Semin Respir Crit Care Med 2017; 38(05): 561-570
DOI: 10.1055/s-0037-1606577
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Pulmonary Hypertension: Definition, Classification, and Diagnosis

Jose Leonidas Alves-Jr
1   Pulmonary Department, Heart Institute, University of Sao Paulo Medical School, Sao Paulo, Brazil
,
Francisca Gavilanes Oleas
1   Pulmonary Department, Heart Institute, University of Sao Paulo Medical School, Sao Paulo, Brazil
,
Rogerio Souza
1   Pulmonary Department, Heart Institute, University of Sao Paulo Medical School, Sao Paulo, Brazil
› Author Affiliations
Further Information

Publication History

Publication Date:
15 October 2017 (online)

Abstract

Pulmonary hypertension (PH) is a hemodynamic condition characterized by the presence of mean pulmonary artery pressure ≥ 25 mm Hg. Many different baseline conditions and/or risk factors are associated with the development of PH, increasing the complexity for the appropriate diagnosis and classification. Since it was first proposed, PH classification has been adapted to incorporate the increasing knowledge about the disease gathered along the past two decades. Current classification groups together the patients with similar pathophysiological mechanisms, clinical presentation, and management. Nevertheless, correct classification is only possible after a comprehensive diagnostic approach, assessing the most prevalent conditions associated with PH. Most importantly, left ventricular function, lung parenchyma, and the presence of chronic thromboembolic disease have to be evaluated by means of different diagnostic tools. The aim of this review is to summarize the current definitions of PH, which in turn is necessary for determination of the appropriate diagnostic approach.

