•• Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RMF, Brida M, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;43(38):3618–731. https://doi.org/10.1093/eurheartj/ehac237.

Wijeratne DT, Lajkosz K, Brogly SB, Lougheed MD, Jiang L, Housin A, et al. Increasing incidence and prevalence of world health organization groups 1 to 4 pulmonary hypertension: a population-based cohort study in Ontario, Canada. Circ Cardiovasc Qual Outcomes. 2018;11(2):e003973. https://doi.org/10.1161/circoutcomes.117.003973.

Prins KW, Rose L, Archer SL, Pritzker M, Weir EK, Kazmirczak F, et al. Disproportionate right ventricular dysfunction and poor survival in group 3 pulmonary hypertension. Am J Respir Crit Care Med. 2018;197(11):1496–9. https://doi.org/10.1164/rccm.201712-2405LE.

Article  PubMed  PubMed Central  Google Scholar 

Mejía M, Carrillo G, Rojas-Serrano J, Estrada A, Suárez T, Alonso D, et al. Idiopathic pulmonary fibrosis and emphysema: decreased survival associated with severe pulmonary arterial hypertension. Chest. 2009;136(1):10–5. https://doi.org/10.1378/chest.08-2306.

Article  PubMed  Google Scholar 

Takahashi K, Taniguchi H, Ando M, Sakamoto K, Kondoh Y, Watanabe N, et al. Mean pulmonary arterial pressure as a prognostic indicator in connective tissue disease associated with interstitial lung disease: a retrospective cohort study. BMC Pulm Med. 2016;16(1):55. https://doi.org/10.1186/s12890-016-0207-3.

Article  PubMed  PubMed Central  Google Scholar 

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(3):746–52. https://doi.org/10.1378/chest.129.3.746.

Article  PubMed  Google Scholar 

DuBrock HM, Nathan SD, Reeve BB, Kolaitis NA, Mathai SC, Classi PM, et al. Pulmonary hypertension due to interstitial lung disease or chronic obstructive pulmonary disease: a patient experience study of symptoms and their impact on quality of life. Pulmonary circulation. 2021;11(2):20458940211005640. https://doi.org/10.1177/20458940211005641.

Article  PubMed  PubMed Central  Google Scholar 

Judge EP, Fabre A, Adamali HI, Egan JJ. Acute exacerbations and pulmonary hypertension in advanced idiopathic pulmonary fibrosis. Eur Respir J. 2012;40(1):93–100. https://doi.org/10.1183/09031936.00115511.

Article  PubMed  Google Scholar 

Nathan SD, Barbera JA, Gaine SP, Harari S, Martinez FJ, Olschewski H, et al. Pulmonary hypertension in chronic lung disease and hypoxia. Eur Res J. 2019;53(1). https://doi.org/10.1183/13993003.01914-2018.

• Rahaghi FF, Kolaitis NA, Adegunsoye A, de Andrade JA, Flaherty KR, Lancaster LH, et al. Screening strategies for pulmonary hypertension in patients with interstitial lung disease: a multidisciplinary Delphi study. Chest. 2022;162(1):145–55. https://doi.org/10.1016/j.chest.2022.02.012.

Hoeper MM, Bogaard HJ, Condliffe R, Frantz R, Khanna D, Kurzyna M, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25 Suppl):D42–50. https://doi.org/10.1016/j.jacc.2013.10.032.

Article  PubMed  Google Scholar 

Galiè N, Hoeper MM, Humbert M, Torbicki A, Vachiery JL, Barbera JA, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2009;34(6):1219–63. https://doi.org/10.1183/09031936.00139009.

Article  PubMed  Google Scholar 

Simonneau G, Montani D, Celermajer DS, Denton CP, Gatzoulis MA, Krowka M, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Res J. 2019;53(1). https://doi.org/10.1183/13993003.01913-2018.

King CS, Shlobin OA. The trouble with group 3 pulmonary hypertension in interstitial lung disease: dilemmas in diagnosis and the conundrum of treatment. Chest. 2020;158(4):1651–64. https://doi.org/10.1016/j.chest.2020.04.046.

Article  PubMed  Google Scholar 

Continuous or nocturnal oxygen therapy in hypoxemic chronic obstructive lung disease: a clinical trial. Nocturnal Oxygen Therapy Trial Group. Ann Intern Med. 1980;93(3):391–8. https://doi.org/10.7326/0003-4819-93-3-391.

