Skip to main content
Log in

Pulmonary hypertension associated with lung diseases and hypoxemia

  • Published:
Heart Failure Reviews Aims and scope Submit manuscript

Abstract

Pulmonary hypertension that develops in the setting of underlying lung diseases such as COPD or idiopathic pulmonary fibrosis (IPF) is associated with decreased functional status, worsening hypoxemia and quality of life, and increased mortality. This complication of lung disease is complex in its origin and carries a unique set of diagnostic and therapeutic issues. This review attempts to provide an overview of mechanisms associated with the onset of pulmonary hypertension in COPD and IPF, touches on appropriate evaluation, and reviews the state of knowledge on treating pulmonary hypertension related to underlying lung disease.

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

Access this article

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

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Hoeper MM, Bogaard HJ, Condliffe R et al (2013) Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol 62(25 Suppl):D42–D50

    Article  PubMed  Google Scholar 

  2. Weitzenblum E, Chaouat A (2009) Cor pulmonale. Chron Respir Dis 6(3):177–185

    Article  CAS  PubMed  Google Scholar 

  3. Simonneau G, Gatzoulis MA, Adatia I et al (2013) Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 62(25 Suppl):D34–D41

    Article  PubMed  Google Scholar 

  4. Cuttica MJ, Kalhan R, Shlobin OA et al (2010) Categorization and impact of pulmonary hypertension in patients with advanced COPD. Respir Med 104(12):1877–1882

    Article  PubMed  Google Scholar 

  5. Lettieri CJ, Nathan SD, Barnett SD, Ahmad S, Shorr AF (2006) Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest 129(3):746–752

    Article  PubMed  Google Scholar 

  6. Thabut G, Dauriat G, Stern JB et al (2005) Pulmonary hemodynamics in advanced COPD candidates for lung volume reduction surgery or lung transplantation. Chest 127(5):1531–1536

    Article  PubMed  Google Scholar 

  7. Chaouat A, Bugnet AS, Kadaoui N et al (2005) Severe pulmonary hypertension and chronic obstructive pulmonary disease. Am J Respir Crit Care Med 172(2):189–194

    Article  PubMed  Google Scholar 

  8. Andersen CU, Mellemkjaer S, Nielsen-Kudsk JE, Bendstrup E, Hilberg O, Simonsen U (2013) Pulmonary hypertension in chronic obstructive and interstitial lung diseases. Int J Cardiol 168(3):1795–1804

    Article  PubMed  Google Scholar 

  9. Farkas L, Gauldie J, Voelkel NF, Kolb M (2011) Pulmonary hypertension and idiopathic pulmonary fibrosis: a tale of angiogenesis, apoptosis, and growth factors. Am J Respir Cell Mol Biol 45(1):1–15

    Article  CAS  PubMed  Google Scholar 

  10. Scharf SM, Iqbal M, Keller C et al (2002) Hemodynamic characterization of patients with severe emphysema. Am J Respir Crit Care Med 166(3):314–322

    Article  PubMed  Google Scholar 

  11. Weitzenblum E, Sautegeau A, Ehrhart M, Mammosser M, Pelletier A (1985) Long-term oxygen therapy can reverse the progression of pulmonary hypertension in patients with chronic obstructive pulmonary disease. Am Rev Respir Dis 131(4):493–498

    CAS  PubMed  Google Scholar 

  12. Timms RM, Khaja FU, Williams GW (1985) Hemodynamic response to oxygen therapy in chronic obstructive pulmonary disease. Ann Intern Med 102(1):29–36

    Article  CAS  PubMed  Google Scholar 

  13. Wright JL, Petty T, Thurlbeck WM (1992) Analysis of the structure of the muscular pulmonary arteries in patients with pulmonary hypertension and COPD: National Institutes of Health nocturnal oxygen therapy trial. Lung 170(2):109–124

    Article  CAS  PubMed  Google Scholar 

  14. Magee F, Wright JL, Wiggs BR, Pare PD, Hogg JC (1988) Pulmonary vascular structure and function in chronic obstructive pulmonary disease. Thorax 43(3):183–189

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Santos S, Peinado VI, Ramirez J et al (2002) Characterization of pulmonary vascular remodelling in smokers and patients with mild COPD. Eur Respir J 19(4):632–638

