Pulmonary hypertension: Diagnostic andtherapeutic challenges

Therapeutics and Clinical Risk Management

Volumn 11, Issue , 2015, Pages 1221-1233

  Bazan, Isabel S ^a   Fares, Wassim H ^a  

^a YALE UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

Diagnosis; Management; Pulmonary arterial hypertension; Pulmonary hypertension; Right heart failure

Indexed keywords

AMINOREX; AMPHETAMINE; BOSENTAN; CALCIUM CHANNEL BLOCKING AGENT; COCAINE; DASATINIB; DIGOXIN; ENDOTHELIN RECEPTOR ANTAGONIST; FENFLURAMINE; GUANYLATE CYCLASE ACTIVATOR; ILOPROST; MAZINDOL; PHENTERMINE; PHOSPHODIESTERASE V INHIBITOR; PROSTACYCLIN; PROSTANOID; RIOCIGUAT; SEROTONIN UPTAKE INHIBITOR; SILDENAFIL; SPIRONOLACTONE; TADALAFIL; TREPROSTINIL;

CHRONIC LUNG DISEASE; CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION; CONGENITAL HEART DISEASE; CONNECTIVE TISSUE DISEASE; DIAGNOSTIC ACCURACY; DISEASE ASSOCIATION; DISEASE CLASSIFICATION; DOPPLER ECHOCARDIOGRAPHY; DRUG EFFICACY; DYSPEPSIA; EPISTAXIS; HEADACHE; HEART CATHETERIZATION; HEART LEFT VENTRICLE FAILURE; HERITABILITY; HOT FLUSH; HUMAN; HUMAN IMMUNODEFICIENCY VIRUS INFECTION; HYPOXEMIA; IDIOPATHIC DISEASE; NONHUMAN; OXYGEN THERAPY; PORTOPULMONARY HYPERTENSION; PULMONARY ARTERY OCCLUSION PRESSURE; PULMONARY HYPERTENSION; RANDOMIZED CONTROLLED TRIAL (TOPIC); REVIEW; SCHISTOSOMIASIS; SYSTOLIC BLOOD PRESSURE; TREATMENT INDICATION; TRICUSPID VALVE REGURGITATION; WORLD HEALTH ORGANIZATION;

EID: 84940102375     PISSN: 11766336     EISSN: 1178203X     Source Type: Journal    
DOI: 10.2147/TCRM.S74881     Document Type: Review

References (95)

1. Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25_S):D34–D41.
2. Mozaffarian D, Benjamin EJ, Go AS, et al; American Heart Association Statistics Committee and Stroke Statistics Subcommittee. Heart disease and stroke statistics – 2015 update: a report from the American Heart Association. Circulation. 2015;131(4):e29–e322.
3. Tuder RM, Archer SL, Dorfmüller P, et al. Relevant issues in the pathology and pathobiology of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25):D4–D12.
4. Humbert M, Morrell NW, Archer SL, et al. Cellular and molecular pathobiology of pulmonary arterial hypertension. J Am Coll Cardiol. 2004;43(12 suppl):S13–S24.
5. Galiè N, Hoeper MM, Humbert M, et al; Task Force for Diagnosis and Treatment of Pulmonary Hypertension of European Society of Cardiology (ESC); European Respiratory Society (ERS); International Society of Heart and Lung Transplantation (ISHLT). Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2009;34(6):1219–1263.
6. Fisher MR, Forfia PR, Chamera E, et al. Accuracy of Doppler echocardiography in the hemodynamic assessment of pulmonary hypertension. Am J Respir Crit Care Med. 2009;179:615–621.
7. Janda S, Shahidi N, Gin K, Swiston J. Diagnostic accuracy of echocardiography for pulmonary hypertension: a systematic review and meta-analysis. Heart. 2011;97:612–622.
8. Raymond RJ, Hinderliter AL, Willis PW, et al. Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. JAm Coll Cardiol. 2002;39(7):1214–1219.
9. Sauler M, Fares WH, Trow TK. Standard nonspecific therapies in the management of pulmonary arterial hypertension. Clin Chest Med. 2013;34(4):799–810.
10. Maron BA, Waxman AB, Opotowsky AR, et al. Effectiveness of spironolactone plus ambrisentan for treatment of pulmonary arterial hypertension (from the [ARIES] study 1 and 2 trials). Am J Cardiol. 2013;112(5):720–725.
11. Maron BA, Leopold JA. The role of the renin-angiotensin-aldosterone system in the pathobiology of pulmonary arterial hypertension (2013 Grover Conference series). Pulm Circ. 2014;4:200–210.
12. McLaughlin VV, Archer SL, Badesch DB, et al; American College of Cardiology Foundation Task Force on Expert Consensus Documents; American Heart Association; American College of Chest Physicians; American Thoracic Society, Inc; Pulmonary Hypertension Association. ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol. 2009;53(17):1573–1619.
13. Rich S, Kaufmann E, Levy PS. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med. 1992;327(2):76–81.
14. Fares WH, Trow TK. Targeted approaches to the treatment of pulmonary hypertension. Ther Adv Respir Dis. 2012;6:147–159.
15. Ruan CH, Dixon RA, Willerson JT, Ruan KH. Prostacyclin therapy for pulmonary arterial hypertension. Tex Heart Inst J. 2010;37(4):391–399.
16. Rubin LJ, Mendoza J, Hood M, et al. Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trial. Ann Intern Med. 1990;112(7):485–491.
17. Barst RJ, Rubin LJ, Long WA, et al; Primary Pulmonary Hypertension Study Group. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med. 1996;334(5):296–301.
18. Badesch DB, Tapson VF, McGoon MD, et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med. 2000;132:425–434.
19. Benza RL, Tapson VF, Gomberg-Maitland M, Poms A, Barst RJ, McLaughlin VV. One-year experience with intravenous treprostinil for pulmonary arterial hypertension. J Heart Lung Transplant. 2013;32(9):889–896.
20. Jing ZC, Parikh K, Pulido T, et al. Efficacy and safety of oral treprostinil monotherapy for the treatment of pulmonary arterial hypertension: arandomized, controlled trial. Circulation. 2013;127(5):624–633.
21. Krishnan U, Takatsuki S, Ivy DD, et al. Effectiveness and safety of inhaled treprostinil for the treatment of pulmonary arterial hypertension in children. Am J Cardiol. 2012;110(11):1704–1709.
22. Fares WH. Orenitram…. not verified. Am J Respir Crit Care Med. 2015;191(6):713–714.
23. Kholdani CA, Fares WH, Trow TK. Macitentan for the treatment of pulmonary arterial hypertension. Vasc Health Risk Manag. 2014;10:665–673.
