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Management of Pulmonary Hypertension in Patients with Chronic Lung Disease

  • Pulmonary Hypertension (JR Klinger, Section Editor)
  • Published:
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Abstract

Pulmonary hypertension (PH) is a common complication of chronic pulmonary diseases, especially in advanced disease, and is associated with greater mortality and worse clinical course. Patients with symptoms that exceed those expected by their pulmonary disease should be further evaluated by echocardiography. Confirmatory right heart catheterization is indicated in those conditions where the results of the hemodynamic assessment will determine treatment options. The treatment of choice for patients who are hypoxemic and have pulmonary hypertension associated with chronic lung disease is long-term oxygen therapy. Conventional vasodilators or drugs approved for pulmonary arterial hypertension are not recommended in patients with mild-to-moderate PH because they may impair gas exchange and because there is a lack of evidence supporting their efficacy. Patients with severe PH should be considered for referral to a center with expertise in PH and lung diseases. Ideally, these patients should be included in randomized controlled trials to determine which patients are more likely to derive benefit and which therapies are most likely to be successful.

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References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Peinado VI, Pizarro S, Barberà JA. Pulmonary vascular involvement in COPD. Chest. 2008;134:808–14.

    Article  CAS  PubMed  Google Scholar 

  2. Barberà JA, Peinado VI, Santos S. Pulmonary hypertension in chronic obstructive pulmonary disease. Eur Respir J. 2003;21:892–905.

    Article  PubMed  Google Scholar 

  3. Peinado VI, Barberà JA, Ramirez J, Gomez FP, Roca J, Jover L, et al. Endothelial dysfunction in pulmonary arteries of patients with mild COPD. Am J Physiol. 1998;274:L908–13.

    CAS  PubMed  Google Scholar 

  4. Seeger W, Adir Y, Barberà JA, Champion H, Coghlan JG, Cottin V, et al. Pulmonary hypertension in chronic lung diseases. J Am Coll Cardiol. 2013;62:D109–16. Task Force recommendations from the 5th world symposium on pulmonary hypertension for the management of pulmonary hypertension associated with lung diseases.

    Article  PubMed  Google Scholar 

  5. Vestbo J, Hurd SS, Agusti AG, Jones PW, Vogelmeier C, Anzueto A, et al. Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease: GOLD executive summary. Am J Respir Crit Care Med. 2013;187:347–65.

    Article  CAS  PubMed  Google Scholar 

  6. Andersen KH, Iversen M, Kjaergaard J, Mortensen J, Nielsen-Kudsk JE, Bendstrup E, et al. Prevalence, predictors, and survival in pulmonary hypertension related to end-stage chronic obstructive pulmonary disease. J Heart Lung Transplant. 2012;31:373–80. Recent study on the characteristics and prognosis of COPD patients presenting with pulmonary hypertension.

    Article  PubMed  Google Scholar 

  7. Cuttica MJ, Kalhan R, Shlobin OA, Ahmad S, Gladwin M, Machado RF, et al. Categorization and impact of pulmonary hypertension in patients with advanced COPD. Respir Med. 2010;104:1877–82.

    Article  PubMed  Google Scholar 

  8. Thabut G, Dauriat G, Stern JB, Logeart D, Levy A, Marrash-Chahla R, et al. Pulmonary hemodynamics in advanced COPD candidates for lung volume reduction surgery or lung transplantation. Chest. 2005;127:1531–6.

    Article  PubMed  Google Scholar 

  9. Chaouat A, Bugnet AS, Kadaoui N, Schott R, Enache I, Ducolone A, et al. Severe pulmonary hypertension and chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 2005;172:189–94.

    Article  PubMed  Google Scholar 

  10. Chaouat A, Naeije R, Weitzenblum E. Pulmonary hypertension in COPD. Eur Respir J. 2008;32:1371–85.

    Article  CAS  PubMed  Google Scholar 

  11. Hilde JM, Skjorten I, Hansteen V, Melsom MN, Hisdal J, Humerfelt S, et al. Haemodynamic responses to exercise in patients with COPD. Eur Respir J. 2013;41:1031–41.