Sugarman J, Weatherald J. Management of pulmonary hypertension due to chronic lung disease. Methodist Debakey Cardiovasc J. 2021;17(2):124–33. https://doi.org/10.14797/zkut3813.

Article  PubMed  PubMed Central  Google Scholar 

Farber HW, Badesch DB, Benza RL, Elliott CG, Frantz RP, McGoon MD, et al. Use of supplemental oxygen in patients with pulmonary arterial hypertension in REVEAL. J Heart Lung Transplant. 2018;37(8):948–55. https://doi.org/10.1016/j.healun.2018.03.010.

Article  PubMed  Google Scholar 

Adir Y, Humbert M, Chaouat A. Sleep-related breathing disorders and pulmonary hypertension. Eur Respir J. 2021;57(1). https://doi.org/10.1183/13993003.02258-2020.

Galiè N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, 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;46(4):903–75. https://doi.org/10.1183/13993003.01032-2015.

Article  CAS  PubMed  Google Scholar 

Moth L, Fitzgerald C, Marino P, Osman L, Mcdonnell L, Hogg L, et al. Pulmonary rehabilitation in patients with group 3 pulmonary hypertension: evaluation of clinical outcomes, adherence and safety. Eur Res J. 2019;54(suppl 63):PA522. https://doi.org/10.1183/13993003.congress-2019.PA522.

Frost AE, Zhao C, Farber HW, Benza R, Yen J, Selej M, et al. Smoking history and pulmonary arterial hypertension: demographics, onset, and outcomes. J Heart Lung Transplant. 2023;42(3):377–89. https://doi.org/10.1016/j.healun.2022.10.007.

Article  PubMed  Google Scholar 

Oakes JM, Xu J, Morris TM, Fried ND, Pearson CS, Lobell TD, et al. Effects of chronic nicotine inhalation on systemic and pulmonary blood pressure and right ventricular remodeling in mice. Hypertension (Dallas, Tex : 1979). 2020;75(5):1305–14. https://doi.org/10.1161/hypertensionaha.119.14608.

Kopsaftis Z, Wood-Baker R, Poole P. Influenza vaccine for chronic obstructive pulmonary disease (COPD). The Cochrane database of systematic reviews. 2018;6(6):Cd002733. https://doi.org/10.1002/14651858.CD002733.pub3.

Marijic P, Schwarzkopf L, Maier W, Trudzinski F, Schwettmann L, Kreuter M. Effects of influenza vaccination in patients with interstitial lung diseases: an epidemiological claims data analysis. Ann Am Thorac Soc. 2022;19(9):1479–88. https://doi.org/10.1513/AnnalsATS.202112-1359OC.

Article  PubMed  PubMed Central  Google Scholar 

Tonelli AR, Arelli V, Minai OA, Newman J, Bair N, Heresi GA, et al. Causes and circumstances of death in pulmonary arterial hypertension. Am J Respir Crit Care Med. 2013;188(3):365–9. https://doi.org/10.1164/rccm.201209-1640OC.

Article  PubMed  PubMed Central  Google Scholar 

Nakamura J, Tsujino I, Ohira H, Nakaya T, Sugimoto A, Sato T, et al. Various factors contribute to death in patients with different types of pulmonary hypertension: a retrospective pilot study from a single tertiary center. Respir Investig. 2022;60(5):647–57. https://doi.org/10.1016/j.resinv.2022.05.001.

Article  CAS  PubMed  Google Scholar 

Wieteska-Miłek M, Szmit S, Florczyk M, Kuśmierczyk-Droszcz B, Ryczek R, Kurzyna M. COVID-19 vaccination in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: safety profile and reasons for opting against vaccination. Vaccines. 2021;9(12). https://doi.org/10.3390/vaccines9121395.

Zeder K, Avian A, Bachmaier G, Douschan P, Foris V, Sassmann T, et al. Elevated pulmonary vascular resistance predicts mortality in COPD patients. Eur Respir J. 2021;58(2). https://doi.org/10.1183/13993003.00944-2021.

