Skip to main content
SpringerLink
Log in

Pulmonary arterial hypertension

  • Published:
Current Atherosclerosis Reports Aims and scope Submit manuscript

Abstract

Pulmonary arterial hypertension is a progressive disease of pulmonary vasculature characterized by increased mean pulmonary arterial pressure and elevated vascular resistance with normal left-sided pressures, differentiating it from left-sided heart disease. The pathogenesis involves thrombosis, vasoconstriction, and remodeling of small pulmonary arteries. It presents with common symptoms such as shortness of breath, chest pain, and decreased exercise tolerance. Left untreated, progressive increase in right ventricular overload leads to right heart failure and death. Over the course of the past decade, better understanding of the pathogenesis and an increasing number of treatment options have resulted in improved prognosis and quality of life.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References and Recommended Reading

  1. D’Alonzo GE, Barst RJ, Ayres SM, et al.: Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 1991:115:343–349.

    PubMed  CAS  Google Scholar 

  2. Gaine SP, Rubin LJ: Primary pulmonary hypertension. Lancet 1998, 352:719–725.

    Article  PubMed  CAS  Google Scholar 

  3. Simonneau G, Galie N, Rubin LJ, et al.: Clinical classification of pulmonary hypertension. J Am Coll Cardiol 2004, 43(Suppl 1):5S–12S.

    Article  PubMed  Google Scholar 

  4. Newman JH, Trembath RC, Morse JA, et al.: Genetic basis of pulmonary arterial hypertension: current understanding and future directions. J Am Coll Cardiol 2004, 43(Suppl 1):33S–39S.

    Article  PubMed  CAS  Google Scholar 

  5. Magliano M, Isenberg DA, Hillson J: Pulmonary hypertension in autoimmune rheumatic diseases: where are we now? Arthritis Rheum 2002, 46:1997–2009.

    Article  PubMed  CAS  Google Scholar 

  6. Chang B, Schachna L, White B, et al.: Natural history of mild moderate pulmonary hypertension and the risk factors for severe pulmonary hypertension in scleroderma. J Rheumatol 2006, 33:269–274.

    PubMed  Google Scholar 

  7. Koh ET, Lee P, Gladman DD, Abu-Shakra M: Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients. Br J Rheumatol 1996, 35:989–993.

    Article  PubMed  CAS  Google Scholar 

  8. Hopkins WE, Ochoa LL, Richardson GW, Trulock EP: Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome. J Heart Lung Transplant 1996, 15:100–105.

    PubMed  CAS  Google Scholar 

  9. Halank M, Ewert R, Seyfarth HJ, Hoeffken G: Portopulmonary hypertension. J Gastroenterol 2006, 41:837–847.

    Article  PubMed  Google Scholar 

  10. Ashfaq M, Chinnakotla S, Rogers L, et al.: The impact of treatment of portopulmonary hypertension on survival following liver transplantation. Am J Transplant 2007, 7:1258–1264.

    Article  PubMed  CAS  Google Scholar 

  11. Mehta NJ, Khan IA, Mehta RN, Sepkowitz DA: HIV-related pulmonary hypertension: analytic review of 131 cases. Chest 2000, 118:1133–1141.

    Article  PubMed  CAS  Google Scholar 

  12. Zuber JP, Calmy A, Evison JM, et al.: Pulmonary arterial hypertension related to HIV infection: improved hemodynamics and survival associated with antiretroviral therapy. Clin Infect Dis 2004, 38:1178–1185.

    Article  PubMed  Google Scholar 

  13. Rich S, Rubin L, Walker AM, et al.: Anorexigens and pulmonary hypertension in the United States: results from the surveillance of North American pulmonary hypertension. Chest 2000, 117:870–874.

    Article  PubMed  CAS  Google Scholar 

  14. Marcos E, Fadel E, Sanchez O: Serotonin-induced smooth muscle hyperplasia in various forms of human pulmonary hypertension. Circ Res 2004, 94:1263–1270.

    Article  PubMed  CAS  Google Scholar 

  15. Chin KM, Channick RN, Rubin LJ: Is methamphetamine use associated with idiopathic pulmonary arterial hypertension? Chest 2006, 130:1657–1663.

    Article  PubMed  CAS  Google Scholar 

  16. Fawzy ME, Hassan W, Stefadouros M, et al.: Prevalence and fate of severe pulmonary hypertension in 559 consecutive patients with severe rheumatic mitral stenosis undergoing mitral balloon valvotomy. J Heart Valve Dis 2004, 13:942–947.

    PubMed  Google Scholar 

  17. Califf RM, Adams KF, McKenna WJ, et al.: A randomized controlled trial of epoprostenol therapy for severe congestive heart failure: the Flolan International Randomized Survival Trial (FIRST). Am Heart J 1997, 134:44–54.

    Article  PubMed  CAS  Google Scholar 

  18. Lewis GD, Shah R, Shahzad K, et al.: Sildenafil improves exercise capacity and quality of life in patients with systolic heart failure and secondary pulmonary hypertension. Circulation 2007, 116:1555–1562.

    Article  PubMed  CAS  Google Scholar 

  19. Patel NM, Lederer DJ, Borczuk AC, Kawut SM: Pulmonary hypertension in idiopathic pulmonary fibrosis. Chest 2007, 132:998–1006.