    Article  CAS  PubMed  Google Scholar 

  12. Portillo K, Torralba Y, Blanco I, Burgos F, Rodriguez-Roisin R, Rios J, Roca J, Barbera JA. Pulmonary hemodynamic profile in chronic obstructive pulmonary disease. Int J Chron Obstruct Pulm Dis. 2015. doi:10.2147/COPD.S78180.

  13. Kessler R, Faller M, Weitzenblum E, Chaouat A, Aykut A, Ducolone A, et al. “Natural history” of pulmonary hypertension in a series of 131 patients with chronic obstructive lung disease. Am J Respir Crit Care Med. 2001;164:219–24.

    Article  CAS  PubMed  Google Scholar 

  14. Weitzenblum E, Sautegeau A, Ehrhart M, Mammosser M, Hirth C, Roegel E. Long-term course of pulmonary arterial pressure in chronic obstructive pulmonary disease. Am Rev Respir Dis. 1984;130:993–8.

    CAS  PubMed  Google Scholar 

  15. Kessler R. Predictive factors of hospitalization for acute exacerbation in a series of 64 patients with chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 1999;159(1):158–64.

  16. Wells JM, Washko GR, Han MK, Abbas N, Nath H, Mamary AJ, et al. Pulmonary arterial enlargement and acute exacerbations of COPD. N Engl J Med. 2012;367:913–21.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  17. Barberà JA, Riverola A, Roca J, Ramirez J, Wagner PD, Ros D, et al. Pulmonary vascular abnormalities and ventilation-perfusion relationships in mild chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 1994;149:423–9.

    Article  PubMed  Google Scholar 

  18. Santos S, Peinado VI, Ramirez J, Melgosa T, Roca J, Rodriguez-Roisin R, et al. Characterization of pulmonary vascular remodelling in smokers and patients with mild COPD. Eur Respir J. 2002;19:632–8.

    Article  CAS  PubMed  Google Scholar 

  19. Dinh-Xuan AT, Higenbottam TW, Clelland CA, Pepke-Zaba J, Cremona G, Butt AY, et al. Impairment of endothelium-dependent pulmonary-artery relaxation in chronic obstructive lung disease. N Engl J Med. 1991;324:1539–47.

    Article  CAS  PubMed  Google Scholar 

  20. Barberà JA, Peinado VI, Santos S, Ramirez J, Roca J, Rodriguez-Roisin R. Reduced expression of endothelial nitric oxide synthase in pulmonary arteries of smokers. Am J Respir Crit Care Med. 2001;164:709–13.

    Article  PubMed  Google Scholar 

  21. Nana-Sinkam SP, Lee JD, Sotto-Santiago S, Stearman RS, Keith RL, Choudhury Q, et al. Prostacyclin prevents pulmonary endothelial cell apoptosis induced by cigarette smoke. Am J Respir Crit Care Med. 2007;175:676–85.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  22. Santos S, Peinado VI, Ramirez J, Morales-Blanhir J, Bastos R, Roca J, et al. Enhanced expression of vascular endothelial growth factor in pulmonary arteries of smokers and patients with moderate chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 2003;167:1250–6.

    Article  PubMed  Google Scholar 

  23. Weitzenblum E, Sautegeau A, Ehrhart M, Mammosser M, Pelletier A. Long-term oxygen therapy can reverse the progression of pulmonary hypertension in patients with chronic obstructive pulmonary disease. Am Rev Respir Dis. 1985;131:493–8.

    CAS  PubMed  Google Scholar 

  24. MacNee W, Wathen CG, Flenley DC, Muir AD. The effects of controlled oxygen therapy on ventricular function in patients with stable and decompensated cor pulmonale. Am Rev Respir Dis. 1988;137:1289–95.

    Article  CAS  PubMed  Google Scholar 

  25. Peinado VI, Barberà JA, Abate P, Ramirez J, Roca J, Santos S, et al. Inflammatory reaction in pulmonary muscular arteries of patients with mild chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 1999;159:1605–11.

    Article  CAS  PubMed  Google Scholar 

  26. Llinas L, Peinado VI, Ramon GJ, Rabinovich R, Pizarro S, Rodriguez-Roisin R, et al. Similar gene expression profiles in smokers and patients with moderate COPD. Pulm Pharmacol Ther. 2011;24:32–41.

