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Influence of Pulmonary Hypertension on Survival in Advanced Lung Disease

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Abstract

Background

Effects of pulmonary hypertension (PH) in advanced lung disease remain unclear.

Methods

The United Network for Organ Sharing database was queried from 1987 to 2013 to assess survival lung transplant candidates to determine influence of PH in advanced lung disease. Thresholds included mean pulmonary artery pressure ≥25 mmHg (mild PH) and 35 mmHg (severe PH).

Results

Of 12,405 listed possible candidates, 10,158 were used for univariate analysis, 7050 for Kaplan–Meier (KM) function, 6196 for multivariate Cox models, and 5328 (mild PH) and 1910 (severe PH) for propensity score matching (PSM). For mild and severe PH, univariate revealed that PH was associated with survival difference (HR = 1.530, 95 % CI 1.416, 1.654, p < 0.001) and (HR = 2.033, 95 % CI 1.851, 2.232, p < 0.001), respectively. KM function curves demonstrated a significant difference for mild PH (Log-rank test: Chi square (df = 1): 117.76, p < 0.0001) and severe PH (Log-rank test: Chi square (df = 1): 230.91, p < 0.0001). Multivariate Cox models also found a significant increased risk for death for mild PH (HR = 1.750, 95 % CI 1.606, 1.907, p < 0.001) and severe PH (HR = 2.088, 95 % CI 1.879, 2.319, p < 0.001). PSM confirmed this increased risk for death for mild PH (HR = 1.695, 95 % CI 1.502, 1.914, p < 0.001) and severe PH (HR = 1.976, 95 % CI 1.641, 2.379, p < 0.001).

Conclusions

PH is associated with significant increased risk for death in patients with advanced lung disease.

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References

  1. Hatano S, Strasser T (1973) Primary Pulmonary Hypertension. Report on a WHO Meeting. World Health Organization, Geneva, 15–17 Oct 1973(1975)

  2. Wagenvoort CA, Wagenvoort N (1970) Primary pulmonary hypertension: a pathologic study of the lung vessels in 156 clinically diagnosed cases. Circulation 42:1163–1184

    Article  Google Scholar 

  3. Fishman AP (2001) Clinical classification of pulmonary hypertension. Clin Chest Med 22(3):385–391

    Article  CAS  PubMed  Google Scholar 

  4. Simonneau G, Robbins IM, Beghetti M et al (2009) Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 54(1 Suppl):S43–S54

    Article  PubMed  Google Scholar 

  5. Simonneau G, Gatzoulis MA, Adatia I et al (2013) Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 62(25 Suppl):D34–41 Erratum in: J Am Coll Cardiol 2014;63(7):746

    Article  PubMed  Google Scholar 

  6. 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 

  7. 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 

  8. 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 Transpl 31(4):373–380

    Article  Google Scholar 

  9. Hayes D Jr, Tobias JD, Mansour HM et al (2014 Oct 15) Pulmonary hypertension in cystic fibrosis with advanced lung disease. Am J Respir Crit Care Med 190(8):898–905. doi:10.1164/rccm.201407-1382OC

    Article  PubMed  Google Scholar 

  10. Tonelli AR, Fernandez-Bussy S, Lodhi S et al (2010) Prevalence of pulmonary hypertension in end-stage cystic fibrosis and correlation with survival. J Heart Lung Transpl 29(8):865–872

    Article  Google Scholar 

  11. Venuta F, Tonelli AR, Anile M et al (2012) Pulmonary hypertension is associated with higher mortality in cystic fibrosis patients awaiting lung transplantation. J Cardiovasc Surg (Torino) 53(6):817–820

    CAS  Google Scholar 

  12. Belle-van Meerkerk G, Cramer MJ, Kwakkel-van Erp JM et al (2013) Pulmonary hypertension is a mild comorbidity in end-stage cystic fibrosis patients. J Heart Lung Transpl 32(6):609–614

    Article  Google Scholar 

  13. Hayes D Jr, Daniels CJ, Mansour HM et al (2013) Right heart catheterization measuring central hemodynamics in cystic fibrosis during exercise. Respir Med 107(9):1365–1369

    Article  PubMed  Google Scholar 

  14. Hayes D Jr, Higgins RS, Kirkby S et al (2013) Impact of pulmonary hypertension on survival in patients with cystic fibrosis undergoing lung transplantation: an analysis of the UNOS registry. J Cyst Fibros 3(4):416–423

    Google Scholar 

  15. Andersen CU, Mellemkjær S, Nielsen-Kudsk JE et al (2012) Diagnostic and prognostic role of biomarkers for pulmonary hypertension in interstitial lung disease. Respir Med 106(12):1749–1755

    Article  PubMed  Google Scholar 

  16. Nadrous HF, Pellikka PA, Krowka MJ et al (2005) Pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Chest 128(4):2393–2399

    Article  PubMed  Google Scholar 

  17. Lettieri CJ, Nathan SD, Barnett SD et al (2006) Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest 129(3):746–752

    Article  PubMed  Google Scholar 

  18. Shorr AF, Wainright JL, Cors CS et al (2007) Pulmonary hypertension in patients with pulmonary fibrosis awaiting lung transplant. Eur Respir J 30(4):715–721

    Article  CAS  PubMed  Google Scholar 

  19. Mejía M, Carrillo G, Rojas-Serrano J et al (2009) Idiopathic pulmonary fibrosis and emphysema: decreased survival associated with severe pulmonary arterial hypertension. Chest 136(1):10–15