    Article  CAS  PubMed  Google Scholar 

  16. Seimetz M, Parajuli N, Pichl A et al (2011) Inducible NOS inhibition reverses tobacco-smoke-induced emphysema and pulmonary hypertension in mice. Cell 147(2):293–305

    Article  CAS  PubMed  Google Scholar 

  17. Barbera JA, Riverola A, Roca J et al (1994) Pulmonary vascular abnormalities and ventilation-perfusion relationships in mild chronic obstructive pulmonary disease. Am J Respir Crit Care Med 149(2 Pt 1):423–429

    Article  CAS  PubMed  Google Scholar 

  18. Elbehairy AF, Ciavaglia CE, Webb KA et al (2015) Pulmonary gas exchange abnormalities in mild chronic obstructive pulmonary disease. Implications for dyspnea and exercise intolerance. Am J Respir Crit Care Med 191(12):1384–1394

    Article  PubMed  Google Scholar 

  19. Cuttica MJ, Shah SJ, Rosenberg SR et al (2011) Right heart structural changes are independently associated with exercise capacity in non-severe COPD. PLoS One 6(12):e29069

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. Ryu JH, Krowka MJ, Pellikka PA, Swanson KL, McGoon MD (2007) Pulmonary hypertension in patients with interstitial lung diseases. Mayo Clin Proc 82(3):342–350

    Article  PubMed  Google Scholar 

  21. Nathan SD, Noble PW, Tuder RM (2007) Idiopathic pulmonary fibrosis and pulmonary hypertension: connecting the dots. Am J Respir Crit Care Med 175(9):875–880

    Article  PubMed  Google Scholar 

  22. Ebina M, Shimizukawa M, Shibata N et al (2004) Heterogeneous increase in CD34-positive alveolar capillaries in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 169(11):1203–1208

    Article  PubMed  Google Scholar 

  23. Cosgrove GP, Brown KK, Schiemann WP et al (2004) Pigment epithelium-derived factor in idiopathic pulmonary fibrosis: a role in aberrant angiogenesis. Am J Respir Crit Care Med 170(3):242–251

    Article  PubMed  Google Scholar 

  24. Chaouat A, Savale L, Chouaid C et al (2009) Role for interleukin-6 in COPD-related pulmonary hypertension. Chest 136(3):678–687

    Article  CAS  PubMed  Google Scholar 

  25. Kwon YS, Chi SY, Shin HJ et al (2010) Plasma C-reactive protein and endothelin-1 level in patients with chronic obstructive pulmonary disease and pulmonary hypertension. J Korean Med Sci 25(10):1487–1491

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Peinado VI, Pizarro S, Barbera JA (2008) Pulmonary vascular involvement in COPD. Chest 134(4):808–814

    Article  CAS  PubMed  Google Scholar 

  27. Karmouty-Quintana H, Philip K, Acero LF et al (2015) Deletion of ADORA2B from myeloid cells dampens lung fibrosis and pulmonary hypertension. FASEB J 29(1):50–60

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Peinado VI, Barbera JA, Ramirez J et al (1998) Endothelial dysfunction in pulmonary arteries of patients with mild COPD. Am J Physiol 274(6 Pt 1):L908–L913

    CAS  PubMed  Google Scholar 

  29. Giaid A, Michel RP, Stewart DJ, Sheppard M, Corrin B, Hamid Q (1993) Expression of endothelin-1 in lungs of patients with cryptogenic fibrosing alveolitis. Lancet 341(8860):1550–1554

    Article  CAS  PubMed  Google Scholar 

  30. Simler NR, Brenchley PE, Horrocks AW, Greaves SM, Hasleton PS, Egan JJ (2004) Angiogenic cytokines in patients with idiopathic interstitial pneumonia. Thorax 59(7):581–585

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  31. Renzoni EA, Walsh DA, Salmon M et al (2003) Interstitial vascularity in fibrosing alveolitis. Am J Respir Crit Care Med 167(3):438–443

    Article  PubMed  Google Scholar 

  32. Voelkel NF, Douglas IS, Nicolls M (2007) Angiogenesis in chronic lung disease. Chest 131(3):874–879

    Article  PubMed  PubMed Central  Google Scholar 

  33. Oswald-Mammosser M, Weitzenblum E, Quoix E et al (1995) Prognostic factors in COPD patients receiving long-term oxygen therapy. Importance of pulmonary artery pressure. Chest 107(5):1193–1198