24. Badesch DB, Feldman J, Keogh A, et al; ARIES-3 Study Group. ARIES-3: ambrisentan therapy in a diverse population of patients with pulmonary hypertension. Cardiovasc Ther. 2012;30(2):93–99.
25. Galie N, Olschewski H, Oudiz RJ, et al; Ambrisentan in Pulmonary Arterial Hypertension, Randomized, Double-Blind, Placebo-Controlled, Multicenter, Efficacy Studies (ARIES) Group. Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation. 2008;117:3010–3019.
26. Pulido T, Adzerikho I, Channick RN, et al; SERAPHIN Investigators. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med. 2013;369(9):809–818.
27. Fan YF, Zhang R, Jiang X, et al. The phosphodiesterase-5 inhibitor vardenafil reduces oxidative stress while reversing pulmonary arterial hypertension. Cardiovasc Res. 2013;99:395–403.
28. Galiè N, Brundage BH, Ghofrani HA, et al; Pulmonary Arterial Hypertension and Response to Tadalafil (PHIRST) Study Group. Tadalafil therapy for pulmonary arterial hypertension. Circulation. 2009;119(22):2894–2903.
29. Ghofrani HA, Galiè N, Grimminger F, et al; PATENT-1 Study Group. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med. 2013;369(4):330–340.
30. 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;141:354–362.
31. Galiè N, Corris PA, Frost A, et al. Updated treatment algorithm of pulmonary arterial hypertension. J Am Coll Cardiol. 2013;62(25 suppl):D60–D72.
32. Rubin L, Barbera J, Frost A, et al. Upfront combination therapy with ambrisentan and tadalafiliin treatment naive patients with Pulmonary Arterial Hypertension (PAH): subgroup analyses by Functional Class (FC), etiology, and region from the AMBITION Study. Chest. 2014;146:339A–339A. 4_MeetingAbstracts.
33. Raghu G, Behr J, Brown KK, et al; ARTEMIS-IPF Investigators. Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomized trial. Ann Intern Med. 2013;158:641–649.
34. Taichman DB, Ornelas J, Chung L, et al. Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report. Chest. 2014;146:449–475.
35. Doyle RL, McCrory D, Channick RN, Simonneau G, Conte J. Surgical treatments/interventions for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004;126:63S–71S.
36. Peacock AJ, Murphy NF, McMurray JJV, Caballero L, Stewart S. An epidemiological study of pulmonary arterial hypertension. Eur Respir J. 2007;30(1):104–109.
37. 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;173:1023–1030.
38. Cogan JD, Pauciulo MW, Batchman AP, et al. High frequency of BMPR2 exonic deletions/duplications in familial pulmonary arterial hypertension. Am J Respir Crit Care Med. 2006;174:590–598.
39. Chida A, Shintani M, Nakayama T, et al. Missense mutations of the BMPR1B (ALK6) gene in childhood idiopathic pulmonary arterial hypertension. Circ J. 2012;76:1501–1508.
40. Thomson JR, Machado RD, Pauciulo MW, et al. Sporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR-II, a receptor member of the TGF-beta family. J Med Genet. 2000;37(10):741–745.
41. Austin ED, Loyd JE, Phillips JA. Heritable pulmonary arterial hypertension. GeneReviews(R). Seattle WA: University of Washington, Seattle; 2015.
42. Eddahibi S, Adnot S. Anorexigen-induced pulmonary hypertension and the serotonin (5-HT) hypothesis: lessons for the future in pathogenesis. Respir Res. 2002;3:9.
43. Montani D, Seferian A, Savale L, Simonneau G, Humbert M. Drug-induced pulmonary arterial hypertension: a recent outbreak. Eur Respir Rev. 2013;22:244–250.
44. Goldberg A. Pulmonary arterial hypertension in connective tissue diseases. Cardiol Rev. 2010;18:85–88.
45. Kamel SR, Omar GM, Darwish AF, Asklany HT, Ellabban AS. Asymptomatic pulmonary hypertension in systemic lupus erythematosus. Clin Med Insights Arthritis Musculoskelet Disord. 2011;4:77–86.
46. Badesch DB, Raskob GE, Elliott CG, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL registry. Chest. 2010;137:376–387.
47. McGoon MD, Miller DP. REVEAL: a contemporary US pulmonary arterial hypertension registry. European Respiratory Review. 2012;21(123):8–18.
48. Chung L, Liu J, Parsons L, et al. Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: identifying systemic sclerosis as a unique phenotype. Chest. 2010;138:1383–1394.
