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Management of Pulmonary Hypertension Due to Chronic Lung Disease Cover

Management of Pulmonary Hypertension Due to Chronic Lung Disease

Open Access
|Jul 2021

References

  1. Galiè N, Humbert M, Vachiery J-L, 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): 90375. doi: 10.1183/13993003.01032-2015.
  2. Simmoneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. 2019 Jan 24; 53(1): 1801913. doi: 10.1183/13993003.01913-2018.
  3. Nathan SD, Barbera JA, Gaine SP, et al. Pulmonary hypertension in chronic lung disease and hypoxia. Eur Respir J. 2019 Jan 24; 53(1): 1801914. doi: 10.1183/13993003.01914-2018.
  4. Seeger W, Adir Y, Barberà JA, Champion H, et al. Pulmonary hypertension in chronic lung diseases. J Am Coll Cardiol. 2013 Dec 24; 62(25 Suppl): D10916. doi: 10.1016/j.jacc.2013.10.036.
  5. Sakao S, Voelkel NF, Tatsumi K. The vascular bed in COPD: pulmonary hypertension and pulmonary vascular alterations. Eur Respir Rev. 2014 Sep; 23(133): 3505. doi: 10.1183/09059180.00007913.
  6. Ruffenach G, Hong J, Vaillancourt M, Medzikovic L, Eghbali M. Pulmonary hypertension secondary to pulmonary fibrosis: clinical data, histopathology and molecular insights. Respir Res. 2020 Nov 18;21(1): 303. doi: 10.1186/s12931-020-01570-2.
  7. Prins KW, Duval S, Markowitz J, Pritzker M, Thenappan T. Chronic use of PAH-specific therapy in World Health Organization Group III Pulmonary Hypertension: a systematic review and meta-analysis. Pulm Circ. 2017 Mar 24; 7(1): 145155. doi: 10.1086/690017.
  8. Vogelmeier CF, Criner GJ, Martinez FJ, et al. Global Strategy for the Diagnosis, Management, and Prevention of Chronic Obstructive Lung Disease 2017 Report. GOLD Executive Summary. Am J Respir Crit Care Med. 2017 Mar 1; 195(5): 557582. doi: 10.1164/rccm.201701-0218PP.
  9. Chaouat A, Naeije R, Weitzenblum E. Pulmonary hypertension in COPD. Eur Respir J. 2008 Nov; 32(5): 137185. doi: 10.1183/09031936.00015608.
  10. Wells MJ, Iyer AS, Rahaghi FN, et al. Pulmonary artery enlargement is associated with right ventricular dysfunction and loss of blood volume in small pulmonary vessels in chronic obstructive pulmonary disease. Circ Cardiovasc Imaging. 2015 Apr; 8(4): 10.1161/CIRCIMAGING.114.002546 e002546. doi: 10.1161/CIRCIMAGING.114.002546.
  11. Poor HD, Kawut SM, Chia-Ying Liu, et al. Pulmonary hyperinflation due to gas trapping and pulmonary artery size: The MESA COPD Study. PLoS One. 2017 May 2; 12(5): e0176812.
  12. Continuous or nocturnal oxygen therapy in hypoxemic chronic obstructive lung disease: a clinical trial. Nocturnal Oxygen Therapy Trial Group. Ann Intern Med. 1980 Sep; 93(3): 3918. doi: 10.7326/0003-4819-93-3-391.
  13. Weill D, Benden C, Corris PA, et al. A consensus document for the selection of lung transplant candidates: 2014--an update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant. 2015 Jan; 34(1): 115. doi: 10.1016/j.healun.2014.06.014.
  14. Kovacs G, Agusti A, Barberà JA, et al. Pulmonary Vascular Involvement in Chronic Obstructive Pulmonary Disease. Is There a Pulmonary Vascular Phenotype? Am J Respir Crit Care Med. 2018 Oct 15; 198(8): 10001011. doi: 10.1164/rccm.201801-0095PP.
  15. Weatherald J, Montani D, Humbert M. Seeing the Forest for the (Arterial) Tree: Vascular Pruning and the Chronic Obstructive Pulmonary Disease Pulmonary Vascular Phenotype. Am J Respir Crit Care Med. 2019 Aug 15; 200(4): 406408. doi: 10.1164/rccm.201901-0248ED.
  16. Boerrigter BG, Bogaard HJ, Trip P, et al. Ventilatory and cardiocirculatory exercise profiles in COPD: the role of pulmonary hypertension. Chest. 2012 Nov; 142(5): 116674. doi: 10.1378/chest.11-2798.