    Article  CAS  PubMed  Google Scholar 

  27. Oswald-Mammosser M, Oswald T, Nyankiye E, Dickele MC, Grange D, Weitzenblum E. Non-invasive diagnosis of pulmonary hypertension in chronic obstructive pulmonary disease. Comparison of ECG, radiological measurements, echocardiography and myocardial scintigraphy. Eur J Respir Dis. 1987;71:419–29.

    CAS  PubMed  Google Scholar 

  28. Naeije R, Torbicki A. More on the noninvasive diagnosis of pulmonary hypertension: Doppler echocardiography revisited. Eur Respir J. 1995;8:1445–9.

    CAS  PubMed  Google Scholar 

  29. Arcasoy SM, Christie JD, Ferrari VA, Sutton MS, Zisman DA, Blumenthal NP, et al. Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease. Am J Respir Crit Care Med. 2003;167:735–40.

    Article  PubMed  Google Scholar 

  30. Fisher MR, Criner GJ, Fishman AP, Hassoun PM, Minai OA, Scharf SM, et al. Estimating pulmonary artery pressures by echocardiography in patients with emphysema. Eur Respir J. 2007;30:914–21.

    Article  CAS  PubMed  Google Scholar 

  31. Turhan S, Dincer I, Ozdol C, Rahimov U, Kilickap M, Altin T, et al. Value of tissue Doppler myocardial velocities of tricuspid lateral annulus for the diagnosis of right heart failure in patients with COPD. Echocardiography. 2007;24:126–33.

    Article  PubMed  Google Scholar 

  32. Takakura M, Harada T, Fukuno H, Okushi H, Taniguchi T, Sawada S, et al. Echocardiographic detection of occult cor pulmonale during exercise in patients with chronic obstructive pulmonary disease. Echocardiography. 1999;16:127–34.

    Article  PubMed  Google Scholar 

  33. Burrows B, Kettel LJ, Niden AH, Rabinowitz M, Diener CF. Patterns of cardiovascular dysfunction in chronic obstructive lung disease. N Engl J Med. 1972;286:912–8.

    Article  CAS  PubMed  Google Scholar 

  34. Long term domiciliary oxygen therapy in chronic hypoxic cor pulmonale complicating chronic bronchitis and emphysema. Report of the Medical Research Council Working Party. Lancet. 1981;28(1):(8222):681–6.

  35. Nocturnal oxygen therapy trial group. Continous or nocturnal oxygen therapy in hypoxemic chronic obstructive lung disease. A clinical trial. Ann Intern Med. 1980;93(3):391–98.

  36. Agusti AG, Barberà JA, Roca J, Wagner PD, Guitart R, Rodriguez-Roisin R. Hypoxic pulmonary vasoconstriction and gas exchange during exercise in chronic obstructive pulmonary disease. Chest. 1990;97:268–75.

    Article  CAS  PubMed  Google Scholar 

  37. Simonneau G, Escourrou P, Duroux P, Lockhart A. Inhibition of hypoxic pulmonary vasoconstriction by nifedipine. N Engl J Med. 1981;304:1582–5.

    Article  CAS  PubMed  Google Scholar 

  38. Blanco I, Gimeno E, Munoz PA, Pizarro S, Rodriguez-Roisin R, Roca J, et al. Hemodynamic and gas exchange effects of sildenafil in patients with COPD and pulmonary hypertension. Am J Respir Crit Care Med. 2010;181:270–8. Study showing the acute effects of sildenafil in COPD patients.

    Article  CAS  PubMed  Google Scholar 

  39. Roger N, Barberà JA, Roca J, Rovira I, Gomez FP, Rodriguez-Roisin R. Nitric oxide inhalation during exercise in chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 1997;156:800–6.

    Article  CAS  PubMed  Google Scholar 

  40. Barberà JA, Roger N, Roca J, Rovira I, Higenbottam TW, Rodriguez-Roisin R. Worsening of pulmonary gas exchange with nitric oxide inhalation in chronic obstructive pulmonary disease. Lancet. 1996;347:436–40.

    Article  PubMed  Google Scholar 

  41. Vonbank K, Ziesche R, Higenbottam TW, Stiebellehner L, Petkov V, Schenk P, et al. Controlled prospective randomised trial on the effects on pulmonary haemodynamics of the ambulatory long term use of nitric oxide and oxygen in patients with severe COPD. Thorax. 2003;58:289–93.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  42. Naeije R, Barbera JA. Pulmonary hypertension associated with COPD. Crit Care. 2001;5:286–9.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  43. Stevens D, Sharma K, Szidon P, Rich S, McLaughlin V, Kesten S. Severe pulmonary hypertension associated with COPD. Ann Transplant. 2000;5:8–12.