    Article  CAS  PubMed  Google Scholar 

  34. Andersen KH, Iversen M, Kjaergaard J et al (2012) Prevalence, predictors, and survival in pulmonary hypertension related to end-stage chronic obstructive pulmonary disease. J Heart Lung Transplant 31(4):373–380

    Article  PubMed  Google Scholar 

  35. D’Alonzo GE, Barst RJ, Ayres SM et al (1991) Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 115(5):343–349

    Article  PubMed  Google Scholar 

  36. Benza RL, Gomberg-Maitland M, Miller DP et al (2012) The REVEAL Registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension. Chest 141(2):354–362

    Article  PubMed  Google Scholar 

  37. Humbert M, Sitbon O, Chaouat A et al (2010) Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation 122(2):156–163

    Article  PubMed  Google Scholar 

  38. Stone AC, Machan JT, Mazer J, Casserly B, Klinger JR (2011) Echocardiographic evidence of pulmonary hypertension is associated with increased 1-year mortality in patients admitted with chronic obstructive pulmonary disease. Lung 189(3):207–212

    Article  PubMed  Google Scholar 

  39. Kessler R, Faller M, Fourgaut G, Mennecier B, Weitzenblum E (1999) Predictive factors of hospitalization for acute exacerbation in a series of 64 patients with chronic obstructive pulmonary disease. Am J Respir Crit Care Med 159(1):158–164

    Article  CAS  PubMed  Google Scholar 

  40. Seeger W, Adir Y, Barbera JA et al (2013) Pulmonary hypertension in chronic lung diseases. J Am Coll Cardiol 62(25 Suppl):D109–D116

    Article  PubMed  Google Scholar 

  41. Simon MA, Deible C, Mathier MA et al (2009) Phenotyping the right ventricle in patients with pulmonary hypertension. Clin Transl Sci 2(4):294–299

    Article  PubMed  PubMed Central  Google Scholar 

  42. Badano LP, Ginghina C, Easaw J et al (2010) Right ventricle in pulmonary arterial hypertension: haemodynamics, structural changes, imaging, and proposal of a study protocol aimed to assess remodelling and treatment effects. Eur J Echocardiogr. 11(1):27–37

    Article  PubMed  Google Scholar 

  43. Falk JA, Kadiev S, Criner GJ, Scharf SM, Minai OA, Diaz P (2008) Cardiac disease in chronic obstructive pulmonary disease. Proc Am Thorac Soc 5(4):543–548

    Article  PubMed  PubMed Central  Google Scholar 

  44. Fisher MR, Criner GJ, Fishman AP et al (2007) Estimating pulmonary artery pressures by echocardiography in patients with emphysema. Eur Respir J 30(5):914–921

    Article  CAS  PubMed  Google Scholar 

  45. Badesch DB, Champion HC, Sanchez MA et al (2009) Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol 54(1 Suppl):S55–S66

    Article  PubMed  Google Scholar 

  46. Devaraj A, Wells AU, Meister MG, Corte TJ, Hansell DM (2008) The effect of diffuse pulmonary fibrosis on the reliability of CT signs of pulmonary hypertension. Radiology 249(3):1042–1049

    Article  PubMed  Google Scholar 

  47. Nadrous HF, Pellikka PA, Krowka MJ et al (2005) The impact of pulmonary hypertension on survival in patients with idiopathic pulmonary fibrosis. Chest 128(6 Suppl):616S–617S

    Article  PubMed  Google Scholar 

  48. Hamada K, Nagai S, Tanaka S et al (2007) Significance of pulmonary arterial pressure and diffusion capacity of the lung as prognosticator in patients with idiopathic pulmonary fibrosis. Chest 131(3):650–656

    Article  PubMed  Google Scholar 

  49. Kimura M, Taniguchi H, Kondoh Y et al (2013) Pulmonary hypertension as a prognostic indicator at the initial evaluation in idiopathic pulmonary fibrosis. Respiration 85(6):456–463

    Article  PubMed  Google Scholar 

  50. Behr J, Ryu JH (2008) Pulmonary hypertension in interstitial lung disease. Eur Respir J 31(6):1357–1367

    Article  CAS  PubMed  Google Scholar 

  51. Nathan SD, Shlobin OA, Ahmad S et al (2008) Serial development of pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Respiration 76(3):288–294

