Idiopathic pulmonary arterial hypertension

References

Key articles

Badesch DB, Champion HC, Sanchez MA, et al. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2009 Jun 30;54(1 Suppl):S55-66.Full text  Abstract

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 Jan 1;37(1):67-119.Full text  Abstract

Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med. 1987 Aug;107(2):216-23. Abstract

Reference articles

1. Humbert M, Sitbon O, Simmonnau G. Treatment of pulmonary arterial hypertension. N Engl J Med. 2004 Sep 30;351(14):1425-36. Abstract

2. Badesch DB, Champion HC, Sanchez MA, et al. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2009 Jun 30;54(1 Suppl):S55-66.Full text  Abstract

3. Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D34-41.Full text  Abstract

4. 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 Jan 1;37(1):67-119.Full text  Abstract

5. McLaughlin VV, McGoon MD. Pulmonary arterial hypertension. Circulation. 2006 Sep 26;114(13):1417-31.Full text  Abstract

6. 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 May 1;173(9):1023-30.Full text  Abstract

7. Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med. 1987 Aug;107(2):216-23. Abstract

8. Badesch DB, Raskob GE, Elliott CG, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest. 2010 Feb;137(2):376-87. Abstract

9. Lane KB, Machado RD, Pauciulo MW, et al; International PPH Consortium. Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension. Nat Genet. 2000 Sep;26(1):81-4. Abstract

10. Austin ED, Loyd JE. Genetics and mediators in pulmonary arterial hypertension. Clin Chest Med. 2007 Mar;28(1):43-57. Abstract

11. Ma L, Roman-Campos D, Austin ED, et al. A novel channelopathy in pulmonary arterial hypertension. N Engl J Med. 2013 Jul 25;369(4):351-61. Abstract

12. Speich R, Jenni R, Opravil M, et al. Primary pulmonary hypertension in HIV infection. Chest. 1991 Nov;100(5):1268-71. Abstract

13. Abenhaim L, Moride Y, Brenot F, et al. Appetite-suppressant drugs and the risk of primary pulmonary hypertension. International Primary Pulmonary Hypertension Study group. N Engl J Med. 1996 Aug 29;335(9):609-16. Abstract

14. Dorfmuller P, Perros F, Balabanian K, et al. Inflammation in pulmonary arterial hypertension. Eur Respir J. 2003 Aug;22(2):358-63. Abstract

15. Farber HW, Loscalzo J. Pulmonary arterial hypertension. N Engl J Med. 2004 Oct 14;351(16):1655-65. Abstract

16. Christman BW, McPherson CD, Newman JH, et al. An imbalance between the excretion of thromboxane and prostacyclin metabolites in pulmonary hypertension. N Engl J Med. 1992 Jul 9;327(2):70-5. Abstract

17. Tuder RM, Cool CD, Geraci MW, et al. Prostacyclin synthase expression is decreased in lungs from patients with severe pulmonary hypertension. Am J Respir Crit Care Med. 1999 Jun;159(6):1925-32. Abstract

18. Stewart DJ, Levy RD, Cernacek P, et al. Increased plasma endothelin-1 in pulmonary hypertension: marker or mediator of disease? Ann Intern Med. 1991 Mar 15;114(6):464-9. Abstract

19. Rubens C, Ewert R, Halank M, et al. Big endothelin-1 and endothelin-1 plasma levels are correlated with the severity of primary pulmonary hypertension. Chest. 2001 Nov;120(5):1562-9. Abstract

20. Giaid A, Yanagisawa M, Langleben D, et al. Expression of endothelin-1 in the lungs of patients with pulmonary hypertension. N Engl J Med. 1993 Jun 17;328(24):1732-9. Abstract

21. Dweik RA, Erzurum SC. Effects of nitric oxide and cyclic GMP on smooth muscle proliferation. In: Moss J, ed. LAM and other diseases characterized by smooth muscle proliferation. Lung biology in health and disease series. New York, NY: Marcel Dekker; 1999:131;333-49.