    CAS  PubMed  Google Scholar 

  44. Archer SL, Mike D, Crow J, Long W, Weir EK. A placebo-controlled trial of prostacyclin in acute respiratory failure in COPD. Chest. 1996;109:750–5.

    Article  CAS  PubMed  Google Scholar 

  45. Dernaika TA, Beavin M, Kinasewitz GT. Iloprost improves gas exchange and exercise tolerance in patients with pulmonary hypertension and chronic obstructive pulmonary disease. Respiration. 2010;79:377–82.

    Article  CAS  PubMed  Google Scholar 

  46. Boeck L, Tamm M, Grendelmeier P, Stolz D. Acute effects of aerosolized iloprost in COPD related pulmonary hypertension - a randomized controlled crossover trial. PLoS One. 2012;7:e52248.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  47. Stolz D, Rasch H, Linka A, Valentino MD, Meyer A, Brutsche M, et al. A randomized, controlled trial of bosentan in severe COPD. Eur Respir J. 2008;32:619–28.

    Article  CAS  PubMed  Google Scholar 

  48. Valerio G, Bracciale P, Grazia DA. Effect of bosentan upon pulmonary hypertension in chronic obstructive pulmonary disease. Ther Adv Respir Dis. 2009;3:15–21.

    Article  PubMed  Google Scholar 

  49. Rao RS, Singh S, Sharma BB, Agarwal VV, Singh V. Sildenafil improves six-minute walk distance in chronic obstructive pulmonary disease: a randomised, double-blind, placebo-controlled trial. Indian J Chest Dis Allied Sci. 2011;53:81–5.

    PubMed  Google Scholar 

  50. Lederer DJ, Bartels MN, Schluger NW, Brogan F, Jellen P, Thomashow BM, et al. Sildenafil for chronic obstructive pulmonary disease: a randomized crossover trial. COPD. 2012;9:268–75.

    Article  PubMed  Google Scholar 

  51. Blanco I, Santos S, Gea J, Guell R, Torres F, Gimeno-Santos E, et al. Sildenafil to improve respiratory rehabilitation outcomes in COPD: a controlled trial. Eur Respir J. 2013;42:982–92. Randomized clinical trial evaluating the long-term effects of sildenafil combined with pulmonary rehabilitation in COPD patients.

    Article  CAS  PubMed  Google Scholar 

  52. Goudie AR, Lipworth BJ, Hopkinson PJ, Wei L, Struthers AD. Tadalafil in patients with chronic obstructive pulmonary disease: a randomised, double-blind, parallel-group, placebo-controlled trial. Lancet Respir Med. 2014;2:293–300. Very recent randomized clinical trial showing that treatment with tadalafil did not improve exercise capacity and quality of life in selected patients with COPD and mild pulmonary hypertension.

    Article  CAS  PubMed  Google Scholar 

  53. Corte TJ, Keir GJ, Dimopoulos K, Howard L, Corris PA, Parfitt L, et al. Bosentan in pulmonary hypertension associated with fibrotic idiopathic interstitial pneumonia. Am J Respir Crit Care Med. 2014;190:208–17. First RCT not supporting the use of bosentan in patients with pulmonary hypertension complicating interstitial lung disease.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  54. Idiopathic Pulmonary Fibrosis Clinical Research Network, Zisman DA, Schwarz M, Anstrom KJ, Collard HR, Flaherty KR, Hunninghake GW. A Controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. N Engl J Med. 2010;363:620–8.

  55. American Thoracic Society, European Respiratory Society. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. 2002;165:277–304.

  56. Lettieri CJ, Nathan SD, Barnett SD, Ahmad S, Shorr AF. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest. 2006;129:746–52.

    Article  PubMed  Google Scholar 

  57. Nathan SD, Shlobin OA, Barnett SD, Saggar R, Belperio JA, Ross DJ, et al. Right ventricular systolic pressure by echocardiography as a predictor of pulmonary hypertension in idiopathic pulmonary fibrosis. Respir Med. 2008;102:1305–10.