Olsson KM, Hoeper MM, Pausch C, Grünig E, Huscher D, Pittrow D, et al. Pulmonary vascular resistance predicts mortality in patients with pulmonary hypertension associated with interstitial lung disease: results from the COMPERA registry. Eur  Respir J. 2021;58(2). https://doi.org/10.1183/13993003.01483-2021.

Collard HR, Anstrom KJ, Schwarz MI, Zisman DA. Sildenafil improves walk distance in idiopathic pulmonary fibrosis. Chest. 2007;131(3):897–9. https://doi.org/10.1378/chest.06-2101.

Article  PubMed  Google Scholar 

Günther 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(4):713–9. https://doi.org/10.1183/09031936.00149205.

Article  CAS  PubMed  Google Scholar 

Mittoo S, Jacob T, Craig A, Bshouty Z. Treatment of pulmonary hypertension in patients with connective tissue disease and interstitial lung disease. Can Respir J. 2010;17(6):282–6. https://doi.org/10.1155/2010/686098.

Article  PubMed  PubMed Central  Google Scholar 

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(2):600–7. https://doi.org/10.1164/ajrccm.160.2.9810008.

Article  CAS  PubMed  Google Scholar 

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 (London, England). 2002;360(9337):895–900. https://doi.org/10.1016/s0140-6736(02)11024-5.

Article  CAS  PubMed  Google Scholar 

Chapman TH, Wilde M, Sheth A, Madden BP. Sildenafil therapy in secondary pulmonary hypertension: is there benefit in prolonged use? Vascul Pharmacol. 2009;51(2–3):90–5. https://doi.org/10.1016/j.vph.2009.04.002.

Article  CAS  PubMed  Google Scholar 

Corte TJ, Gatzoulis MA, Parfitt L, Harries C, Wells AU, Wort SJ. The use of sildenafil to treat pulmonary hypertension associated with interstitial lung disease. Respirology (Carlton, Vic). 2010;15(8):1226–32. https://doi.org/10.1111/j.1440-1843.2010.01860.x.

Article  PubMed  Google Scholar 

Zimmermann GS, von Wulffen W, Huppmann P, Meis T, Ihle F, Geiseler J, et al. Haemodynamic changes in pulmonary hypertension in patients with interstitial lung disease treated with PDE-5 inhibitors. Respirology (Carlton, Vic). 2014;19(5):700–6. https://doi.org/10.1111/resp.12294.

Article  PubMed  Google Scholar 

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(7):620–8. https://doi.org/10.1056/NEJMoa1002110.

Article  PubMed  Google Scholar 

Kolb M, Raghu G, Wells AU, Behr J, Richeldi L, Schinzel B, et al. Nintedanib plus sildenafil in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2018;379(18):1722–31. https://doi.org/10.1056/NEJMoa1811737.

Article  CAS  PubMed  Google Scholar 

Gessler N, Gunawardene MA, Wohlmuth P, Arnold D, Behr J, Gloeckner C, et al. Clinical outcome, risk assessment, and seasonal variation in hospitalized COVID-19 patients-results from the CORONA Germany study. PLoS One. 2021;16(6):e0252867. https://doi.org/10.1371/journal.pone.0252867.

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(2):208–17. https://doi.org/10.1164/rccm.201403-0446OC.

Article  CAS  PubMed  PubMed Central  Google Scholar 

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(6):1622–32. https://doi.org/10.1183/09031936.00104612.

Article  CAS  PubMed  Google Scholar 

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(9):641–9. https://doi.org/10.7326/0003-4819-158-9-201305070-00003.

Article  PubMed  Google Scholar 

Nathan SD, Behr J, Collard HR, Cottin V, Hoeper MM, Martinez FJ, et al. Riociguat for idiopathic interstitial pneumonia-associated pulmonary hypertension (RISE-IIP): a randomised, placebo-controlled phase 2b study. Lancet Respir Med. 2019;7(9):780–90. https://doi.org/10.1016/s2213-2600(19)30250-4.

Article  CAS  PubMed  Google Scholar 

•• Waxman A, Restrepo-Jaramillo R, Thenappan T, Ravichandran A, Engel P, Bajwa A, et al. Inhaled treprostinil in pulmonary hypertension due to interstitial lung disease. N Engl J Med. 2021;384(4):325–34. https://doi.org/10.1056/NEJMoa2008470.