Pulmonary Hypertension

  • Peter M. HickeyEmail author
  • Robin Condliffe
  • Allan Lawrie
  • David G. Kiely


While pulmonary hypertension is readily diagnosed at cardiac catheterisation, delayed recognition and targeting treatment to the underlying cause can be challenging. Modern classification distinguishes between pulmonary hypertension secondary to cardiac disease, hypoxia with or without lung disease, thromboembolic pulmonary vascular disease, pulmonary hypertension associated with systemic diseases, and idiopathic forms. The physiological consequences and the use of various imaging modalities in refining diagnosis are discussed. For patients with idiopathic pulmonary hypertension, novel pharmacological approaches, either singly or in combination, have been more rigorously evaluated, and the use of surgery for chronic thromboembolic pulmonary hypertension is now well established.


Definition and classification Diagnostic strategies Investigation Imaging Cardiac catheterisation Pregnancy Anaesthesia Sepsis Pulmonary arterial hypertension Left heart disease Chronic lung disease Chronic thromboembolic disease Pulmonary endarterectomy Targeted treatments 


  1. 1.
    Hatano S, Strasser T. Primary pulmonary hypertension. In: Report on a WHO meeting, Geneva, 15–17 October 1973. Geneva: World Health Organization; 1975.Google 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.
    Kiely DG, Elliot CA, Sabroe I, Condliffe R. Pulmonary hypertension: diagnosis and management. BMJ. 2013;346:f2028.CrossRefPubMedGoogle Scholar
  4. 4.
    Hurdman J, Condliffe R, Elliot CA, Davies C, Hill C, Wild JM, et al. ASPIRE registry: assessing the Spectrum of pulmonary hypertension identified at a REferral Centre. Eur Respir J. 2012;39(4):945–55.CrossRefPubMedGoogle Scholar
  5. 5.
    Ling Y, Johnson MK, Kiely DG, Condliffe R, Elliot CA, Gibbs JS, et al. Changing demographics, epidemiology, and survival of incident pulmonary arterial hypertension: results from the pulmonary hypertension registry of the United Kingdom and Ireland. Am J Respir Crit Care Med. 2012;186(8):790–6.CrossRefGoogle Scholar
  6. 6.
    Edelman JD. Clinical presentation, differential diagnosis, and vasodilator testing of pulmonary hypertension. Semin Cardiothorac Vasc Anesth. 2007;11(2):110–8.CrossRefPubMedGoogle Scholar
  7. 7.
    Swift AJ, Rajaram S, Condliffe R, Capener D, Hurdman J, Elliot CA, et al. Diagnostic accuracy of cardiovascular magnetic resonance imaging of right ventricular morphology and function in the assessment of suspected pulmonary hypertension results from the ASPIRE registry. J Cardiovasc Magn Reson. 2012;14:40.CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Rajaram S, Swift AJ, Telfer A, Hurdman J, Marshall H, Lorenz E, et al. 3D contrast-enhanced lung perfusion MRI is an effective screening tool for chronic thromboembolic pulmonary hypertension: results from the ASPIRE Registry. Thorax. 2013;68(7):677–8.CrossRefPubMedGoogle Scholar
  9. 9.
    McCann C, Gopalan D, Sheares K, Screaton N. Imaging in pulmonary hypertension, part 1: clinical perspectives, classification, imaging techniques and imaging algorithm. Postgrad Med J. 2012;88(1039):271–9.CrossRefPubMedGoogle Scholar
  10. 10.
    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(5):735–40.CrossRefPubMedGoogle Scholar
  11. 11.
    Worsley DF, Palevsky HI, Alavi A. Ventilation-perfusion lung scanning in the evaluation of pulmonary hypertension. J Nucl Med. 1994;35(5):793–6.PubMedGoogle Scholar
  12. 12.
    Rajaram S, Swift AJ, Condliffe R, Johns C, Elliot CA, Hill C, et al. CT features of pulmonary arterial hypertension and its major subtypes: a systematic CT evaluation of 292 patients from the ASPIRE registry. Thorax. 2015;70(4):382–7.CrossRefPubMedGoogle Scholar
  13. 13.
    Montani D, Gunther S, Dorfmuller P, Perros F, Girerd B, Garcia G, et al. Pulmonary arterial hypertension. Orphanet J Rare Dis. 2013;8:97.CrossRefPubMedPubMedCentralGoogle Scholar
  14. 14.