    Article  PubMed Central  PubMed  Google Scholar 

  58. Shorr AF, Wainright JL, Cors CS, Lettieri CJ, Nathan SD. Pulmonary hypertension in patients with pulmonary fibrosis awaiting lung transplant. Eur Respir J. 2007;30:715–21.

    Article  CAS  PubMed  Google Scholar 

  59. Zisman DA, Ross DJ, Belperio JA, Saggar R, Lynch III JP, Ardehali A, et al. Prediction of pulmonary hypertension in idiopathic pulmonary fibrosis. Respir Med. 2007;101:2153–9.

    Article  PubMed Central  PubMed  Google Scholar 

  60. Cottin V, Nunes H, Brillet PY, Delaval P, Devouassoux G, Tillie-Leblond I, et al. Court-Fortune, Valeyre D, Cordier JF. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J. 2005;26:586–93.

    Article  CAS  PubMed  Google Scholar 

  61. Mejia M, Carrillo G, Rojas-Serrano J, Estrada A, Suarez T, Alonso D, et al. Idiopathic pulmonary fibrosis and emphysema: decreased survival associated with severe pulmonary arterial hypertension. Chest. 2009;136:10–5.

    Article  PubMed  Google Scholar 

  62. Cottin V, Le PJ, Prevot G, Mal H, Humbert M, Simonneau G, et al. Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome. Eur Respir J. 2010;35:105–11.

    Article  CAS  PubMed  Google Scholar 

  63. Nadrous HF, Pellikka PA, Krowka MJ, Swanson KL, Chaowalit N, Decker PA, et al. Pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Chest. 2005;128:2393–9.

    Article  PubMed  Google Scholar 

  64. Martinez FJ, Safrin S, Weycker D, Starko KM, Bradford WZ, King Jr TE, et al. The clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med. 2005;142:963–7.

    Article  PubMed  Google Scholar 

  65. Ebina M, Shimizukawa M, Shibata N, Kimura Y, Suzuki T, Endo M, et al. Heterogeneous increase in CD34-positive alveolar capillaries in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2004;169:1203–8.

    Article  PubMed  Google Scholar 

  66. Weitzenblum E, Ehrhart M, Rasaholinjanahary J, Hirth C. Pulmonary hemodynamics in idiopathic pulmonary fibrosis and other interstitial pulmonary diseases. Respiration. 1983;44:118–27.

    Article  CAS  PubMed  Google Scholar 

  67. Giaid A, Michel RP, Stewart DJ, Sheppard M, Corrin B, Hamid Q. Expression of endothelin-1 in lungs of patients with cryptogenic fibrosing alveolitis. Lancet. 1993;341:1550–4.

    Article  CAS  PubMed  Google Scholar 

  68. Simler NR, Brenchley PE, Horrocks AW, Greaves SM, Hasleton PS, Egan JJ. Angiogenic cytokines in patients with idiopathic interstitial pneumonia. Thorax. 2004;59:581–5.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  69. Nathan SD, Shlobin OA, Ahmad S, Koch J, Barnett SD, Ad N, et al. Serial development of pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Respiration. 2008;76:288–94.

    Article  CAS  PubMed  Google Scholar 

  70. Agusti AG, Roca J, Gea J, Wagner PD, Xaubet A, Rodriguez-Roisin R. Mechanisms of gas-exchange impairment in idiopathic pulmonary fibrosis. Am Rev Respir Dis. 1991;143:219–25.

    Article  CAS  PubMed  Google Scholar 

  71. Bonay M, Bancal C, de Zuttere D, Arnoult F, Saumon G, Camus F. Normal pulmonary capillary blood volume in patients with chronic infiltrative lung disease and high pulmonary artery pressure. Chest. 2004;126:1460–6.

    Article  PubMed  Google Scholar 

  72. Kennedy JI, Fulmer JD. Pulmonary hypertension in the interstitial lung diseases. Chest. 1985;87:558–60.

    Article  CAS  PubMed  Google Scholar 

  73. Blanco I, Ribas J, Xaubet A, Gomez FP, Roca J, Rodriguez-Roisin R, et al. Effects of inhaled nitric oxide at rest and during exercise in idiopathic pulmonary fibrosis. J Appl Physiol (1985). 2011;110:638–45.