    Article  PubMed  Google Scholar 

  20. Papakosta D, Pitsiou G, Daniil Z et al (2011) Prevalence of pulmonary hypertension in patients with idiopathic pulmonary fibrosis: correlation with physiological parameters. Lung 189(5):391–399

    Article  CAS  PubMed  Google Scholar 

  21. Andersen CU, Mellemkjær S, Hilberg O et al (2012) Pulmonary hypertension in interstitial lung disease: prevalence, prognosis and 6 min walk test. Respir Med 106(6):875–882

    Article  PubMed  Google Scholar 

  22. 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 

  23. Rivera-Lebron BN, Forfia PR, Kreider M et al (2013) Echocardiographic and hemodynamic predictors of mortality in idiopathic pulmonary fibrosis. Chest 144(2):564–570

    Article  PubMed Central  PubMed  Google Scholar 

  24. Baughman RP, Engel PJ, Taylor L et al (2010) Survival in sarcoidosis-associated pulmonary hypertension: the importance of hemodynamic evaluation. Chest 138(5):1078–1085

    Article  PubMed  Google Scholar 

  25. Nardi A, Brillet PY, Letoumelin P et al (2011) Stage IV sarcoidosis: comparison of survival with the general population and causes of death. Eur Respir J 38(6):1368–1373

    Article  CAS  PubMed  Google Scholar 

  26. Rapti A, Kouranos V, Gialafos E et al (2013) Elevated pulmonary arterial systolic pressure in patients with sarcoidosis: prevalence and risk factors. Lung 191(1):61–67

    Article  PubMed  Google Scholar 

  27. United Network for Organ Sharing/Organ Procurement and Transplantation Network Standard Transplant Analysis and Research Database: http://optn.transplant.hrsa.gov/data/about/OPTNDatabase.asp. Accessed 6 Sep 2013

  28. Han MK, McLaughlin VV, Criner GJ et al (2007) Pulmonary diseases and the heart. Circulation 116(25):2992–3005

    Article  PubMed  Google Scholar 

  29. Farber MO, Roberts LR, Weinberger MH et al (1982) Abnormalities of sodium and H2O handling in chronic obstructive lung disease. Arch Intern Med 142(7):1326–1330

    Article  CAS  PubMed  Google Scholar 

  30. Schafer JA, Robert F (1984) Pitts Memorial Lecture. Mechanisms coupling the absorption of solutes and water in the proximal nephron. Kidney Int 25(4):708–716

    Article  CAS  PubMed  Google Scholar 

  31. Brijker F, Heijdra YF, van den Elshout FJ et al (2002) Discontinuation of furosemide decreases PaCO(2) in patients with COPD. Chest 121(2):377–382

    Article  CAS  PubMed  Google Scholar 

  32. de Leeuw PW, Dees A (2003) Fluid homeostasis in chronic obstructive lung disease. Eur Respir J Suppl 46:33s–40s

    Article  PubMed  Google Scholar 

  33. Tsangaris I, Tsaknis G, Anthi A et al (2012) Pulmonary hypertension in parenchymal lung disease. Pulm Med 2012:684781

    Article  PubMed Central  PubMed  Google Scholar 

  34. Arcasoy SM, Christie JD, Ferrari VA et al (2003) Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease. Am J Respir Crit Care Med 167(5):735–740

    Article  PubMed  Google Scholar 

  35. Nathan SD, Shlobin OA, Barnett SD et al (2008) Right ventricular systolic pressure by echocardiography as a predictor of pulmonary hypertension in idiopathic pulmonary fibrosis. Respir Med 102(9):1305–1310

    Article  PubMed Central  PubMed  Google Scholar 

  36. Enson Y, Giuntini C, Lewis ML et al (1964) The influence of hydrogen ion concentration and hypoxia on the pulmonary circulation. J Clin Invest 43:1146–1162

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  37. Durand J, Le Roy Ladurie M, Ranson-Bitker B (1970) Effects of hypoxia and hypercapnia on the repartition of pulmonary blood flow in supine subjects. Respir Res 5:156–165

    Article  Google Scholar 

  38. Weitzenblum E, Chaouat A (2005) Severe pulmonary hypertension in COPD: is it a distinct disease? Chest 127(5):1480–1482

    Article  PubMed  Google Scholar 

  39. Wrobel JP, Thompson BR, Williams TJ (2012) Mechanisms of pulmonary hypertension in chronic obstructive pulmonary disease: a pathophysiologic review. J Heart Lung Transpl 31(6):557–564

    Article  Google Scholar 

  40. Carlsen J, Hasseriis Andersen K, Boesgaard S et al (2013) Pulmonary arterial lesions in explanted lungs after transplantation correlate with severity of pulmonary hypertension in chronic obstructive pulmonary disease. J Heart Lung Transpl 32(3):347–354

    Article  Google Scholar 

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Acknowledgments

The authors would like to acknowledge Dmitry Tumin for his statistical expertise in the data analysis. No funding was required to complete this work.

Conflict of interest

The authors report no conflicts of interest and have no relevant disclosures.

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Correspondence to Don Hayes Jr..

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Hayes, D., Black, S.M., Tobias, J.D. et al. Influence of Pulmonary Hypertension on Survival in Advanced Lung Disease. Lung 193, 213–221 (2015). https://doi.org/10.1007/s00408-015-9696-8

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  • DOI: https://doi.org/10.1007/s00408-015-9696-8

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