pulmonary hypertension

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Chronic disorder with mean pulmonary artery pressure of > 20-25 mm Hg at rest or > 30 mm Hg with exercise & a pulmonary capillary wedge pressure < 15 mm Hg measured during right heart catheterization.*

* see Differential diagnosis: below for causes of acute elevation in pulmonary artery pressure.


* left heart disease & hypoxemia due to chronic lung disease most common causes[3]

* see causes of pulmonary hypertension for more detail


* increased mean pulmonary artery pressure with elevated pulmonary artery capillary wedge pressure indicates pulmonary venous hypertension


Clinical manifestations

(also see primary pulmonary hypertension)


Diagnostic procedures



* mean pulmonary artery pressure of 19-24 mm Hg

Differential diagnosis


More general terms

More specific terms

Additional terms


  1. Manual of Medical Therapeutics, 28th ed, Ewald & McKenzie (eds), Little, Brown & Co, Boston, 1995, pg 256
  2. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 778-81
  3. 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 3.11 3.12 3.13 3.14 3.15 3.16 3.17 3.18 3.19 3.20 3.21 3.22 3.23 3.24 3.25 3.26 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2018, 2022.
    Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
    McGlothlin DP, Granton J, Klepetko W, et al. ISHLT consensus statement: Perioperative management of patients with pulmonary hypertension and right heart failure undergoing surgery [Editorial]. J Heart Lung Transplant. 2022;41:1135-1194. PMID: https://www.ncbi.nlm.nih.gov/pubmed/36123001
  4. Smith R., Jewish Home for the Aging, Reseda CA, 2001, unpublished
  5. 5.0 5.1 Galie N et al, Sildenafil citrate therapy for pulmonary arterial hypertension N Engl J Med 353(20):2148, 2005 PMID: https://www.ncbi.nlm.nih.gov/pubmed/16291984
  6. Badesch DB, Abman SH, Simonneau G, Rubin LJ, McLaughlin VV. Medical Therapy for Pulmonary Arterial Hypertension: Updated ACCP Evidence-Based Clinical Practice Guidelines. Chest. 2007 Jun;131(6):1917-28. PMID: https://www.ncbi.nlm.nih.gov/pubmed/17565025 Corresponding NGC guideline withdrawn Jan 2013 http://www.chestjournal.org/cgi/content/full/131/6/1917
  7. McLaughlin VV 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. Circulation. 2009 Mar 30 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/19332472 <Internet> http://content.onlinejacc.org/cgi/content/full/j.jacc.2009.01.004
  8. 8.0 8.1 8.2 8.3 Parent F et al. A hemodynamic study of pulmonary hypertension in sickle cell disease. N Engl J Med 2011 Jul 7; 365:44. PMID: https://www.ncbi.nlm.nih.gov/pubmed/21732836
  9. 9.0 9.1 9.2 Geriatrics at your Fingertips, 13th edition, 2011 Reuben DB et al (eds) American Geriatric Society
  10. Brown LM, Chen H, Halpern S et al Delay in recognition of pulmonary arterial hypertension: factors identified from the REVEAL Registry. Chest. 2011 Jul;140(1):19-26 PMID: https://www.ncbi.nlm.nih.gov/pubmed/21393391
  11. 11.0 11.1 Shah SJ. Pulmonary hypertension. JAMA. 2012;308(13):1366-1374. PMID: https://www.ncbi.nlm.nih.gov/pubmed/23032553
  12. 12.0 12.1 Snomed :accession 697899000 Snomed :accession 697900005
  13. 13.0 13.1 13.2 Pugh ME et al. Causes of pulmonary hypertension in the elderly. Chest 2014 Jul; 146:159 http://journal.publications.chestnet.org/article.aspx?articleid=1819592
  14. ARUP Consult: Pulmonary Arterial Hypertension - PAH The Physician's Guide to Laboratory Test Selection & Interpretation https://arupconsult.com/content/pulmonary-arterial-hypertension
    ARUP Consult: Pulmonary Arterial Hypertension https://arupconsult.com/ati/pulmonary-arterial-hypertension
