Pulmonary Arterial Hypertension References

Last updated: 17 March 2026
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  1. Doyle RL, McCrory D, Channick RN, et al. Surgical treatments/interventions for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004 Jul;126(1)(Suppl 1):63S-71S. doi: 10.1378/chest.126.1_suppl.63S. PMID: 15249495
  2. Badesch DB, Abman SH, Ahearn GS, et al. Medical therapy for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004 Jul;126(1)(Suppl 1):35S-62S. doi: 10.1378/chest.126.1_suppl.35S. PMID: 15249494
  3. McGoon M, Gutterman D, Steen V, et al. Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004 Jul;126(1)(Suppl Suppl):14S-34S. doi: 10.1378/chest.126.1_suppl.14S. PMID: 15249493
  4. Galiè N, Seeger W, Naeije R, et al. Comparative analysis of clinical trials and evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol. 2004 Jun;43(12)(Suppl S):81S-88S. PMID: 15194183
  5. Nauser TD, Stites SW. Diagnosis and treatment of pulmonary hypertension. Am Fam Physician. 2001 May;63(9):1789-1798. http://www.aafp.org/. PMID: 11352291
  6. McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol. 2009 Apr;53(17):1573-1619. PMID: 19389575
  7. Galiè N, Hoeper MM, Humbert M, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: The 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 the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J. 2009 Oct;30(20):2493-2537. doi: 10.1093/eurheartj/ehp297. PMID: 19713419
  8. Simonneau G, Galiè N, Rubin LJ, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004 Jun;43(Suppl 12):5S-12S. doi: 10.1016/j.jacc.2004.02.037. PMID: 15194173
  9. Galiè N, Torbicki A, Barst R, et al. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology. Eur Heart J. 2004 Dec;25(24):2243-2278. doi: 10.1016/j.ehj.2004.09.014. PMID: 15589643
  10. Channick RN, Rubin LJ. Pulmonary vasculitis and pulmonary hypertension. In: Mason RJ, Murray JF, Broaddus VC, et al, eds. Murray and Nadel’s Textbook of Respiratory Medicine. 4th. Philadelphia: Elsevier Saunders; 2005:1465-1474.
  11. MedWormhttp://www.medworm.com/rss/index.php/Cardiology/7/http://www.medworm.com/rss/medicalfeeds/specialities/Cardiology.xml
  12. MedWorm http://www.medworm.com/rss/index.php/Pediatrics/33/ http://www.medworm.com/rss/medicalfeeds/specialities/Pediatrics-News.xml
  13. MedWorm http://www.medworm.com/rss/index.php/Respiratory-Medicine/40/ http://www.medworm.com/rss/medicalfeeds/specialities/Respiratory-Medicine-News.xml
  14. Medworm - Pulmonary Hypertensionhttp://www.medworm.com/rss/medicalfeeds/conditions/Pulmonary-Hypertension.xml
  15. McCrory DC, Coeytaux RR, Schmit KM, et al. Pulmonary arterial hypertension: screening, management, and treatment. Comparative effectiveness review no. 117. AHRQ publication no. 13-EHC087-EF. Rockville, MD: Agency for Healthcare Research and Quality; 2013 Apr. http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0056718/pdf/TOC.pdf.
  16. Hopkins W, Rubin LJ. Treatment of pulmonary hypertension in adults. UpToDate. http://www.uptodate.com/. Feb 2019.
  17. Galiè N, Corris PA, Frost A, et al. Updated treatment algorithm of pulmonary arterial hypertension. J Am Coll Cardiol. 2013 Dec;62(Suppl 25):D60-D72. doi: 10.1016/j.jacc.2013.10.031. PMID: 24355643
  18. Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013 Dec;62(Suppl 25):D34-D41. http://content.onlinejacc.org/data/Journals/JAC/929484/10029.pdf. PMID: 24355639
  19. U.S. Food & Drug Administration (US FDA). Press announcements: FDA approves Opsumit to treat pulmonary arterial hypertension. US FDA. http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm371362.htm. Oct 2013. Accessed 06 Nov 2014.