 
  • References

  • 1 McLaughlin VV, Shah SJ, Souza R, Humbert M. Management of pulmonary arterial hypertension. J Am Coll Cardiol 2015; 65 (18) 1976-1997
  • 2 Costa EL, Jardim C, Bogossian HB, Amato MB, Carvalho CR, Souza R. Acute vasodilator test in pulmonary arterial hypertension: evaluation of two response criteria. Vascul Pharmacol 2005; 43 (03) 143-147
  • 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 (04) 888-894
  • 4 Hamada K, Nagai S, Tanaka S. , et al. Significance of pulmonary arterial pressure and diffusion capacity of the lung as prognosticator in patients with idiopathic pulmonary fibrosis. Chest 2007; 131 (03) 650-656
  • 5 Bae S, Saggar R, Bolster MB. , et al. Baseline characteristics and follow-up in patients with normal haemodynamics versus borderline mean pulmonary arterial pressure in systemic sclerosis: results from the PHAROS registry. Ann Rheum Dis 2012; 71 (08) 1335-1342
  • 6 Naeije R, Vonk Noordegraaf A, Kovacs G. Exercise-induced pulmonary hypertension: at last!. Eur Respir J 2015; 46 (03) 583-586
  • 7 Herve P, Lau EM, Sitbon O. , et al. Criteria for diagnosis of exercise pulmonary hypertension. Eur Respir J 2015; 46 (03) 728-737
  • 8 Hatano S, Strasser T. Primary pulmonary hypertension. Report on WHO Meeting. October 15–17, 1973. Geneva, WHO. 1975
  • 9 Frere C, Farge D. Clinical practice guidelines for prophylaxis of venous thomboembolism in cancer patients. Thromb Haemost 2016; 116 (04) 618-625
  • 10 Simonneau G, Galiè N, Rubin LJ. , et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol 2004; 43 (12, Suppl S) 5S-12S
  • 11 Simonneau G, Robbins IM, Beghetti M. , et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009; 54 (1, Suppl) S43-S54
  • 12 Hoeper MM, Humbert M, Souza R. , et al. A global view of pulmonary hypertension. Lancet Respir Med 2016; 4 (04) 306-322
  • 13 McGoon MD, Benza RL, Escribano-Subias P. , et al. Pulmonary arterial hypertension: epidemiology and registries. J Am Coll Cardiol 2013; 62 (25, Suppl) D51-D59
  • 14 Gavilanes F, Alves Jr JL, Fernandes C. , et al. Left ventricular dysfunction in patients with suspected pulmonary arterial hypertension. J Bras Pneumol 2014; 40 (06) 609-616
  • 15 Alves Jr JL, Gavilanes F, Jardim C. , et al. Pulmonary arterial hypertension in the southern hemisphere: results from a registry of incident Brazilian cases. Chest 2015; 147 (02) 495-501
  • 16 Galiè N, Humbert M, Vachiery JL. , 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 (01) 67-119
  • 17 Machado RD, Eickelberg O, Elliott CG. , et al. Genetics and genomics of pulmonary arterial hypertension. J Am Coll Cardiol 2009; 54 (1, Suppl) S32-S42
  • 18 Soubrier F, Chung WK, Machado R. , et al. Genetics and genomics of pulmonary arterial hypertension. J Am Coll Cardiol 2013; 62 (25, Suppl) D13-D21
  • 19 Simonneau G, Gatzoulis MA, Adatia I. , et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2013; 62 (25, Suppl) D34-D41
  • 20 Souza R, Humbert M, Sztrymf B. , et al. Pulmonary arterial hypertension associated with fenfluramine exposure: report of 109 cases. Eur Respir J 2008; 31 (02) 343-348
  • 21 Hoeper MM, Barst RJ, Bourge RC. , et al. Imatinib mesylate as add-on therapy for pulmonary arterial hypertension: results of the randomized IMPRES study. Circulation 2013; 127 (10) 1128-1138
  • 22 Rasheed W, Flaim B, Seymour JF. Reversible severe pulmonary hypertension secondary to dasatinib in a patient with chronic myeloid leukemia. Leuk Res 2009; 33 (06) 861-864
  • 23 Montani D, Bergot E, Günther S. , et al. Pulmonary arterial hypertension in patients treated by dasatinib. Circulation 2012; 125 (17) 2128-2137
  • 24 Savale L, Chaumais MC, O'Connell C, Humbert M, Sitbon O. Interferon-induced pulmonary hypertension: an update. Curr Opin Pulm Med 2016; 22 (05) 415-420
  • 25 Alwan S, Bandoli G, Chambers CD. Maternal use of selective serotonin-reuptake inhibitors and risk of persistent pulmonary hypertension of the newborn. Clin Pharmacol Ther 2016; 100 (01) 34-41
  • 26 Aithala R, Alex AG, Danda D. Pulmonary hypertension in connective tissue diseases: an update. Int J Rheum Dis 2017; 20 (01) 5-24
  • 27 Hachulla E, Gressin V, Guillevin L. , et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum 2005; 52 (12) 3792-3800
  • 28 Benza RL, Gomberg-Maitland M, Miller DP. , et al. The REVEAL registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension. Chest 2012; 141 (02) 354-362
  • 29 Condliffe R, Kiely DG, Peacock AJ. , et al. Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med 2009; 179 (02) 151-157
  • 30 Sitbon O, Lascoux-Combe C, Delfraissy JF. , et al. Prevalence of HIV-related pulmonary arterial hypertension in the current antiretroviral therapy era. Am J Respir Crit Care Med 2008; 177 (01) 108-113
  • 31 Humbert M, Khaltaev N, Bousquet J, Souza R. Pulmonary hypertension: from an orphan disease to a public health problem. Chest 2007; 132 (02) 365-367