22. Kaneko FT, Arroliga AC, Dweik RA, et al. Biochemical reaction products of nitric oxide as quantitative markers of primary pulmonary hypertension. Am J Respir Crit Care Med. 1998 Sep;158(3):917-23. Abstract

23. Ozkan M, Dweik RA, Laskowski D, et al. High levels of nitric oxide in individuals with pulmonary hypertension receiving epoprostenol therapy. Lung. 2001;179(4):233-43. Abstract

24. Giaid A, Saleh D. Reduced expression of endothelial nitric oxide synthase in the lungs of patients with pulmonary hypertension. N Engl J Med. 1995 Jul 27;333(4):214-21. Abstract

25. Xu W, Kaneko FT, Zheng S, et al. Increased arginase II and decreased NO synthesis in endothelial cells of patients with pulmonary arterial hypertension. FASEB J. 2004 Nov;18(14):1746-8. Abstract

26. Schermuly RT, Dony E, Ghofrani HA, et al. Reversal of experimental pulmonary hypertension by PDGF inhibition. J Clin Invest. 2005 Oct;115(10):2811-21. Abstract

27. Yuan JX, Aldinger AM, Juhaszova M, et al. Dysfunctional voltage-gated K+ channels in pulmonary artery smooth muscle cells of patients with primary pulmonary hypertension. Circulation. 1998 Oct 6;98(14):1400-6. Abstract

28. Humbert M, Morrell NW, Archer SL, et al. Cellular and molecular pathobiology of pulmonary arterial hypertension. J Am Coll Cardiol. 2004 Jun 16;43(12 Suppl S):13-24S. Abstract

29. Taichman DB, Mandel J. Epidemiology of pulmonary arterial hypertension. Clin Chest Med. 2007 Mar;28(1):1-22. Abstract

30. Humbert M, Nunes H, Sitbon O, et al. Risk factors for pulmonary arterial hypertension. Clin Chest Med. 2001 Sep;22(3):459-75. Abstract

31. Rich JD, Rich S. Clinical diagnosis of pulmonary hypertension. Circulation. 2014 Nov 11;130(20):1820-30.Full text  Abstract

32. Badesch DB, Abman SH, Simonneau G, et al. Medical therapy for pulmonary arterial hypertension: updated ACCP evidence-based clinical practice guidelines. Chest. 2007 Jun;131(6):1917-28. Abstract

33. Sitbon O, Humbert M, Jais X, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation. 2005 Jun 14;111(23):3105-11.Full text  Abstract

34. Trow TK, McArdle JR. Diagnosis of pulmonary arterial hypertension. Clin Chest Med. 2007 Mar;28(1):59-73. Abstract

35. Chin KM, Channick RN, Rubin LJ. Is methamphetamine use associated with idiopathic pulmonary arterial hypertension? Chest. 2006 Dec;130(6):1657-63. Abstract

36. Ahearn GS, Tapson VF, Rebeiz A, et al. Electrocardiography to define clinical status in primary pulmonary hypertension and pulmonary arterial hypertension secondary to collagen vascular disease. Chest. 2002 Aug;122(2):524-7. Abstract

37. McGoon M, Gutterman D, Steen V, et al. Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004 Jul;126(1 Suppl):14-34S. Abstract

38. Minai OA, Pandya CM, Golish JA, et al. Predictors of nocturnal oxygen desaturation in pulmonary arterial hypertension. Chest. 2007 Jan;131(1):109-17. Abstract

39. Auger WR, Kim NH, Kerr KM, et al. Chronic thromboembolic pulmonary hypertension. Clin Chest Med. 2007 Mar;28(1):255-69. Abstract

40. Landzberg MJ. Congenital heart disease associated pulmonary arterial hypertension. Clin Chest Med. 2007 Mar;28(1):243-53. Abstract

41. Golbin JM, Krowka MJ. Portopulmonary hypertension. Clin Chest Med. 2007 Mar;28(1):203-18. Abstract

42. Hoeper MM, Bogaard HJ, Condliffe R, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D42-50.Full text  Abstract

43. McLaughlin VV, Gaine SP, Howard LS, et al. Treatment goals of pulmonary hypertension. J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D73-81.Full text  Abstract

44. Galiè N, Corris PA, Frost A, et al. Updated treatment algorithm of pulmonary arterial hypertension. J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D60-72.Full text  Abstract

