555.77","first-page":"2242","article-title":"T lymphocyte subsets in primary antiphospholipid syndrome","volume":"21","author":"Papo","year":"1994","journal-title":"J Rheumatol"},{"key":"2024102021115565000_40.6.1555.78","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200608-1189OC"},{"key":"2024102021115565000_40.6.1555.79","doi-asserted-by":"publisher","DOI":"10.1038\/ni1428"},{"key":"2024102021115565000_40.6.1555.80","doi-asserted-by":"publisher","DOI":"10.1177\/0961203311403347"},{"key":"2024102021115565000_40.6.1555.81","doi-asserted-by":"publisher","DOI":"10.1177\/0961203307087610"},{"key":"2024102021115565000_40.6.1555.82","doi-asserted-by":"publisher","DOI":"10.1378\/chest.130.1.182"},{"key":"2024102021115565000_40.6.1555.83","doi-asserted-by":"publisher","DOI":"10.1002\/art.23303"},{"key":"2024102021115565000_40.6.1555.84","first-page":"100","article-title":"Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome","volume":"15","author":"Hopkins","year":"1996","journal-title":"J Heart Lung Transplant"},{"key":"2024102021115565000_40.6.1555.85","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201006-0869WS"},{"key":"2024102021115565000_40.6.1555.86","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.109.883843"},{"key":"2024102021115565000_40.6.1555.87","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201108-1536PP"},{"key":"2024102021115565000_40.6.1555.88","doi-asserted-by":"publisher","DOI":"10.1097\/RLU.0b013e3182177389"},{"key":"2024102021115565000_40.6.1555.89","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2005.02.065"},{"key":"2024102021115565000_40.6.1555.90","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCHEARTFAILURE.111.962381"},{"key":"2024102021115565000_40.6.1555.91","doi-asserted-by":"crossref","first-page":"2435","DOI":"10.1161\/01.CIR.91.9.2435","article-title":"Fundamental limitations of [18F] 2-deoxy-2-fluoro-D-glucose for assessing myocardial glucose uptake","volume":"91","author":"Hariharan","year":"1995","journal-title":"Circulation"},{"key":"2024102021115565000_40.6.1555.92","first-page":"565","article-title":"The clinical role of metabolic imaging of the heart by positron emission tomography","volume":"32","author":"Schwaiger","year":"1991","journal-title":"J Nucl Med"},{"key":"2024102021115565000_40.6.1555.93","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1016\/S0021-9258(18)64941-5","article-title":"On the mechanism of action of phosphoglucose isomerase and phosphomannose isomerase","volume":"225","author":"Topper","year":"1957","journal-title":"J Biol Chem"},{"key":"2024102021115565000_40.6.1555.94","doi-asserted-by":"publisher","DOI":"10.1165\/rcmb.2010-0412OC"},{"key":"2024102021115565000_40.6.1555.95","doi-asserted-by":"publisher","DOI":"10.1007\/s00109-009-0524-6"},{"key":"2024102021115565000_40.6.1555.96","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200604-547OC"},{"key":"2024102021115565000_40.6.1555.97","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehl477"},{"key":"2024102021115565000_40.6.1555.98","doi-asserted-by":"publisher","DOI":"10.1378\/chest.10-3277"},{"key":"2024102021115565000_40.6.1555.99","doi-asserted-by":"publisher","DOI":"10.1378\/chest.10-2015"},{"key":"2024102021115565000_40.6.1555.100","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2011.06.068"},{"key":"2024102021115565000_40.6.1555.101","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2008.08.033"},{"key":"2024102021115565000_40.6.1555.102","doi-asserted-by":"publisher","DOI":"10.1183\/09059180.00006511"},{"key":"2024102021115565000_40.6.1555.103","doi-asserted-by":"publisher","DOI":"10.1183\/09059180.00006911"},{"key":"2024102021115565000_40.6.1555.104","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehr490"},{"key":"2024102021115565000_40.6.1555.105","doi-asserted-by":"publisher","DOI":"10.1378\/chest.10-1166"},{"key":"2024102021115565000_40.6.1555.106","doi-asserted-by":"publisher","DOI":"10.1016\/j.ahj.2007.02.037"},{"key":"2024102021115565000_40.6.1555.107","doi-asserted-by":"publisher","DOI":"10.1016\/j.ahj.2009.11.028"},{"key":"2024102021115565000_40.6.1555.108","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehp022"},{"key":"2024102021115565000_40.6.1555.109","doi-asserted-by":"crossref","unstructured":"G\u00f3mez A Bialostozky D Zajarias A . Right ventricular ischemia in patients with primary pulmonary hypertension. JAAC 200;38: 1137\u20131142.","DOI":"10.1016\/S0735-1097(01)01496-6"},{"key":"2024102021115565000_40.6.1555.110","doi-asserted-by":"publisher","DOI":"10.1038\/nrc2106"},{"key":"2024102021115565000_40.6.1555.111","first-page":"197","article-title":"Gene signatures developed from patient tumor explants grown in nude mice to predict tumor response to 11 cytotoxic drugs","volume":"4","author":"Fiebig","year":"2007","journal-title":"Cancer Genomics Proteomics"},{"key":"2024102021115565000_40.6.1555.112","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0030550"}],"container-title":["European Respiratory Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1183\/09031936.00046612","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,2,24]],"date-time":"2025-02-24T06:38:19Z","timestamp":1740379099000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.ersnet.org\/lookup\/doi\/10.1183\/09031936.00046612"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,6,27]]},"references-count":112,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2012,11,30]]},"published-print":{"date-parts":[[2012,12]]}},"alternative-id":["10.1183\/09031936.00046612"],"URL":"https:\/\/doi.org\/10.1183\/09031936.00046612","relation":{},"ISSN":["0903-1936","1399-3003"],"issn-type":[{"value":"0903-1936","type":"print"},{"value":"1399-3003","type":"electronic"}],"subject":[],"published":{"date-parts":[[2012,6,27]]}}}