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Strong scientific evidence supports the use of intravenous epoprostenol in severe PAH; however, despite recommendations, many patients do not receive parenteral prostanoids and there is a lack of evidence from randomised clinical trials supporting the value of other PAH medications alone in severe PAH.<\/jats:p><jats:p>Lung transplantation is an important option in patients with severe PAH who have not responded sufficiently to therapy, or who have worsened despite maximal treatment. Bridging techniques are available for patients who worsen while awaiting transplantation. The type of bridging technique used depends on various factors including patient illness severity, physician experience and the anticipated waiting time for transplantation. With the aim to facilitate the treatment decision-making process, herein we review the medical treatment options available for patients with severe PAH, and the bridging techniques that may be used to sustain patients awaiting transplantation.<\/jats:p>","DOI":"10.1183\/09059180.00007214","type":"journal-article","created":{"date-parts":[[2014,12,1]],"date-time":"2014-12-01T10:40:23Z","timestamp":1417430423000},"page":"488-497","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":30,"title":["Severe pulmonary arterial hypertension: treatment options and the bridge to transplantation"],"prefix":"10.1183","volume":"23","author":[{"given":"Paul","family":"Corris","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bruno","family":"Degano","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"81","published-online":{"date-parts":[[2014,12,1]]},"reference":[{"key":"2024102403130597000_23.134.488.1","doi-asserted-by":"publisher","DOI":"10.1183\/09059180.00001713"},{"key":"2024102403130597000_23.134.488.2","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200510-1668OC"},{"key":"2024102403130597000_23.134.488.3","doi-asserted-by":"publisher","DOI":"10.1016\/j.ahj.2011.09.015"},{"key":"2024102403130597000_23.134.488.4","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.109.911818"},{"key":"2024102403130597000_23.134.488.5","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00196611"},{"key":"2024102403130597000_23.134.488.6","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra040291"},{"key":"2024102403130597000_23.134.488.7","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2013.10.031"},{"key":"2024102403130597000_23.134.488.8","first-page":"107","article-title":"Lung transplantation: a treatment option in end-stage lung disease","volume":"111","author":"Hartert","year":"2014","journal-title":"Dtsch Arztebl Int"},{"key":"2024102403130597000_23.134.488.9","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2006.03.011"},{"key":"2024102403130597000_23.134.488.10","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2013.10.023"},{"key":"2024102403130597000_23.134.488.11","doi-asserted-by":"publisher","DOI":"10.1378\/chest.12-2417"},{"key":"2024102403130597000_23.134.488.12","doi-asserted-by":"publisher","DOI":"10.1016\/S0735-1097(02)02012-0"},{"key":"2024102403130597000_23.134.488.13","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199602013340504"},{"key":"2024102403130597000_23.134.488.14","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.107.742510"},{"key":"2024102403130597000_23.134.488.15","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa012212"},{"key":"2024102403130597000_23.134.488.16","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1213917"},{"key":"2024102403130597000_23.134.488.17","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1209655"},{"key":"2024102403130597000_23.134.488.18","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa050010"},{"key":"2024102403130597000_23.134.488.19","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.165.6.2106079"},{"key":"2024102403130597000_23.134.488.20","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2010.01.027"},{"key":"2024102403130597000_23.134.488.21","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.108.839274"},{"key":"2024102403130597000_23.134.488.22","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2009.09.005"},{"key":"2024102403130597000_23.134.488.23","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa020204"},{"key":"2024102403130597000_23.134.488.24","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.05.00075305"},{"key":"2024102403130597000_23.134.488.25","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.112.000765"},{"key":"2024102403130597000_23.134.488.26","doi-asserted-by":"publisher","DOI":"10.1183\/09059180.00007314"},{"key":"2024102403130597000_23.134.488.27","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00116313"},{"key":"2024102403130597000_23.134.488.28","doi-asserted-by":"publisher","DOI":"10.1183\/09059180.00009214"},{"key":"2024102403130597000_23.134.488.29","doi-asserted-by":"publisher","DOI":"10.1378\/chest.11-3060"},{"key":"2024102403130597000_23.134.488.30","doi-asserted-by":"crossref","first-page":"1114","DOI":"10.1016\/j.healun.2013.08.010","article-title":"Treatment of patients with pulmonary arterial hypertension at the time of death or deterioration to functional class IV: insights from the REVEAL Registry","volume":"31","author":"Farber","year":"2013","journal-title":"J Heart Lung Transplant"},{"key":"2024102403130597000_23.134.488.31","unstructured":"emc. 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