  • 32 Zopey R, Susanto I, Barjaktarevic I, Wang T. Transition from hepatopulmonary syndrome to portopulmonary hypertension: a case series of 3 patients. Case Rep Pulmonol 2013; 2013: 561870
  • 33 World Health Organization. Shistosomiasis Fact sheet 115, Updated March 2013
  • 34 Gavilanes F, Fernandes CJ, Souza R. Pulmonary arterial hypertension in schistosomiasis. Curr Opin Pulm Med 2016; 22 (05) 408-414
  • 35 Mauad T, Pozzan G, Lanças T. , et al. Immunopathological aspects of schistosomiasis-associated pulmonary arterial hypertension. J Infect 2014; 68 (01) 90-98
  • 36 Lapa M, Dias B, Jardim C. , et al. Cardiopulmonary manifestations of hepatosplenic schistosomiasis. Circulation 2009; 119 (11) 1518-1523
  • 37 Fernandes CJ, Jardim CV, Hovnanian A, Hoette S, Morinaga LK, Souza R. Schistosomiasis and pulmonary hypertension. Expert Rev Respir Med 2011; 5 (05) 675-681
  • 38 Hoette S, Figueiredo C, Dias B. , et al. Pulmonary artery enlargement in schistosomiasis associated pulmonary arterial hypertension. BMC Pulm Med 2015; 15: 118
  • 39 dos Santos Fernandes CJ, Jardim CV, Hovnanian A. , et al. Survival in schistosomiasis-associated pulmonary arterial hypertension. J Am Coll Cardiol 2010; 56 (09) 715-720
  • 40 Fernandes CJCS, Dias BA, Jardim CVP. , et al. The role of target therapies in schistosomiasis-associated pulmonary arterial hypertension. Chest 2012; 141 (04) 923-928
  • 41 Montani D, Lau EM, Dorfmüller P. , et al. Pulmonary veno-occlusive disease. Eur Respir J 2016; 47 (05) 1518-1534
  • 42 Vachiéry JL, Adir Y, Barberà JA. , et al. Pulmonary hypertension due to left heart diseases. J Am Coll Cardiol 2013; 62 (25, Suppl) D100-D108
  • 43 Oudiz RJ. Pulmonary hypertension associated with left-sided heart disease. Clin Chest Med 2007; 28 (01) 233-241 , x
  • 44 Seeger W, Adir Y, Barberà JA. , et al. Pulmonary hypertension in chronic lung diseases. J Am Coll Cardiol 2013; 62 (25, Suppl) D109-D116
  • 45 Kim NH, Delcroix M, Jenkins DP. , et al. Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol 2013; 62 (25, Suppl) D92-D99
  • 46 Guérin L, Couturaud F, Parent F. , et al. Prevalence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism. Prevalence of CTEPH after pulmonary embolism. Thromb Haemost 2014; 112 (03) 598-605
  • 47 Fonseca GH, Souza R, Salemi VM, Jardim CV, Gualandro SF. Pulmonary hypertension diagnosed by right heart catheterisation in sickle cell disease. Eur Respir J 2012; 39 (01) 112-118
  • 48 Parent F, Bachir D, Inamo J. , et al. A hemodynamic study of pulmonary hypertension in sickle cell disease. N Engl J Med 2011; 365 (01) 44-53
  • 49 Mehari A, Gladwin MT, Tian X, Machado RF, Kato GJ. Mortality in adults with sickle cell disease and pulmonary hypertension. JAMA 2012; 307 (12) 1254-1256
  • 50 Kalantari S, Gomberg-Maitland M. Group 5 pulmonary hypertension: the Orphan's orphan disease. Cardiol Clin 2016; 34 (03) 443-449
  • 51 Garypidou V, Vakalopoulou S, Dimitriadis D, Tziomalos K, Sfikas G, Perifanis V. Incidence of pulmonary hypertension in patients with chronic myeloproliferative disorders. Haematologica 2004; 89 (02) 245-246
  • 52 Handa T, Nagai S, Miki S. , et al. Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis. Chest 2006; 129 (05) 1246-1252
  • 53 Baughman RP, Engel PJ, Taylor L, Lower EE. Survival in sarcoidosis-associated pulmonary hypertension: the importance of hemodynamic evaluation. Chest 2010; 138 (05) 1078-1085
  • 54 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; 142 (05) 1150-1157
  • 55 Freitas CSG, Baldi BG, Jardim C. , et al. Pulmonary hypertension in lymphangioleiomyomatosis: prevalence, severity and the role of carbon monoxide diffusion capacity as a screening method. Orphanet J Rare Dis 2017; 12 (01) 74
  • 56 Humbert M, Sitbon O, Chaouat A. , et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 2006; 173 (09) 1023-1030
  • 57 Taichman DB, McGoon MD, Harhay MO. , et al. Wide variation in clinicians' assessment of New York Heart Association/World Health Organization functional class in patients with pulmonary arterial hypertension. Mayo Clin Proc 2009; 84 (07) 586-592
  • 58 Ahearn GS, Tapson VF, Rebeiz A, Greenfield Jr JC. Electrocardiography to define clinical status in primary pulmonary hypertension and pulmonary arterial hypertension secondary to collagen vascular disease. Chest 2002; 122 (02) 524-527
  • 59 Vonk-Noordegraaf A, Souza R. Cardiac magnetic resonance imaging: what can it add to our knowledge of the right ventricle in pulmonary arterial hypertension?. Am J Cardiol 2012; 110 (6, Suppl) 25S-31S
  • 60 Hovnanian A, Menezes E, Hoette S, Jardim C, Jasinowodolinski D, Souza R. The role of imaging techniques in the assessment of pulmonary circulation. J Bras Pneumol 2011; 37 (03) 389-403
  • 61 Hoeper MM, Bogaard HJ, Condliffe R. , et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol 2013; 62 (25, Suppl) D42-D50
  • 62 Sitbon O, Humbert M, Jaïs X. , et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005; 111 (23) 3105-3111