    Article  PubMed  Google Scholar 

  20. Auger WR, Kim NH, Kerr KM, et al.: Chronic thromboembolic pulmonary hypertension. Clin Chest Med 2007, 28:255–269.

    Article  PubMed  Google Scholar 

  21. Barst RJ, McGoon M, Torbicki A, et al.: Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol 2004, 43(Suppl 12):40s–47s.

    Article  PubMed  Google Scholar 

  22. Lilienfeld DE, Rubin LJ: Mortality from primary pulmonary hypertension in the United States, 1979–96. Chest 2000, 117:796–800.

    Article  PubMed  CAS  Google Scholar 

  23. Rich S [editor]: Executive summary from the World Symposium on Primary Pulmonary Hypertension. Presented at World Symposium on Primary Pulmonary Hypertension. Evian, France; September 6–10, 1998.

  24. 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, 126(Suppl 1):14S–34S.

    Article  PubMed  Google Scholar 

  25. Rubin LJ; American College of Chest Physicians: Diagnosis and management of pulmonary arterial hypertension: ACCP evidence based clinical practice guidelines. Chest 2004, 126(Suppl 1):7s–10s.

    Article  PubMed  Google Scholar 

  26. Chin KM, Rubin LJ: Pulmonary arterial hypertension. J Am Coll Cardiol 2008, 51:1527–1538.

    Article  PubMed  Google Scholar 

  27. Sitbon O, Humbert M, Jais X, et al.: Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005, 111:3105–3111.

    Article  PubMed  CAS  Google Scholar 

  28. Schannwell CM, Steiner S, Strauer BE: Diagnostics in pulmonary hypertension. J Physiol Pharmacol 2007, 58(Suppl 5):591–602.

    PubMed  Google Scholar 

  29. Johnson SR, Mehta S, Granton JT: Anticoagulation in pulmonary arterial hypertension: a qualitative systematic review. Eur Respir J 2006, 28:999–1004.

    Article  PubMed  CAS  Google Scholar 

  30. Badesch DB, Abman SH, Ahearn GS, et al.: Medical therapy for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004, 126(Suppl 1):35S–62S.

    Article  PubMed  Google Scholar 

  31. Rich S, Kaufmann E, Levy PS: The effect of high doses of calcium channel blockers on survival in primary pulmonary hypertension. N Engl J Med 1992, 327:76–81.

    PubMed  CAS  Google Scholar 

  32. Badesch DB, Abman SH, Simonneau G: Medical therapy for pulmonary arterial hypertension: Updated ACCP evidence-based clinical practice guidelines. Chest 2007, 131:1917–1928.

    Article  PubMed  Google Scholar 

  33. Barst RJ, Rubin LJ, Long WA, et al.: The Primary Pulmonary Hypertension Study Group: A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med 1996, 334:296–302.

    Article  PubMed  CAS  Google Scholar 

  34. McLaughlin VV, Shillington A, Rich S: Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation 2002, 106:1477–1482.

    Article  PubMed  CAS  Google Scholar 

  35. Simonneau G, Barst RJ, Galie N, et al., for the Treprostinil Study Group: Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a doubleblind, randomized, placebocontrolled trial. Am J Respir Crit Care Med 2002, 165:800–804.

    PubMed  Google Scholar 

  36. Olschewski H, Simonneau G, Galie N, et al., for the Aerosolized Iloprost Randomized Study Group: Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 2002, 347:322–329.

    Article  PubMed  CAS  Google Scholar 

  37. Galie N, Ghofrani HA, Torbicki A, et al.: Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005, 353:2148–2157.

    Article  PubMed  CAS  Google Scholar 

  38. Rubin LJ, Badesch DB, Barst RJ, et al.: Bosentan therapy for pulmonary arterial hypertension [published correction appears in N Engl J Med. 2002;346:1258]. N Engl J Med 2002, 346:896–903.

    Article  PubMed  CAS  Google Scholar 

  39. Galiè N, Olschewski H, Oudiz RJ, et al.: Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation 2008, 117:3010–3019.

    Article  PubMed  Google Scholar 

  40. McLaughlin VV, Oudiz RJ, Frost A, et al.: Randomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertension. Am J Respir Crit Care Med 2006, 174:1257–1263.

    Article  PubMed  CAS  Google Scholar 

  41. Hoeper MM, Leuchte H, Halank M, et al.: Combining inhaled iloprost with bosentan in patients with idiopathic pulmonary arterial hypertension. Eur Respir J 2006; 28 :691–694.

    Google Scholar 

  42. Humbert M, Barst RJ, Robbins IM, et al.: Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2. Eur Respir J 2004, 24:353–359.

    Article  PubMed  CAS  Google Scholar 

  43. Doyle RL, McCrory D, Channick RN, et al.; American College of Chest Physicians: Surgical treatments/interventions for pulmonary arterial hypertension: ACCP evidence based clinical practice guidelines. Chest 2004, 126(Suppl 1):63s–71s.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. University of Cincinnati Medical Center, 231 Albert Sabin Way, Cincinnati, OH, 45267, USA

    Mehmet Cilingiroglu

Authors
  1. Aydin Uzunpinar
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Mehmet Cilingiroglu
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Mehmet Cilingiroglu.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Uzunpinar, A., Cilingiroglu, M. Pulmonary arterial hypertension. Curr Atheroscler Rep 11, 139–145 (2009). https://doi.org/10.1007/s11883-009-0022-x

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11883-009-0022-x

Keywords

Search

Navigation