49. Steen V, Medsger TA Jr. Predictors of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement. Arthritis Rheum. 2003;48(2):516–522.
50. Xia YK, Tu SH, Hu YH, et al. Pulmonary hypertension in systemic lupus erythematosus: a systematic review and analysis of 642 cases in Chinese population. Rheumatol Int. 2013;33(5):1211–1217.
51. Dhala A. Pulmonary arterial hypertension in systemic lupus erythematosus: current status and future direction. Clin Dev Immunol. 2012;2012:854941.
52. Barnett CF, Hsue PY. Human immunodeficiency virus-associated pulmonary arterial hypertension. Clin Chest Med. 2013;34(2):283–292.
53. Selby VN, Scherzer R, Barnett CF, et al. Doppler echocardiography does not accurately estimate pulmonary artery systolic pressure in HIV-infected patients. AIDS. 2012;26(15):1967–1969.
54. Pugliese A, Isnardi D, Saini A, Scarabelli T, Raddino R, Torre D. Impact of highly active antiretroviral therapy in HIV-positive patients with cardiac involvement. J Infect. 2000;40(3):282–284.
55. Degano B, Guillaume M, Savale L, et al. HIV-associated pulmonary arterial hypertension: survival and prognostic factors in the modern therapeutic era. AIDS. 2010;24(1):67–75.
56. Gary-Bobo G, Houssaini A, Amsellem V, et al. Effects of HIV protease inhibitors on progression of monocrotaline- and hypoxia-induced pulmonary hypertension in rats. Circulation. 2010;122(19):1937–1947.
57. Thompson MA, Aberg JA, Hoy JF, et al. Antiretroviral treatment of adult HIV infection: 2012 Recommendations of the International Antiviral Society-USA panel. JAMA. 2012;308(4):387–402.
58. Ieong MH, Farber HW. Noninfectious pulmonary complications of HIV. Clin Pulm Med. 2006;13(3):194–202.
59. Schumacher YO, Zdebik A, Huonker M, Kreisel W. Sildenafil in HIV-related pulmonary hypertensin [3]. AIDS. 2001;15(13):1747–1748.
60. Aguilar RV, Farber HW. Epoprostenol (Prostacyclin) therapy in HIV-associated pulmonary hypertension. Am J Respir Crit Care Med. 2000;162(5):1846–1850.
61. Myers PO, Tissot C, Beghetti M. Assessment of operability of patients with pulmonary arterial hypertension associated with congenital heart disease. Circ J. 2014;78:4–11.
62. Berger RM, Beghetti M, Humpl T, et al. Clinical features of paediatric pulmonary hypertension: a registry study. Lancet. 2012;379(9815):537–546.
63. Galie N, Manes A, Palazzini M, et al. Management of pulmonary arterial hypertension associated with congenital systemic-to-pulmonary shunts and Eisenmenger’s syndrome. Drugs. 2008;68:1049–1066.
64. McDonnell PJ, Toye PA, Hutchins GM. Primary pulmonary hypertension and cirrhosis: are they related? Am Rev Respir Dis. 1983;127(4):437–441.
65. Hoeper MM, Krowka MJ, Strassburg CP. Portopulmonary hypertension and hepatopulmonary syndrome. Lancet. 2004;363:1461–1468.
66. Fuster V, Steele PM, Edwards WD, Gersh BJ, McGoon MD, Frye RL. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation. 1984;70(4):580–587.
67. Kuo PC, Johnson LB, Plotkin JS, Howell CD, Bartlett ST, Rubin LJ. Continuous intravenous infusion of epoprostenol for the treatment of portopulmonary hypertension. Transplantation. 1997;63(4):604–606.
68. Plotkin JS, Kuo PC, Rubin LJ, et al. Successful use of chronic epoprostenol as a bridge to liver transplantation in severe portopulmonary hypertension. Transplantation. 1998;65(4):457–459.
69. Graham BB, Bandeira AP, Morrell NW, Butrous G, Tuder RM. Schistosomiasis-associated pulmonary hypertension: pulmonary vascular disease: the global perspective. Chest. 2010;137:20S–29S.
70. Ghio S, Gavazzi A, Campana C, et al. Independent and additive prognostic value of right ventricular systolic function and pulmonary artery pressure in patients with chronic heart failure. J Am Coll Cardiol. 2001;37:183–188.
71. Vahanian A, Baumgartner H, Bax J, et al. Guidelines on the management of valvular heart disease. Eur Heart J. 2007;28(2):230–268.
72. Redfield MM, Chen HH, Borlaug BA, et al; RELAX Trial. Effect of phosphodiesterase-5 inhibition on exercise capacity and clinical status in heart failure with preserved ejection fraction: a randomized clinical trial. JAMA. 2013;309(12):1268–1277.