  17. 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. Apr-Jun 2011; 53(2): 815.
  18. Lederer DJ, Bartels MN, Schluger NW, et al. Sildenafil for chronic obstructive pulmonary disease: a randomized crossover trial. COPD. 2012 Jun; 9(3): 26875. doi: 10.3109/15412555.2011.651180.
  19. Blanco I, Santos S, Gea J, et al. Sildenafil to improve respiratory rehabilitation outcomes in COPD: a controlled trial. 2013 Oct; 42(4): 98292. doi: 10.1183/09031936.00176312.
  20. 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 Apr; 2(4): 293300. doi: 10.1016/S2213-2600(14)70013-X.
  21. Vitulo P, Stanziola A, Confalonieri M, et al. Sildenafil in severe pulmonary hypertension associated with chronic obstructive pulmonary disease: A randomized controlled multicenter clinical trial. J Heart Lung Transplant. 2017 Feb; 36(2): 166174. doi: 10.1016/j.healun.2016.04.010.
  22. Hao Y, Zhu Y, Mao Y, et al. Efficacy and safety of Sildenafil treatment in pulmonary hypertension caused by chronic obstructive pulmonary disease: A meta-analysis. Life Sci. 2020 Sep 15; 257: 118001. doi: 10.1016/j.lfs.2020.118001.
  23. Stolz D, Rasch H, Linka A, et al. A randomised, controlled trial of bosentan in severe COPD. Eur Respir J. 2008 Sep; 32(3): 61928. doi: 10.1183/09031936.00011308.
  24. Valerio G, Bracciale P, Grazia D’Agostino A. Effect of bosentan upon pulmonary hypertension in chronic obstructive pulmonary disease. Ther Adv Respir Dis. 2009 Feb; 3(1): 1521. doi: 10.1177/1753465808103499.
  25. Katayama Y, Higenbottam TW, Diaz de Atauri MJ, et al. Inhaled nitric oxide and arterial oxygen tension in patients with chronic obstructive pulmonary disease and severe pulmonary hypertension. Thorax. 1997 Feb; 52(2): 1204. doi: 10.1136/thx.52.2.120.
  26. 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 Feb 17; 347(8999): 43640. doi: 10.1016/s0140-6736(96)90011-2.
  27. Phillips DB, Brotto AR, Ross BA, et al.; Canadian Respiratory Research Network. Inhaled nitric oxide improves ventilatory efficiency and exercise capacity in patients with mild COPD: A randomized-control cross-over trial. J Physiol. 2021 Mar; 599(5): 16651683. doi: 10.1113/JP280913.
  28. Hajian B, De Backer J, Vos W, et al. Pulmonary vascular effects of pulsed inhaled nitric oxide in COPD patients with pulmonary hypertension. Int J Chron Obstruct Pulmon Dis. 2016 Jul 5; 11: 153341. doi: 10.2147/COPD.S106480.
  29. Wang L, Jin YZ, Zhao QH, et al. Hemodynamic and gas exchange effects of inhaled iloprost in patients with COPD and pulmonary hypertension. Int J Chron Obstruct Pulmon Dis. 2017 Nov 22; 12: 33533360. doi: 10.2147/COPD.S141798.
  30. 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(5): 37782. doi: 10.1159/000242498.
  31. Sprunger DB, Olson AL, Huie TJ, et al. Pulmonary fibrosis is associated with an elevated risk of thromboembolic disease. Eur Respir J. 2012 Jan; 39(1): 12532. doi: 10.1183/09031936.00041411.
  32. Lettieri CJ, Nathan SD, Barnett SD, Ahmad S, Shorr AF. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest. 2006 Mar; 129(3): 74652. doi: 10.1378/chest.129.3.746.
  33. Lancaster LH, Mason WR, Parnell JA, et al. Obstructive sleep apnea is common in idiopathic pulmonary fibrosis. 2009 Sep; 136(3): 7728. doi: 10.1378/chest.08-2776.
  34. Ghigna MR, Mooi WJ, Grünberg K. Pulmonary hypertensive vasculopathy in parenchymal lung diseases and/or hypoxia: Number 1 in the Series “Pathology for the clinician” Edited by Peter Dorfmüller and Alberto Cavazza. Eur Respir Rev. 2017 Jun 28; 26(144): 170003. doi: 10.1183/16000617.0003-2017.
  35. King TE, Behr J, Brown KK, et al. BUILD-1: a randomized placebo-controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2008 Jan 1; 177(1): 7581. doi: 10.1164/rccm.200705-732OC.
