Pulmonary Hypertension

  • Jordan D. Awerbach
  • Richard A. KrasuskiEmail author
Part of the Contemporary Cardiology book series (CONCARD)


This chapter explores the epidemiology, clinical presentation, and evaluation of patients with suspected pulmonary hypertension (PH). Right heart catheterization remains the gold standard for diagnosing PH. Accurate assessment of hemodynamics, in particular the pulmonary capillary wedge pressure, is critical to accurately make the diagnosis and to differentiate pre- from postcapillary forms of PH. All patients with pulmonary arterial hypertension (PAH) should also undergo pulmonary vasoreactivity testing on initial catheterization, most commonly performed with inhaled nitric oxide. It is also reasonable to perform a full oxygen saturation run in this population to rule out congenital heart disease as an etiology of PAH. The chapter concludes with a brief discussion on advanced therapies for the treatment of pulmonary arterial hypertension and several illustrative case examples.


Pulmonary hypertension Pulmonary arterial hypertension Pulmonary vascular resistance Vasoreactivity Congenital heart disease 


  1. 1.
    Galie N, Torbicki A, Barst R, Dartevelle P, Haworth S, Higenbottam T, et al. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The task force on diagnosis and treatment of pulmonary arterial hypertension of the European Society of Cardiology. Eur Heart J. 2004;25(24):2243–78.CrossRefGoogle Scholar
  2. 2.
    Simonneau G, Gatzoulis MA, Adatia I, Celermajer D, Denton C, Ghofrani A, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25 Suppl):D34–41.CrossRefGoogle Scholar
  3. 3.
    Kovacs G, Berghold A, Scheidl S, Olschewski H. Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review. Eur Respir J. 2009;34(4):888–94.CrossRefGoogle Scholar
  4. 4.
    Galie 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 Heart J. 2016;37(1):67–119.CrossRefGoogle Scholar
  5. 5.
    Hoeper MM, Humbert M, Souza R, Idrees M, Kawut SM, Sliwa-Hahnle K, et al. A global view of pulmonary hypertension. Lancet Respir Med. 2016;4(4):306–22.CrossRefGoogle Scholar
  6. 6.
    Lai YC, Potoka KC, Champion HC, Mora AL, Gladwin MT. Pulmonary arterial hypertension: the clinical syndrome. Circ Res. 2014;115(1):115–30.CrossRefGoogle Scholar
  7. 7.
    Soubrier F, Chung WK, Machado R, Grunig E, Aldred M, Geraci M, et al. Genetics and genomics of pulmonary arterial hypertension. J Am Coll Cardiol. 2013;62(25 Suppl):D13–21.CrossRefGoogle Scholar
  8. 8.
    Evans JDW, Girerd B, Montani D, Wang X-J, Galiè N, Austin ED, et al. BMPR2 mutations and survival in pulmonary arterial hypertension: an individual participant data meta-analysis. Lancet Respir Med. 2016;4(2):129–37.CrossRefGoogle Scholar
  9. 9.
    Hoeper MM, Simon RGJ. The changing landscape of pulmonary arterial hypertension and implications for patient care. Eur Respir Rev. 2014;23(134):450–7.CrossRefGoogle Scholar
  10. 10.
    McGoon MD, Miller DP. REVEAL: a contemporary US pulmonary arterial hypertension registry. Eur Respir Rev. 2012;21(123):8–18.CrossRefGoogle Scholar
  11. 11.
    D’Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension: results from a national prospective registry. Ann Intern Med. 1991;115(5):343–9.CrossRefGoogle Scholar
  12. 12.
    Benza RL, Miller DP, Barst RJ, Badesch DB, Frost AE, McGoon MD. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL registry. Chest. 2012;142(2):448–56.CrossRefGoogle Scholar
  13. 13.
    Brown LM, Chen H, Halpern S, Taichman D, McGoon MD, Farber HW, et al. Delay in recognition of pulmonary arterial hypertension: factors identified from the REVEAL registry. Chest. 2011;140(1):19–26.CrossRefGoogle Scholar
  14. 14.
    Runo JR, Loyd JE. Primary pulmonary hypertension. Lancet. 2003;361(9368):1533–44.CrossRefGoogle Scholar
  15. 15.
    Benza RL, Miller DP, Gomberg-Maitland M, Frantz RP, Foreman AJ, Coffey CS, et al. Predicting survival in pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management (REVEAL). Circulation. 2010;122(2):164–72.CrossRefGoogle Scholar
  16. 16.
    Gopalan D, Blanchard D, Auger WR. Diagnostic evaluation of chronic thromboembolic pulmonary hypertension. Ann Am Thorac Soc. 2016;13(Suppl 3):S222–39.CrossRefGoogle Scholar
  17. 17.
    Seeger W, Adir Y, Barbera JA, Champion H, Coghlan JG, Cottin V, et al. Pulmonary hypertension in chronic lung diseases. J Am Coll Cardiol. 2013;62(25 Suppl):D109–16.CrossRefGoogle Scholar
  18. 18.
    Forfia PR, Fisher MR, Mathai SC, Housten-Harris T, Hemnes AR, Borlaug BA, et al. Tricuspid annular displacement predicts survival in pulmonary hypertension. Am J Respir Crit Care Med. 2006;174(9):1034–41.CrossRefGoogle Scholar
  19. 19.