45. 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 Feb 1;334(5):296-301.Full text  Abstract

46. Pulido T, Adzerikho I, Channick RN, et al; SERAPHIN Investigators. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med. 2013 Aug 29;369(9):809-18.Full text  Abstract

47. Galiè N, Ghofrani HA, Torbicki A, et al; Sildenafil Use in Pulmonary Arterial Hypertension (SUPER) Study Group. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med. 2005 Nov 17;353(20):2148-57.Full text  Abstract

48. 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 Jun 9;119(22):2894-903.Full text  Abstract

49. 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 Jul 25;369(4):330-40.Full text  Abstract

50. Sitbon O, Channick R, Chin KM, et al; GRIPHON Investigators. Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med. 2015 Dec 24;373(26):2522-33.Full text  Abstract

51. European Medicines Agency. EMA concludes safety review of Uptravi. Apr 2017 [internet publication].Full text

52. Liu HL, Chen XY, Li JR, et al. Efficacy and safety of pulmonary arterial hypertension-specific therapy in pulmonary arterial hypertension: a meta-analysis of randomized controlled trials. Chest. 2016 Aug;150(2):353-66. Abstract

53. O'Callaghan D, Gaine SP. Combination therapy and new types of agents for pulmonary arterial hypertension. Clin Chest Med. 2007 Mar;28(1):169-85. Abstract

54. Hoeper MM, Markevych I, Spiekerkoetter E, et al. Goal-oriented treatment and combination therapy for pulmonary arterial hypertension. Eur Respir J. 2005 Nov;26(5):858-63. Abstract

55. McLaughlin VV, Oudiz RJ, Frost A, et al. Randomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertension. Am J Respir Crit Care Med. 2006 Dec 1;174(11):1257-63.Full text  Abstract

56. Hoeper MM, Leuchte H, Halank M, et al. Combining inhaled iloprost with bosentan in patients with idiopathic pulmonary arterial hypertension. Eur Respir J. 2006 Oct;28(4):691-4.Full text  Abstract

57. McLaughlin VV, Benza RL, Rubin LJ, et al. Addition of inhaled treprostinil to oral therapy for pulmonary arterial hypertension: a randomized controlled clinical trial. J Am Coll Cardiol. 2010 May 4;55(18):1915-22. Abstract

58. Simonneau G, Rubin LJ, Galiè N, et al; PACES Study Group. Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial. Ann Intern Med. 2008 Oct 21;149(8):521-30. Abstract

59. Barst RJ, Oudiz RJ, Beardsworth A, et al; Pulmonary Arterial Hypertension and Response to Tadalafil (PHIRST) Study Group. Tadalafil monotherapy and as add-on to background bosentan in patients with pulmonary arterial hypertension. J Heart Lung Transplant. 2011 Jun;30(6):632-43. Abstract

60. Bai Y, Sun L, Hu S, et al. Combination therapy in pulmonary arterial hypertension: a meta-analysis. Cardiology. 2011;120(3):157-65. Abstract

61. Fox BD, Shimony A, Langleben D. Meta-analysis of monotherapy versus combination therapy for pulmonary arterial hypertension. Am J Cardiol. 2011 Oct 15;108(8):1177-82. Abstract

62. Coeytaux RR, Schmit KM, Kraft BD, et al. Comparative effectiveness and safety of drug therapy for pulmonary arterial hypertension: a systematic review and meta-analysis. Chest. 2014 May;145(5):1055-63. Abstract

63. Fox BD, Shtraichman O, Langleben D, et al. Combination therapy for pulmonary arterial hypertension: a systematic review and meta-analysis. Can J Cardiol. 2016 Mar 17;32(12):1520-30. Abstract

64. Lajoie AC, Lauzière G, Lega JC, et al. Combination therapy versus monotherapy for pulmonary arterial hypertension: a meta-analysis. Lancet Respir Med. 2016 Feb 27;4(4):291-305. [Erratum in: Lancet Respir Med. 2016 Jun;4(6):e34.] Abstract

65. McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension. J Am Coll Cardiol. 2009 Apr 28;53(17):1573-619. Abstract