    Article  CAS  Google Scholar 

  74. Ghofrani HA, Wiedemann R, Rose F, Schermuly RT, Olschewski H, Weissmann N, et al. Sildenafil for treatment of lung fibrosis and pulmonary hypertension: a randomised controlled trial. Lancet. 2002;360:895–900.

    Article  CAS  PubMed  Google Scholar 

  75. Olschewski H, Ghofrani HA, Walmrath D, Schermuly R, Temmesfeld-Wollbruck B, Grimminger F, et al. Inhaled prostacyclin and iloprost in severe pulmonary hypertension secondary to lung fibrosis. Am J Respir Crit Care Med. 1999;160:600–7.

    Article  CAS  PubMed  Google Scholar 

  76. Gunther A, Enke B, Markart P, Hammerl P, Morr H, Behr J, et al. Safety and tolerability of bosentan in idiopathic pulmonary fibrosis: an open label study. Eur Respir J. 2007;29:713–9.

    Article  CAS  PubMed  Google Scholar 

  77. King Jr TE, Behr J, Brown KK, du Bois RM, Lancaster L, de Andrade JA, et al. BUILD-1: a randomized placebo-controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2008;177:75–81.

    Article  CAS  PubMed  Google Scholar 

  78. Seibold JR, Denton CP, Furst DE, Guillevin L, Rubin LJ, Wells A, et al. Randomized, prospective, placebo-controlled trial of bosentan in interstitial lung disease secondary to systemic sclerosis. Arthritis Rheum. 2010;62:2101–8.

    Article  CAS  PubMed  Google Scholar 

  79. King Jr TE, Brown KK, Raghu G, du Bois RM, Lynch DA, Martinez F, et al. BUILD-3: a randomized, controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;184:92–9. Negative study where a big cohort of IPF patients were treated with bosentan versus placebo. Despite the treatment, disease progresses.

    Article  PubMed  Google Scholar 

  80. Raghu G, Million-Rousseau R, Morganti A, Perchenet L, Behr J. Macitentan for the treatment of idiopathic pulmonary fibrosis: the randomised controlled MUSIC trial. Eur Respir J. 2013;42:1622–32. Randomized clinical trial evaluating long-term effects of a new endothelin receptor antagonist (macitentan) in IPF.

    Article  CAS  PubMed  Google Scholar 

  81. Raghu G, Behr J, Brown KK, Egan JJ, Kawut SM, Flaherty KR, et al. Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomized trial. Ann Intern Med. 2013;158:641–9. Prematurely terminated study in IPF patients treated with ambrisentan because an interim analysis indicated a low likelihood of showing efficacy and patients experimented disease progression.

    Article  PubMed  Google Scholar 

  82. Han MK, Bach DS, Hagan PG, Yow E, Flaherty KR, Toews GB, et al. Sildenafil preserves exercise capacity in patients with idiopathic pulmonary fibrosis and right-sided ventricular dysfunction. Chest. 2013;143:1699–708.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  83. Collard HR, Anstrom KJ, Schwarz MI, Zisman DA. Sildenafil improves walk distance in idiopathic pulmonary fibrosis. Chest. 2007;131:897–9.

    Article  PubMed Central  PubMed  Google Scholar 

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Conflict of Interest

Joan Albert Barberà reports grants and personal fees from Actelion, Bayer, GlaxoSmithKline, and Pfizer. Isabel Blanco declares grants and personal fees from Actelion and Bayer.

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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  1. Department of Pulmonary Medicine, Hospital Clínic and August Pi i Sunyer Biomedical Research Institute (IDIBAPS), University of Barcelona, Barcelona, Spain

    Joan Albert Barberà & Isabel Blanco

  2. Biomedical Research Networking Center in Respiratory Diseases (CIBERES), Madrid, Spain

    Joan Albert Barberà & Isabel Blanco

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  1. Joan Albert Barberà
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Correspondence to Joan Albert Barberà.

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This article is part of the Topical Collection on Pulmonary Hypertension

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Barberà, J.A., Blanco, I. Management of Pulmonary Hypertension in Patients with Chronic Lung Disease. Curr Hypertens Rep 17, 62 (2015). https://doi.org/10.1007/s11906-015-0574-9

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