    Article  CAS  PubMed  Google Scholar 

  52. Shorr AF, Wainright JL, Cors CS, Lettieri CJ, Nathan SD (2007) Pulmonary hypertension in patients with pulmonary fibrosis awaiting lung transplant. Eur Respir J 30(4):715–721

    Article  CAS  PubMed  Google Scholar 

  53. Andersen CU, Mellemkjaer S, Hilberg O, Nielsen-Kudsk JE, Simonsen U, Bendstrup E (2012) Pulmonary hypertension in interstitial lung disease: prevalence, prognosis and 6 min walk test. Respir Med 106(6):875–882

    Article  PubMed  Google Scholar 

  54. Modrykamien AM, Gudavalli R, McCarthy K, Parambil J (2010) Echocardiography, 6-min walk distance, and distance-saturation product as predictors of pulmonary arterial hypertension in idiopathic pulmonary fibrosis. Respir Care 55(5):584–588

    PubMed  Google Scholar 

  55. Swigris JJ, Olson AL, Shlobin OA, Ahmad S, Brown KK, Nathan SD (2011) Heart rate recovery after 6-min walk test predicts pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Respirology 16(3):439–445

    Article  PubMed  PubMed Central  Google Scholar 

  56. Patel NM, Lederer DJ, Borczuk AC, Kawut SM (2007) Pulmonary hypertension in idiopathic pulmonary fibrosis. Chest 132(3):998–1006

    Article  PubMed  Google Scholar 

  57. Zisman DA, Ross DJ, Belperio JA et al (2007) Prediction of pulmonary hypertension in idiopathic pulmonary fibrosis. Respir Med 101(10):2153–2159

    Article  PubMed  PubMed Central  Google Scholar 

  58. Chaouat A, Weitzenblum E, Krieger J, Oswald M, Kessler R (1996) Pulmonary hemodynamics in the obstructive sleep apnea syndrome. Results in 220 consecutive patients. Chest 109(2):380–386

    Article  CAS  PubMed  Google Scholar 

  59. Minai OA, Ricaurte B, Kaw R et al (2009) Frequency and impact of pulmonary hypertension in patients with obstructive sleep apnea syndrome. Am J Cardiol 104(9):1300–1306

    Article  PubMed  Google Scholar 

  60. Bady E, Achkar A, Pascal S, Orvoen-Frija E, Laaban JP (2000) Pulmonary arterial hypertension in patients with sleep apnoea syndrome. Thorax 55(11):934–939

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  61. Niijima M, Kimura H, Edo H et al (1999) Manifestation of pulmonary hypertension during REM sleep in obstructive sleep apnea syndrome. Am J Respir Crit Care Med 159(6):1766–1772

    Article  CAS  PubMed  Google Scholar 

  62. Sajkov D, Wang T, Saunders NA, Bune AJ, Neill AM (1999) Douglas Mcevoy R. Daytime pulmonary hemodynamics in patients with obstructive sleep apnea without lung disease. Am J Respir Crit Care Med 159(5 Pt 1):1518–1526

    Article  CAS  PubMed  Google Scholar 

  63. Sanner BM, Doberauer C, Konermann M, Sturm A, Zidek W (1997) Pulmonary hypertension in patients with obstructive sleep apnea syndrome. Arch Intern Med 157(21):2483–2487

    Article  CAS  PubMed  Google Scholar 

  64. Laks L, Lehrhaft B, Grunstein RR, Sullivan CE (1995) Pulmonary hypertension in obstructive sleep apnoea. Eur Respir J 8(4):537–541

    CAS  PubMed  Google Scholar 

  65. Kessler R, Chaouat A, Schinkewitch P et al (2001) The obesity-hypoventilation syndrome revisited: a prospective study of 34 consecutive cases. Chest 120(2):369–376

    Article  CAS  PubMed  Google Scholar 

  66. Sugerman HJ, Baron PL, Fairman RP, Evans CR, Vetrovec GW (1988) Hemodynamic dysfunction in obesity hypoventilation syndrome and the effects of treatment with surgically induced weight loss. Ann Surg 207(5):604–613

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  67. Ahmed Q, Chung-Park M, Tomashefski JF Jr (1997) Cardiopulmonary pathology in patients with sleep apnea/obesity hypoventilation syndrome. Hum Pathol 28(3):264–269