  36. King TE, Brown KK, Raghu G, et al. BUILD-3: a randomized, controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011 Jul 1; 184(1): 929. doi: 10.1164/rccm.201011-1874OC.
  37. Raghu G, Behr J, Brown KK, et al. Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomized trial. Ann Intern Med. 2013 May 7; 158(9): 6419. doi: 10.7326/0003-4819-158-9-201305070-00003.
  38. Raghu G, Million-Rousseau R, Morganti A, Perchenet L, Behr J, MUSIC Study Group. Macitentan for the treatment of idiopathic pulmonary fibrosis: the randomised controlled MUSIC trial. Eur Respir J. 2013 Dec; 42(6): 162232. doi: 10.1183/09031936.00104612.
  39. Nathan SD, Behr J, Collard HR, et al. Riociguat for idiopathic interstitial pneumonia-associated pulmonary hypertension (RISE-IIP): a randomised, placebo-controlled phase 2b study. Lancet Respir Med. 2019 Sep; 7(9): 780790. doi: 10.1016/S2213-2600(19)30250-4.
  40. Zisman DA, Schwarz M, Anstrom KJ, Collard HR, Flaherty KR. Idiopathic Pulmonary Fibrosis Clinical Research Network. A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. 2010 Aug 12; 363(7): 6208. doi: 10.1056/NEJMoa1002110.
  41. Han MK, Bach DS, Hagan PG, et al. Sildenafil preserves exercise capacity in patients with idiopathic pulmonary fibrosis and right-sided ventricular dysfunction. Chest. 2013 Jun; 143(6): 16991708. doi: 10.1378/chest.12-1594.
  42. Behr J, Nathan SD, Wuyts WA, et al. Efficacy and safety of sildenafil added to pirfenidone in patients with advanced idiopathic pulmonary fibrosis and risk of pulmonary hypertension: a double-blind, randomised, placebo-controlled, phase 2b trial. Lancet Respir Med. 2021 Jan; 9(1): 8595. doi: 10.1016/S2213-2600(20)30356-8.
  43. Nathan SD, Flaherty KR, Glassberg MK, et al. A Randomized, Double-Blind, Placebo-Controlled Study of Pulsed, Inhaled Nitric Oxide in Subjects at Risk of Pulmonary Hypertension Associated With Pulmonary Fibrosis. Chest. 2020 Aug; 158(2): 637645. doi: 10.1016/j.chest.2020.02.016.
  44. Lefèvre G, Dauchet L, Hachulla E, et al. Survival and prognostic factors in systemic sclerosis-associated pulmonary hypertension: a systematic review and meta-analysis. Arthritis Rheum. 2013 Sep; 65(9): 241223. doi: 10.1002/art.38029.
  45. Le Pavec J, Girgis RE, Lechtzin N, et al. Systemic sclerosis-related pulmonary hypertension associated with interstitial lung disease: impact of pulmonary arterial hypertension therapies. Arthritis Rheum. 2011 Aug; 63(8): 245664. doi: 10.1002/art.30423.
  46. Chauvelot L, Gamondes D, Berthiller J, et al. Hemodynamic Response to Treatment and Outcomes in Pulmonary Hypertension Associated With Interstitial Lung Disease Versus Pulmonary Arterial Hypertension in Systemic Sclerosis: Data From a Study Identifying Prognostic Factors in Pulmonary Hypertension Associated With Interstitial Lung Disease. Arthritis Rheum. 2021 Feb; 73(2): 295304. doi: 10.1002/art.41512.
  47. Waxman A, Restrepo-Jaramillo R, Thenappan T, et al. Inhaled Treprostinil in Pulmonary Hypertension Due to Interstitial Lung Disease. N Engl J Med. 2021 Jan 28; 384(4): 325334. doi: 10.1056/NEJMoa2008470.
  48. Raghu G, Rochwerg B, Zhang Y, et al. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. An Update of the 2011 Clinical Practice Guideline. Am J Respir Crit Care Med. 2015 Jul 15; 192(2): e319. doi: 10.1164/rccm.201506-1063ST.
  49. Vis D, Boucly A, Humbert M, Weatherald J. Which patients are SaPHe in sarcoidosis-associated pulmonary hypertension? Eur Respir J. 2020 May 14; 55(5): 2000700. doi: 10.1183/13993003.00700-2020.
  50. Nunes H, Humbert M, Capron F, et al. Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis. Thorax. 2006 Jan; 61(1): 6874. doi: 10.1136/thx.2005.042838.