  20. Patel T, McKeage K. Macitentan: first global approval. Drugs. 2014 Jan;74(1):127-133. doi: 10.1007/s40265-013-0156-6. Accessed 06 Nov 2014. PMID: 24297706
  21. Galiè N, Humbert M, Vachiery JL, et al; Authors/Task Force Members. 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). Eur Heart J. 2016 Jan;37(1):67-119. doi: 10.1093/eurheartj/ehv317. Accessed 26 Nov 2015. PMID: 26320113
  22. Kamangar N. Secondary pulmonary hypertension treatment & management. eMedicine. http://emedicine.medscape.com/article/303098-treatment. Oct 2015. Accessed 03 Dec 2015.
  23. McLaughlin VV, Channick R, Chin K, et al. Effect of selexipag on morbidity/mortality in pulmonary arterial hypertension: results of GRIPHON study. J Am Coll Cardiol. 2015 Dec;373(26):2522-2533. doi:10.1016/S0735-1097(15)61538-8. Accessed 26 Nov 2015. PMID: 26699168
  24. Taichman DB, Ornelas J, Chung L, et al. Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report. Chest. 2014 Aug;146(2):449-475. doi: 10.1378/chest.14-0793. Accessed 01 Jun 2015. PMID: 24937180
  25. Frost A, Badesch D, Giibs JSR, et al. Diagnosis of pulmonary hypertension. Eur Respir J. 2019 Jan;53(1). doi: 10.1183/13993003.01904-2018. Accessed 06 Mar 2019. PMID: 30545972
  26. Galiè N, Channick RN, Frantz RP, et al. Risk stratification and medical therapy of pulmonary arterial hypertension. Eur Respir J. 2019 Jan;53(1). doi: 10.1183/13993003.01889-2018. Accessed 06 Mar 2019. PMID: 30545971
  27. Galiè N, McLaughlin VV, Rubin LJ, et al. An overview of the 6th World Symposium on Pulmonary Hypertension. Eur Respir J. 2019 Jan;53(1). doi: 10.1183/13993003.02148-2018. Accessed 06 Mar 2019. PMID: 30552088
  28. Hoeper MM, Benza RL, Corris P, et al. Intensive care, right ventricular support and lung transplantation in patients with pulmonary hypertension. Eur Respir J. 2019 Jan;53(1). doi: 10.1183/13993003.01906-2018. Accessed 06 Mar 2019. PMID: 30545979
  29. Humbert M, Guignabert C, Bonnet S, et al. Pathology and pathobiology of pulmonary hypertension: state of the art and research perspectives. Eur Respir J. 2019 Jan;53(1). doi: 10.1183/13993003.01887-2018. Accessed 06 Mar 2019. PMID: 30545970
  30. Klinger JR, Elliott CG, Levine DJ, et al. Therapy for pulmonary arterial hypertension in adults: update of the CHEST Guideline and Expert Panel Report. Chest. 2019 Mar;155(3):565-586. doi: 10.1016/j.chest.2018.11.030. Accessed 06 Mar 2019. PMID: 30660783
  31. Rubin LJ, Hopkins W. Classification and prognosis of pulmonary hypertension in adults. UptoDate. https://www.uptodate.com. Feb 2019. Accessed 06 Mar 2019.
  32. Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019 Jan;53(1). doi: 10.1183/13993003.01913-2018. Accessed 06 Mar 2019. PMID: 30545968
  33. Condon DF, Nickel NP, Anderson R, et al. The 6th world symposium on pulmonary hypertension: what’s old is new. F1000Res. 2019 Jun;8:F1000 Faculty Rev-888. doi: 10.12688/f1000research.18811.1. Accessed 19 Oct 2020. PMID: 31249672.
  34. Hirani N, Brunner NW, Kapasi A, et al. Canadian Cardiovascular Society/Canadian Thoracic Society position statement on pulmonary hypertension. Can J Cardiol. 2020 Jul;36(7):977-992. doi: 10.1016/j.cjca.2019.11.041. Accessed 19 Oct 2020. PMID: 32682511
  35. Hopkins W, Rubin LJ. Treatment and prognosis of pulmonary arterial hypertension in adults (group 1). UpToDate. https://www.uptodate.com. 24 Jan 2020. Accessed 19 Oct 2020.