    Article  CAS  PubMed  Google Scholar 

  68. Atwood CW Jr, McCrory D, Garcia JG, Abman SH, Ahearn GS (2004) Pulmonary artery hypertension and sleep-disordered breathing: ACCP evidence-based clinical practice guidelines. Chest 126(1 Suppl):72S–77S

    Article  PubMed  Google Scholar 

  69. Arias MA, Garcia-Rio F, Alonso-Fernandez A, Martinez I, Villamor J (2006) Pulmonary hypertension in obstructive sleep apnoea: effects of continuous positive airway pressure: a randomized, controlled cross-over study. Eur Heart J 27(9):1106–1113

    Article  PubMed  Google Scholar 

  70. Colish J, Walker JR, Elmayergi N et al (2012) Obstructive sleep apnea: effects of continuous positive airway pressure on cardiac remodeling as assessed by cardiac biomarkers, echocardiography, and cardiac MRI. Chest 141(3):674–681

    Article  PubMed  Google Scholar 

  71. Sajkov D, Wang T, Saunders NA, Bune AJ, McEvoy RD (2002) Continuous positive airway pressure treatment improves pulmonary hemodynamics in patients with obstructive sleep apnea. Am J Respir Crit Care Med 165(2):152–158

    Article  PubMed  Google Scholar 

  72. Phillips BG, Narkiewicz K, Pesek CA, Haynes WG, Dyken ME, Somers VK (1999) Effects of obstructive sleep apnea on endothelin-1 and blood pressure. J Hypertens 17(1):61–66

    Article  CAS  PubMed  Google Scholar 

  73. Yokoe T, Minoguchi K, Matsuo H et al (2003) Elevated levels of C-reactive protein and interleukin-6 in patients with obstructive sleep apnea syndrome are decreased by nasal continuous positive airway pressure. Circulation 107(8):1129–1134

    Article  CAS  PubMed  Google Scholar 

  74. Celli BR, MacNee W (2004) Standards for the diagnosis and treatment of patients with COPD: a summary of the ATS/ERS position paper. Eur Respir J 23(6):932–946

    Article  CAS  PubMed  Google Scholar 

  75. Vestbo J, Hurd SS, Agusti AG et al (2013) Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease: GOLD executive summary. Am J Respir Crit Care Med 187(4):347–365

    Article  CAS  PubMed  Google Scholar 

  76. Raghu G, Collard HR, Egan JJ et al (2011) An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 183(6):788–824

    Article  PubMed  Google Scholar 

  77. Finlay M, Middleton HC, Peake MD, Howard P (1983) Cardiac output, pulmonary hypertension, hypoxaemia and survival in patients with chronic obstructive airways disease. Eur J Respir Dis 64(4):252–263

    CAS  PubMed  Google Scholar 

  78. Medical Research Council Working Party (1981) Long term domiciliary oxygen therapy in chronic hypoxic cor pulmonale complicating chronic bronchitis and emphysema. Report of the Medical Research Council Working Party. Lancet 1(8222):681–686

    Google Scholar 

  79. Nocturnal Oxygen Therapy Working Group (1980) Continuous or nocturnal oxygen therapy in hypoxemic chronic obstructive lung disease: a clinical trial. Nocturnal Oxygen Therapy Trial Group. Ann Intern Med 93(3):391–398

    Article  Google Scholar 

  80. Zielinski J, Tobiasz M, Hawrylkiewicz I, Sliwinski P, Palasiewicz G (1998) Effects of long-term oxygen therapy on pulmonary hemodynamics in COPD patients: a 6-year prospective study. Chest 113(1):65–70

    Article  CAS  PubMed  Google Scholar 

  81. McNicholas WT, Verbraecken J, Marin JM (2013) Sleep disorders in COPD: the forgotten dimension. Eur Respir Rev Off J Eur Respir Soc 22(129):365–375

    Article  Google Scholar 

  82. Stolz D, Rasch H, Linka A et al (2008) A randomised, controlled trial of bosentan in severe COPD. Eur Respir J 32(3):619–628

    Article  CAS  PubMed  Google Scholar 

  83. Valerio G, Bracciale P, Grazia D’Agostino A (2009) Effect of bosentan upon pulmonary hypertension in chronic obstructive pulmonary disease. Ther Adv Respir Dis 3(1):15–21

    Article  PubMed  Google Scholar 

  84. King TE Jr, Brown KK, Raghu G et al (2011) BUILD-3: a randomized, controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 184(1):92–99