66. Alam S, Palevsky HI. Standard therapies for pulmonary arterial hypertension. Clin Chest Med. 2007 Mar;28(1):91-115. Abstract

67. Olsson KM, Delcroix M, Ghofrani HA, et al. Anticoagulation and survival in pulmonary arterial hypertension: results from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA). Circulation. 2014 Jan 7;129(1):57-65.Full text  Abstract

68. Galiè N, Barbera JA, Frost AE, et al AMBITION Investigators. Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension. N Engl J Med. 2015 Aug 27;373(9):834-44.Full text  Abstract

69. Galiè N, Olschewski H, Oudiz RJ, et al. 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 Jun 10;117(23):3010-9.Full text  Abstract

70. Galiè N, Badesch D, Oudiz R, et al. Ambrisentan therapy for pulmonary arterial hypertension. J Am Coll Cardiol. 2005 Aug 2;46(3):529-35. Abstract

71. Spiekerkoetter E, Tian X, Cai J, et al. FK506 activates BMPR2, rescues endothelial dysfunction, and reverses pulmonary hypertension. J Clin Invest. 2013 Aug;123(8):3600-13.Full text  Abstract

72. Spiekerkoetter E, Sung YK, Sudheendra D, et al. Low-dose FK506 (tacrolimus) in end-stage pulmonary arterial hypertension. Am J Respir Crit Care Med. 2015 Jul 15;192(2):254-7. Abstract

73. Tian W, Jiang X, Tamosiuniene R, et al. Blocking macrophage leukotriene b4 prevents endothelial injury and reverses pulmonary hypertension. Sci Transl Med. 2013 Aug 28;5(200):200ra117.Full text  Abstract

74. Taichman DB, Ornelas J, Chung L, et al. Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report. Chest. 2014 Aug;146(2):449-75.Full text  Abstract

75. 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 Sep 1;115(5):343-9. Abstract

76. McLaughlin VV, Presberg KW, Doyle RL, et al. Prognosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004 Jul;126(1 Suppl):78-92S. Abstract

77. McLaughlin VV, Sitbon O, Badesch DB, et al. Survival with first-line bosentan in patients with primary pulmonary hypertension. Eur Respir J. 2005 Feb;25(2):244-9. [Erratum in: Eur Respir J. 2005 May;25(5):942.]Full text  Abstract

78. Savarese G, Paolillo S, Costanzo P, et al. Do changes of 6-minute walk distance predict clinical events in patients with pulmonary arterial hypertension? A meta-analysis of 22 randomized trials. J Am Coll Cardiol. 2012 Sep 25;60(13):1192-201. Abstract

79. Galiè N, Manes A, Negro L, et al. A meta-analysis of randomized controlled trials in pulmonary arterial hypertension. Eur Heart J. 2009 Feb;30(4):394-403.Full text  Abstract

80. Benza RL, Miller DP, Barst RJ, et al. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest. 2012 Aug;142(2):448-56. Abstract

81. Benza RL, Miller DP, Gomberg-Maitland M, 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 Jul 13;122(2):164-72.Full text  Abstract

82. Humbert M, Sitbon O, Chaouat A, et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation. 2010 Jul 13;122(2):156-63.Full text  Abstract

83. Fritz JS, Blair C, Oudiz RJ, et al. Baseline and follow-up 6-min walk distance and brain natriuretic peptide predict 2-year mortality in pulmonary arterial hypertension. Chest. 2013 Feb 1;143(2):315-23. Abstract

84. Nickel N, Golpon H, Greer M, et al. The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension. Eur Respir J. 2012 Mar;39(3):589-96.Full text  Abstract

85. Tongers J, Schwerdtfeger B, Klein G, et al. Incidence and clinical relevance of supraventricular tachyarrhythmias in pulmonary hypertension. Am Heart J. 2007 Jan;153(1):127-32. Abstract

86. Doran AK, Ivy DD, Barst RJ, et al; Scientific Leadership Council of the Pulmonary Hypertension Association. Guidelines for the prevention of central venous catheter-related blood stream infections with prostanoid therapy for pulmonary arterial hypertension. Int J Clin Pract Suppl. 2008 Jul;(160):5-9.Full text  Abstract

Use of this content is subject to our disclaimer