  36. Hopkins W, Rubin LJ. Treatment of pulmonary arterial hypertension (group 1) in adults: pulmonary hypertension-specific therapy. UpToDate. https://www.uptodate.com. 20 Aug 2020. Accessed 19 Oct 2020.
  37. Rubin LJ, Hopkins W. The epidemiology and pathogenesis of pulmonary arterial hypertension. UpToDate. https://www.uptodate.com. 29 Feb 2020. Accessed 19 Oct 2020.
  38. Sitbon O, Cottin V, Canuet M, et al. Initial combination therapy of macitentan and tadalafil in pulmonary arterial hypertension. Eur Respir J. 2020 Sep;56(3):2000673. doi: 10.1183/13993003.00673-2020. Accessed 22 Oct 2020. PMID: 32350101
  39. Thomas CA, Anderson RJ, Condon DF, et al. Diagnosis and management of pulmonary hypertension in the modern era: insights from the 6th world symposium. Pulm Ther. 2020 Jun;6(1):9-22. doi: 10.1007/s41030-019-00105-5. PMID: 32048239
  40. Eichstaedt CA, Belge C, Chung WK, et al. Genetic counselling and testing in pulmonary arterial hypertension: a consensus statement on behalf of the International Consortium for Genetic Studies in PAH. Eur Respir J. 2023 Feb;61(2):2201471. doi: 10.1183/13993003.01471-2022. PMID: 36302552
  41. Hopkins W, Rubin LJ. Treatment of pulmonary arterial hypertension (group 1) in adults: pulmonary hypertension-specific therapy. UpToDate. https://www.uptodate.com. 03 Oct 2023. Accessed 06 Jan 2024.
  42. Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022 Oct;43(38):3618-3731. doi: 10.1093/eurheartj/ehac237. PMID: 36017548
  43. Maron BA. Revised definition of pulmonary hypertension and approach to management: a clinical primer. J Am Heart Assoc. 2023 Apr;12(8):e029024. doi: 10.1161/JAHA.122.029024. PMID: 37026538
  44. Torbic H, Tonelli AR. Sotatercept for pulmonary arterial hypertension in the inpatient setting. J Cardiovasc Pharmacol Ther. 2024 Jan-Dec;29:10742484231225310. doi: 10.1177/10742484231225310. PMID: 38361351
  45. GBD 2021 Stroke Risk Factor Collaborators. Global, regional, and national burden of stroke and its risk factors, 1990-2021: A systematic analysis for the Global Burden of Disease Study 2021. Lancet Neurol. 2024;23(10):973-1003. doi: 10.1016/S1474-4422(24)00369-7. PMID: 39304265
  46. Huang S, Qiu J, Wang A, et al. Burden of pulmonary arterial hypertension in Asia from 1990 to 2021: Findings from Global Burden of Disease Study 2021. Chin Med J (Engl). 2025 Jun;138(11). doi: 10.1097/CM9.0000000000003559. PMID: 40375470
  47. Hopkins W, Rubin LJ. Treatment and prognosis of pulmonary arterial hypertension in adults (group 1). UpToDate. https://www.uptodate.com. 18 Dec 2025.
  48. Hopkins W, Rubin LJ. Treatment of pulmonary arterial hypertension (group 1) in adults: pulmonary hypertension-specific therapy. UpToDate. https://www.uptodate.com. 25 Nov 2025.
  49. Kovacs G, Bartolome S, Denton CP, et al. Definition, classification and diagnosis of pulmonary hypertension. Eur Respir J. 2024 Oct;64(4):2401324. doi: 10.1183/13993003.01324-2024. Accessed 24 Feb 2026. PMID: 39209475
  50. Rubin LJ, Hopkins W. The epidemiology and pathogenesis of pulmonary arterial hypertension (group 1). UpToDate. https://www.uptodate.com. 03 Feb 2026.