Pulmonary Arterial Hypertension References

Last updated: 17 March 2026
Reviewed by
  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. 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
  8. 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.
  9. Medworm - Pulmonary Hypertensionhttp://www.medworm.com/rss/medicalfeeds/conditions/Pulmonary-Hypertension.xml
  10. 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.
  11. 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
  12. 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
  13. 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.
  14. 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
  15. 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
  16. Kamangar N. Secondary pulmonary hypertension treatment & management. eMedicine. http://emedicine.medscape.com/article/303098-treatment. Oct 2015. Accessed 03 Dec 2015.
  17. 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
  18. 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
  19. 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
  20. 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
  21. 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
  22. 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
  23. 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
  24. 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
  25. Rubin LJ, Hopkins W. Classification and prognosis of pulmonary hypertension in adults. UptoDate. https://www.uptodate.com. Feb 2019. Accessed 06 Mar 2019.
  26. 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
  27. 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.
  28. 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
  29. 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
  30. 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
  31. 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
  32. 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
  33. 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
  34. 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
  35. 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
  36. 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
  37. Hopkins W, Rubin LJ. Treatment and prognosis of pulmonary arterial hypertension in adults (group 1). UpToDate. https://www.uptodate.com. 18 Dec 2025.
  38. 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.
  39. 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
  40. Rubin LJ, Hopkins W. The epidemiology and pathogenesis of pulmonary arterial hypertension (group 1). UpToDate. https://www.uptodate.com. 03 Feb 2026.