    Zuckerman WA, Turner ME, Kerstein J, Torres A, Vincent JA, Krishnan U, et al. Safety of cardiac catheterization at a center specializing in the care of patients with pulmonary arterial hypertension. Pulm Circ. 2013;3(4):831–9.CrossRefGoogle Scholar
  20. 20.
    Hoeper MM, Maier R, Tongers J, Niedermeyer J, Hohlfeld JM, Hamm M, et al. Determination of cardiac output by the Fick method, thermodilution, and acetylene rebreathing in pulmonary hypertension. Am J Respir Crit Care Med. 1999;160:535–41.CrossRefGoogle Scholar
  21. 21.
    Narang N, Thibodeau JT, Levine BD, Gore MO, Ayers CR, Lange RA, et al. Inaccuracy of estimated resting oxygen uptake in the clinical setting. Circulation. 2014;129(2):203–10.CrossRefGoogle Scholar
  22. 22.
    Benza RL, Gomberg-Maitland M, Miller DP, Frost A, Frantz RP, Foreman AJ, et al. The REVEAL registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension. Chest. 2012;141(2):354–62.CrossRefGoogle Scholar
  23. 23.
    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.CrossRefGoogle Scholar
  24. 24.
    Ryan JJ, Rich JD, Thiruvoipati T, Swamy R, Kim GH, Rich S. Current practice for determining pulmonary capillary wedge pressure predisposes to serious errors in the classification of patients with pulmonary hypertension. Am Heart J. 2012;163(4):589–94.CrossRefGoogle Scholar
  25. 25.
    Kovacs G, Olschewski A, Berghold A, Olschewski H. Pulmonary vascular resistances during exercise in normal subjects: a systematic review. Eur Respir J. 2012;39(2):319–28.CrossRefGoogle Scholar
  26. 26.
    Thenappan T, Ormiston ML, Ryan JJ, Archer SL. Pulmonary arterial hypertension: pathogenesis and clinical management. BMJ. 2018;360:j5492.CrossRefGoogle Scholar
  27. 27.
    Krasuski RA, Warner JJ, Wang A, Harrison JK, Tapson VF, Bashore TM. Inhaled nitric oxide selectively dilates pulmonary vasculature in adult patients with pulmonary hypertension, irrespective of etiology. J Am Coll Cardiol. 2000;36(7):2204–11.CrossRefGoogle Scholar
  28. 28.
    Krasuski RA, Devendra GP, Hart SA, Wang A, Harrison JK, Bashore TM. Response to inhaled nitric oxide predicts survival in patients with pulmonary hypertension. J Card Fail. 2011;17(4):265–71.CrossRefGoogle Scholar
  29. 29.
    Skoro-Sajer N, Hack N, Sadushi-Kolici R, Bonderman D, Jakowitsch J, Klepetko W, et al. Pulmonary vascular reactivity and prognosis in patients with chronic thromboembolic pulmonary hypertension: a pilot study. Circulation. 2009;119(2):298–305.CrossRefGoogle Scholar
  30. 30.
    Post MC, Janssens S, Van de Werf F, Budts W. Responsiveness to inhaled nitric oxide is a predictor for mid-term survival in adult patients with congenital heart defects and pulmonary arterial hypertension. Eur Heart J. 2004;25(18):1651–6.CrossRefGoogle Scholar
  31. 31.
    McLaughlin VV, Shah SJ, Souza R, Humbert M. Management of pulmonary arterial hypertension. J Am Coll Cardiol. 2015;65(18):1976–97.CrossRefGoogle Scholar
  32. 32.
    Andersen CU, Mellemkjær S, Nielsen-Kudsk JE, Bendstrup E, Hilberg O, Simonsen U. Pulmonary hypertension in chronic obstructive and interstitial lung diseases. Int J Cardiol. 2013;168(3):1795–804.CrossRefGoogle Scholar
  33. 33.
    Opitz CF, Hoeper MM, Gibbs JS, Kaemmerer H, Pepke-Zaba J, Coghlan JG, et al. Pre-capillary, combined, and post-capillary pulmonary hypertension: a pathophysiological continuum. J Am Coll Cardiol. 2016;68(4):368–78.CrossRefGoogle Scholar
  34. 34.
    Leopold JA. Catheter-based therapies for patients with medication-refractory pulmonary arterial hypertension. Circ Cardiovasc Interv. 2015;8(11):e003332.CrossRefGoogle Scholar
  35. 35.
    Esch JJ, Shah PB, Cockrill BA, Farber HW, Landzberg MJ, Mehra MR, et al. Transcatheter Potts shunt creation in patients with severe pulmonary arterial hypertension: initial clinical experience. J Heart Lung Transplant. 2013;32(4):381–7.CrossRefGoogle Scholar
  36. 36.
    Maron BA, Leopold JA. Emerging concepts in the molecular basis of pulmonary arterial hypertension: part II: neurohormonal signaling contributes to the pulmonary vascular and right ventricular pathophenotype of pulmonary arterial hypertension. Circulation. 2015;131(23):2079–91.CrossRefGoogle Scholar
  37. 37.
    Chen SL, Zhang H, Xie DJ, Zhang J, Zhou L, Rothman AM, et al. Hemodynamic, functional, and clinical responses to pulmonary artery denervation in patients with pulmonary arterial hypertension of different causes: phase II results from the pulmonary artery denervation-1 study. Circ Cardiovasc Interv. 2015;8(11):e002837.CrossRefGoogle Scholar
  38. 38.
    Sitbon O, Humbert M, Jais X, Ioos V, Hamid AM, Provencher S, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation. 2005;111(23):3105–11.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Division of Cardiovascular MedicineDuke University Health SystemDurhamUSA

Personalised recommendations