73. Seeger W, Adir Y, Barberà JA, et al. Pulmonary hypertension in chronic lung diseases. J Am Coll Cardiol. 2013;62(25, suppl):D109–D116.
74. Scharf SM, Iqbal M, Keller C, Criner G, Lee S, Fessler HE. Hemodynamic characterization of patients with severe emphysema. Am J Respir Crit Care Med. 2002;166(3):314–322.
75. Eddahibi S, Chaouat A, Morrell N, et al. Polymorphism of the serotonin transporter gene and pulmonary hypertension in chronic obstructive pulmonary disease. Circulation. 2003;108(15):1839–1844.
76. Chaouat A, Bugnet AS, Kadaoui N, et al. Severe pulmonary hypertension and chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 2005;172:189–194.
77. Oswald-Mammosser M, Weitzenblum E, Quoix E, et al. Prognostic factors in COPD patients receiving long-term oxygen therapy. Importance of pulmonary artery pressure. Chest. 1995;107(5):1193–1198.
78. Lettieri CJ, Nathan SD, Barnett SD, Ahmad S, Shorr AF. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest. 2006;129:746–752.
79. 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(3):650–656.
80. Kimura M, Taniguchi H, Kondoh Y, et al. Pulmonary hypertension as a prognostic indicator at the initial evaluation in idiopathic pulmonary fibrosis. Respiration. 2013;85(6):456–463.
81. Nathan SD, Shlobin OA, Ahmad S, et al. Serial development of pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Respiration. 2008;76(3):288–294.
82. Kholdani C, Fares WH, Mohsenin V. Pulmonary hypertension in obstructive sleep apnea: is it clinically significant? A critical analysis of the association and pathophysiology. Pulm Circ. 2015;5(2):220–227.
83. Corte TJ, Wort SJ, Gatzoulis MA, et al. Elevated brain natriuretic peptide predicts mortality in interstitial lung disease. Eur Respir J. 2010;36(4):819–825.
84. Collard HR, Anstrom KJ, Schwarz MI, Zisman DA. Sildenafil improves walk distance in idiopathic pulmonary fibrosis. Chest. 2007;131(3):897–899.
85. 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):620–628.
86. Blanco I, Gimeno E, Munoz PA, et al. Hemodynamic and gas exchange effects of sildenafil in patients with chronic obstructive pulmonary disease and pulmonary hypertension. Am J Respir Crit Care Med. 2010;181(3):270–278.
87. 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;42:1622–1632.
88. Pengo V, Lensing AW, Prins MH, et al; Thromboembolic Pulmonary Hypertension Study Group. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med. 2004;350:2257–2264. [Massachusetts Medical Society].
89. Tunariu N, Gibbs SJ, Win Z, et al. Ventilation-perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic thromboembolic pulmonary disease as a treatable cause of pulmonary hypertension. J Nucl Med. 2007;48:680–684.
90. Mayer E, Jenkins D, Lindner J, et al. Surgical management and outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. J Thorac Cardiovasc Surg. 2011;141:702–710.
91. Freed DH, Thomson BM, Berman M, et al. Survival after pulmonary thromboendarterectomy: effect of residual pulmonary hypertension. J Thorac Cardiovasc Surg. 2011;141:383–387. [United States: A 2011 The American Association for Thoracic Surgery. Published by Mosby, Inc.].
92. Bonderman D, Skoro-Sajer N, Jakowitsch J, et al. Predictors of outcome in chronic thromboembolic pulmonary hypertension. Circulation. 2007;115:2153–2158.
93. Jaïs X, D’Armini AM, Jansa P, et al; Bosentan Effects in iNopErable Forms of chronIc Thromboembolic pulmonary hypertension Study Group. Bosentan for treatment of inoperable chronic thromboembolic pulmonary hypertension: BENEFiT (Bosentan effects in iNopErable forms of chronIc thromboembolic pulmonary hypertension), a randomized, placebo-controlled trial. J Am Coll Cardiol. 2008;52:2127–2134.
94. Ghofrani H-A, D’Armini AM, Grimminger F, et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med. 2013;369(4):319–329.
95. Hoeper MM, Bogaard HJ, Condliffe R, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25_S):D42–D50.