    Lawrie A, Waterman E, Southwood M, Evans D, Suntharalingam J, Francis S, et al. Evidence of a role for osteoprotegerin in the pathogenesis of pulmonary arterial hypertension. Am J Pathol. 2008;172(1):256–64.CrossRefPubMedPubMedCentralGoogle Scholar
  15. 15.
    Ma L, Chung WK. The genetic basis of pulmonary arterial hypertension. Hum Genet. 2014;133(5):471–9.CrossRefPubMedGoogle Scholar
  16. 16.
    Condliffe R, Howard LS. Connective tissue disease-associated pulmonary arterial hypertension. F1000Prime Rep. 2015;7:06.CrossRefPubMedPubMedCentralGoogle Scholar
  17. 17.
    Hussain N, Charalampopoulos A, Ramjug S, Condliffe R, Elliot CA, O’Toole L, et al. Pulmonary hypertension in patients with heart failure and preserved ejection fraction: differential diagnosis and management. Pulm Circ. 2016;6(1):3–14.CrossRefPubMedPubMedCentralGoogle Scholar
  18. 18.
    Guazzi M, Borlaug BA. Pulmonary hypertension due to left heart disease. Circulation. 2012;126(8):975–90.CrossRefPubMedGoogle Scholar
  19. 19.
    Charalampopoulos A, Howard LS, Tzoulaki I, Gin-Sing W, Grapsa J, Wilkins MR, et al. Response to pulmonary arterial hypertension drug therapies in patients with pulmonary arterial hypertension and cardiovascular risk factors. Pulm Circ. 2014;4(4):669–78.CrossRefPubMedPubMedCentralGoogle Scholar
  20. 20.
    Zangiabadi A, De Pasquale CG, Sajkov D. Pulmonary hypertension and right heart dysfunction in chronic lung disease. Biomed Res Int. 2014;2014:739674.CrossRefPubMedPubMedCentralGoogle Scholar
  21. 21.
    Lang IM, Madani M. Update on chronic thromboembolic pulmonary hypertension. Circulation. 2014;130(6):508–18.CrossRefPubMedGoogle Scholar
  22. 22.
    Humbert M. Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: pathophysiology. Eur Respir Rev. 2010;19(115):59–63.CrossRefPubMedGoogle Scholar
  23. 23.
    Delcroix M, Lang I, Pepke-Zaba J, Jansa P, D’Armini AM, Snijder R, et al. Long-yerm outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. Circulation. 2016;133(9):859–71.CrossRefPubMedGoogle Scholar
  24. 24.
    Pritts CD, Pearl RG. Anesthesia for patients with pulmonary hypertension. Curr Opin Anaesthesiol. 2010;23(3):411–6.CrossRefPubMedGoogle Scholar
  25. 25.
    Galie N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, et al. 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.CrossRefPubMedGoogle Scholar
  26. 26.
    Hardinge M, Annandale J, Bourne S, Cooper B, Evans A, Freeman D, et al. British Thoracic Society guidelines for home oxygen use in adults. Thorax. 2015;70(Suppl 1):i1–43.CrossRefPubMedGoogle Scholar
  27. 27.
    Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB, Primary Pulmonary Hypertension Study Group, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med. 1996;334(5):296–301.CrossRefGoogle Scholar
  28. 28.
    Garnock-Jones KP. Riociguat: a review of its use in patients with chronic thromboembolic pulmonary hypertension or pulmonary arterial hypertension. Drugs. 2014;74(17):2065–78.CrossRefPubMedGoogle Scholar
  29. 29.
    Banks DA, Pretorius GV, Kerr KM, Manecke GR. Pulmonary endarterectomy: part I. Pathophysiology, clinical manifestations, and diagnostic evaluation of chronic thromboembolic pulmonary hypertension. Semin Cardiothorac Vasc Anesth. 2014;18(4):319–30.CrossRefPubMedGoogle Scholar
  30. 30.
    Fadel E, Mercier O, Mussot S, Leroy-Ladurie F, Cerrina J, Chapelier A, et al. Long-term outcome of double-lung and heart-lung transplantation for pulmonary hypertension: a comparative retrospective study of 219 patients. Eur J Cardiothorac Surg. 2010;38(3):277–84.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Peter M. Hickey
    • 1
    Email author
  • Robin Condliffe
    • 2
  • Allan Lawrie
    • 3
  • David G. Kiely
    • 2
  1. 1.Pulmonary Vascular Research Group, Department of Infection, Immunity and Cardiovascular Disease (IICD)University of SheffieldSheffieldUK
  2. 2.Pulmonary Vascular Disease UnitRoyal Hallamshire Hospital, Sheffield Teaching Hospitals NHS TrustSheffieldUK
  3. 3.Pulmonary Vascular Research Group, Department of Infection, Immunity and Cardiovascular DiseaseUniversity of SheffieldSheffieldUK

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