  51. Baughman RP, Engel PJ, Taylor L, Lower EE. Survival in sarcoidosis-associated pulmonary hypertension: the importance of hemodynamic evaluation. Chest. 2010 Nov; 138(5): 107885. doi: 10.1378/chest.09-2002.
  52. Boucly A, Cottin V, Nunes H, et al. Management and long-term outcomes of sarcoidosis-associated pulmonary hypertension. Eur Respir J. 2017 Oct 19; 50(4): 1700465. doi: 10.1183/13993003.00465-2017.
  53. Bandyopadhyay D, Humbert M. An update on sarcoidosis-associated pulmonary hypertension. Curr Opin Pulm Med. 2020 Sep; 26(5): 582590. doi: 10.1097/MCP.0000000000000701.
  54. Baughman RP, Culver DA, Cordova FC, et al. Bosentan for sarcoidosis-associated pulmonary hypertension: a double-blind placebo controlled randomized trial. Chest. 2014 Apr; 145(4): 810817. doi: 10.1378/chest.13-1766.
  55. Barnett CF, Bonura EJ, Nathan SD, et al. Treatment of sarcoidosis-associated pulmonary hypertension. A two-center experience. Chest. 2009 Jun; 135(6): 14551461. doi: 10.1378/chest.08-1881.
  56. Bonham CA, Oldham JM, Gomberg-Maitland M, Vij R. Prostacyclin and oral vasodilator therapy in sarcoidosis-associated pulmonary hypertension: a retrospective case series. Chest. 2015 Oct; 148(4): 105562. doi: 10.1378/chest.14-2546.
  57. Shlobin OA, Kouranos V, Barnett SD, et al. Physiological predictors of survival in patients with sarcoidosis-associated pulmonary hypertension: results from an international registry. Eur Respir J. 2020; 55(5): 1901747.
  58. Condado JF, Babaliaros V, Henry TS, Kaebnick B, Kim D, Staton GW Jr. Pulmonary stenting for the treatment of sarcoid induced pulmonary vascular stenosis. Sarcoidosis Vasc Diffuse Lung Dis. 2016 Oct 7; 33(3): 2817.
  59. DaSilva-deAbreu A, Bracamonte-Baran W, Condado JF, Babaliaros V, Tafur-Soto J, Mandras SA. Characteristics and outcomes of pulmonary angioplasty with or without stenting for sarcoidosis-associated pulmonary hypertension: systematic review and individual participant data meta-analysis. Curr Probl Cardiol. 2020; doi: 10.1016/j.cpcardiol.2020.100616.
  60. Fartoukh M, Humbert M, Capron F, et al. Severe pulmonary hypertension in histiocytosis X. 2000 Jan; 161(1): 21623. doi: 10.1164/ajrccm.161.1.9807024.
  61. Lorillon G, Bergeron A, Detourmignies L, et al. Cladribine is effective against cystic pulmonary Langerhans cell histiocytosis. Am J Respir Crit Care Med. 2012 Nov 1; 186(9): 9302. doi: 10.1164/ajrccm.186.9.930.
  62. Le Pavec J, Lorillon G, Jaïs X, et al. Pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension: clinical characteristics and impact of pulmonary arterial hypertension therapies. Chest. 2012 Nov; 142(5): 11501157. doi: 10.1378/chest.11-2490.
  63. McCormack FX, Gupta N, Finlay GR, et al. Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management. Am J Respir Crit Care Med. 2016 Sep 15; 194(6): 74861. doi: 10.1164/rccm.201607-1384ST.
  64. Reynaud-Gaubert M, Mornex J-F, Mal H, et al. Lung transplantation for lymphangioleiomyomatosis: the French experience. Transplantation. 2008 Aug 27; 86(4): 51520. doi: 10.1097/TP.0b013e31817c15df.
  65. Cottin V, Harari S, Humbert M, et al. Pulmonary hypertension in lymphangioleiomyomatosis: characteristics in 20 patients. Eur Respir J. 2012 Sep; 40(3): 63040. doi: 10.1183/09031936.00093111.
DOI: https://doi.org/10.14797/ZKUT3813 | Journal eISSN: 1947-6108
Language: English
Page range: 124 - 133
Accepted on: Dec 12, 2021
Published on: Jul 1, 2021
Published by: Houston Methodist DeBakey Heart & Vascular Center
In partnership with: Paradigm Publishing Services

© 2021 Jordan Sugarman, Jason Weatherald, published by Houston Methodist DeBakey Heart & Vascular Center
This work is licensed under the Creative Commons Attribution-NonCommercial 4.0 License.