  15. 15.0 15.1 Klings ES, Machado RF, Barst RJ et al American Thoracic Society Ad Hoc Committee on Pulmonary Hypertension of Sickle Cell Disease. An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease. Am J Respir Crit Care Med. 2014 Mar 15;189(6):727-40 PMID: https://www.ncbi.nlm.nih.gov/pubmed/24628312
  16. 16.0 16.1 Galie N et al. Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension. N Engl J Med 2015 Aug 27; 373:834 PMID: https://www.ncbi.nlm.nih.gov/pubmed/26308684
  17. 17.0 17.1 Abman SH et al Pediatric Pulmonary Hypertension. Guidelines From the American Heart Association and American Thoracic Society. Circulation. Nov 3, 2015 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/26534956 <Internet> http://circ.ahajournals.org/content/early/2015/10/29/CIR.0000000000000329.full.pdf+html
  18. Galie 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):2493-537. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19713419 Free Article
  19. 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 28;53(17):1573-619 PMID: https://www.ncbi.nlm.nih.gov/pubmed/19389575 Free full text
  20. Galie N, Corris PA, Frost A et al Updated treatment algorithm of pulmonary arterial hypertension. J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D60-72. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/24355643 Free Article
  21. McGoon MD, Kane GC. Pulmonary hypertension: diagnosis and management. Mayo Clin Proc. 2009 Feb;84(2):191-207. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19181654 Free PMC Article
  22. Simonneau G, Gatzoulis MA, Adatia I et al Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D34-41. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/24355639 Free Article
  23. 23.0 23.1 Assad TR, Maron BA, Robbins IM et al. Prognostic effect and longitudinal hemodynamic assessment of borderline pulmonary hypertension. JAMA Cardiol 2017 Oct 25; PMID: https://www.ncbi.nlm.nih.gov/pubmed/29071338
  24. 24.0 24.1 Aviram G, Rozenbaum Z, Ziv-Baran T et al. Identification of pulmonary hypertension caused by left-sided heart disease (World Health Organization group 2) based on cardiac chamber volumes derived from chest CT imaging. Chest 2017 Oct; 152:792. PMID: https://www.ncbi.nlm.nih.gov/pubmed/28506612
  25. Thakkar V, Lau EM. Connective tissue disease-related pulmonary arterial hypertension. Best Pract Res Clin Rheumatol. 2016 Feb;30(1):22-38. PMID: https://www.ncbi.nlm.nih.gov/pubmed/27421214
  26. Vonk Noordegraaf A, Groeneveldt JA, Bogaard HJ. Pulmonary hypertension. Eur Respir Rev. 2016 Mar;25(139):4-11. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/26929415 Free Article
  27. Galie N, Humbert M, Vachiery JL et al 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 Respir J. 2015 Oct;46(4):903-75. PMID: https://www.ncbi.nlm.nih.gov/pubmed/26318161 Free Article
  28. Kato M, Atsumi T. Pulmonary arterial hypertension associated with connective tissue diseases: A review focusing on distinctive clinical aspects. Eur J Clin Invest. 2018 Feb;48(2). Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/29285766
  29. Humbert M, Kovacs G, Hoeper MM, et al; ESC/ERS Scientific Document Group. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2023;61. PMID: https://www.ncbi.nlm.nih.gov/pubmed/36028254
  30. 30.0 30.1 30.2 Hoeper MM, Ewert R, Jansa P et al. Randomized, Multicenter Study to Assess the Effects of Different Doses of Sildenafil on Mortality in Adults With Pulmonary Arterial Hypertension. Circulation. 2024 May 16. PMID: https://www.ncbi.nlm.nih.gov/pubmed/38752352 Free article