    Article  PubMed  Google Scholar 

  85. Raghu G, Behr J, Brown KK et al (2013) Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomized trial. Ann Intern Med 158(9):641–649

    Article  PubMed  Google Scholar 

  86. Raghu G, Million-Rousseau R, Morganti A, Perchenet L, Behr J (2013) Macitentan for the treatment of idiopathic pulmonary fibrosis: the randomised controlled MUSIC trial. Eur Respir J 42(6):1622–1632

    Article  CAS  PubMed  Google Scholar 

  87. Corte TJ, Keir GJ, Dimopoulos K et al (2014) Bosentan in pulmonary hypertension associated with fibrotic idiopathic interstitial pneumonia. Am J Respir Crit Care Med 190(2):208–217

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  88. Blanco I, Gimeno E, Munoz PA et al (2010) Hemodynamic and gas exchange effects of sildenafil in patients with chronic obstructive pulmonary disease and pulmonary hypertension. Am J Respir Crit Care Med 181(3):270–278

    Article  CAS  PubMed  Google Scholar 

  89. Rietema H, Holverda S, Bogaard HJ et al (2008) Sildenafil treatment in COPD does not affect stroke volume or exercise capacity. Eur Respir J 31(4):759–764

    Article  CAS  PubMed  Google Scholar 

  90. Lederer DJ, Bartels MN, Schluger NW et al (2012) Sildenafil for chronic obstructive pulmonary disease: a randomized crossover trial. COPD 9(3):268–275

    Article  PubMed  Google Scholar 

  91. Holverda S, Rietema H, Bogaard HJ et al (2008) Acute effects of sildenafil on exercise pulmonary hemodynamics and capacity in patients with COPD. Pulm Pharmacol Ther 21(3):558–564

    Article  CAS  PubMed  Google Scholar 

  92. Zisman DA, Schwarz M, Anstrom KJ, Collard HR, Flaherty KR, Hunninghake GW (2010) A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. N Engl J Med 363(7):620–628

    Article  PubMed  Google Scholar 

  93. Han MK, Bach DS, Hagan PG et al (2013) Sildenafil preserves exercise capacity in patients with idiopathic pulmonary fibrosis and right-sided ventricular dysfunction. Chest 143(6):1699–1708

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  94. Ghofrani HA, D’Armini AM, Grimminger F et al (2013) Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med 369(4):319–329

    Article  CAS  PubMed  Google Scholar 

  95. Ghofrani HA, Galie N, Grimminger F et al (2013) Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med 369(4):330–340

    Article  CAS  PubMed  Google Scholar 

  96. Archer SL, Mike D, Crow J, Long W, Weir EK (1996) A placebo-controlled trial of prostacyclin in acute respiratory failure in COPD. Chest 109(3):750–755

    Article  CAS  PubMed  Google Scholar 

  97. Hegewald MJ, Elliott CG (2009) Sustained improvement with iloprost in a COPD patient with severe pulmonary hypertension. Chest 135(2):536–537

    Article  PubMed  Google Scholar 

  98. Dernaika TA, Beavin M, Kinasewitz GT (2010) Iloprost improves gas exchange and exercise tolerance in patients with pulmonary hypertension and chronic obstructive pulmonary disease. Respir Int Rev Thorac Dis 79(5):377–382

    CAS  Google Scholar 

  99. Olschewski H, Ghofrani HA, Walmrath D et al (1999) Inhaled prostacyclin and iloprost in severe pulmonary hypertension secondary to lung fibrosis. Am J Respir Crit Care Med 160(2):600–607

    Article  CAS  PubMed  Google Scholar 

  100. Poms AD, Turner M, Farber HW, Meltzer LA, McGoon MD (2013) Comorbid conditions and outcomes in patients with pulmonary arterial hypertension: a REVEAL registry analysis. Chest 144(1):169–176

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Michael J. Cuttica.

Ethics declarations

Conflict of interest

Dr. Cuttica has received consulting and speaking fees from Actelion Pharmaceuticals, Gilead Health Sciences and United Therapeutics. He has received consulting fees from Bayer.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Cuttica, M.J. Pulmonary hypertension associated with lung diseases and hypoxemia. Heart Fail Rev 21, 299–308 (2016). https://doi.org/10.1007/s10741-016-9551-x

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10741-016-9551-x

Keywords

Navigation