Idiopathic pulmonary arterial hypertension

References

Key articles

Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019 Jan;53(1):1801913.Full text  Abstract

Humbert M, Kovacs G, Hoeper MM, et al; ESC/ERS Scientific Document Group. 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022 Oct 11;43(38):3618-731. [Erratum in: Eur Heart J. 2023 Feb 23:ehad005.]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

Klinger JR, Elliott CG, Levine DJ, et al. Therapy for pulmonary arterial hypertension in adults: update of the CHEST guideline and expert panel report. Chest. 2019 Mar;155(3):565-86. [Erratum in: Chest. 2021 Jan;159(1):457.] Abstract

Frost A, Badesch D, Gibbs JSR, et al. Diagnosis of pulmonary hypertension. Eur Respir J. 2019 Jan;53(1):1801904.Full text  Abstract

Galiè N, Channick RN, Frantz RP, et al. Risk stratification and medical therapy of pulmonary arterial hypertension. Eur Respir J. 2019 Jan;53(1):1801889.Full text  Abstract

Reference articles

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2. Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019 Jan;53(1):1801913.Full text  Abstract

3. Humbert M, Kovacs G, Hoeper MM, et al; ESC/ERS Scientific Document Group. 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022 Oct 11;43(38):3618-731. [Erratum in: Eur Heart J. 2023 Feb 23:ehad005.]Full text  Abstract

4. Humbert M, Sitbon O, Guignabert C, et al. Treatment of pulmonary arterial hypertension: recent progress and a look to the future. Lancet Respir Med. 2023 Sep;11(9):804-19.Full text  Abstract

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10. Hoeper MM, Huscher D, Ghofrani HA, et al. Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from the COMPERA registry. Int J Cardiol. 2013 Sep 30;168(2):871-80. Abstract

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30. 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

31. 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):S13-24.Full text  Abstract

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

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

34. Hlavaty A, Roustit M, Montani D, et al. Identifying new drugs associated with pulmonary arterial hypertension: a WHO pharmacovigilance database disproportionality analysis. Br J Clin Pharmacol. 2022 Dec;88(12):5227-37.Full text  Abstract

35. Klinger JR, Elliott CG, Levine DJ, et al. Therapy for pulmonary arterial hypertension in adults: update of the CHEST guideline and expert panel report. Chest. 2019 Mar;155(3):565-86. [Erratum in: Chest. 2021 Jan;159(1):457.] Abstract

36. Frost A, Badesch D, Gibbs JSR, et al. Diagnosis of pulmonary hypertension. Eur Respir J. 2019 Jan;53(1):1801904.Full text  Abstract

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

38. American College of Radiology. ACR appropriateness criteria: suspected pulmonary hypertension. 2022 [internet publication].Full text

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

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

41. 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

42. Farber HW, Foreman AJ, Miller DP, et al. REVEAL Registry: correlation of right heart catheterization and echocardiography in patients with pulmonary arterial hypertension. Congest Heart Fail. 2011 Mar-Apr;17(2):56-63.Full text  Abstract

43. 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

44. Tello K, Wan J, Dalmer A, et al. Validation of the tricuspid annular plane systolic excursion/systolic pulmonary artery pressure ratio for the assessment of right ventricular-arterial coupling in severe pulmonary hypertension. Circ Cardiovasc Imaging. 2019 Sep;12(9):e009047.Full text  Abstract

45. Yan L, Luo Q, Zhao Z, et al. Nocturnal hypoxia in patients with idiopathic pulmonary arterial hypertension. Pulm Circ. 2020 Jul-Sep;10(3):1-7.Full text  Abstract

46. 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

47. 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

48. Yeghiazarians Y, Jneid H, Tietjens JR, et al. Obstructive sleep apnea and cardiovascular disease: a scientific statement From the American Heart Association. Circulation. 2021 Jul 20;144(3):e56-67. [Erratum in: Circulation. 2022 Mar 22;145(12):e775.]Full text  Abstract

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

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

51. Vodoz JF, Cottin V, Glérant JC, et al. Right-to-left shunt with hypoxemia in pulmonary hypertension. BMC Cardiovasc Disord. 2009 Mar 31;9:15.Full text  Abstract

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

53. Barst RJ, McGoon M, Torbicki A, et al. Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2004 Jun 16;43(12 suppl):S40-7.Full text  Abstract

54. Hoeper MM, Kramer T, Pan Z, et al. Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. Eur Respir J. 2017 Aug;50(2):1700740.Full text  Abstract

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56. 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

57. Galiè N, Channick RN, Frantz RP, et al. Risk stratification and medical therapy of pulmonary arterial hypertension. Eur Respir J. 2019 Jan;53(1):1801889.Full text  Abstract

58. Rochester CL, Alison JA, Carlin B, et al. Pulmonary rehabilitation for adults with chronic respiratory disease: an official American Thoracic Society clinical practice guideline. Am J Respir Crit Care Med. 2023 Aug 15;208(4):e7-26.Full text  Abstract

59. Grünig E, Eichstaedt C, Barberà JA, et al. ERS statement on exercise training and rehabilitation in patients with severe chronic pulmonary hypertension. Eur Respir J. 2019 Feb;53(2).Full text  Abstract

60. Morris NR, Kermeen FD, Jones AW, et al. Exercise-based rehabilitation programmes for pulmonary hypertension. Cochrane Database Syst Rev. 2023 Mar 22;3(3):CD011285.Full text  Abstract

61. 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.Full text  Abstract

62. 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

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

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65. Barnes H, Yeoh HL, Fothergill T, et al. Prostacyclin for pulmonary arterial hypertension. Cochrane Database Syst Rev. 2019 May 1;5(5):CD012785.Full text  Abstract

66. 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

67. Simonneau G, Barst RJ, Galie N, et al. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial. Am J Respir Crit Care Med. 2002 Mar 15;165(6):800-4.Full text  Abstract

68. Liu C, Chen J, Gao Y, et al. Endothelin receptor antagonists for pulmonary arterial hypertension. Cochrane Database Syst Rev. 2021 Mar 26;3(3):CD004434.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.Full text  Abstract

71. 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

72. Ruopp NF, Cockrill BA. Diagnosis and treatment of pulmonary arterial hypertension: a review. JAMA. 2022 Apr 12;327(14):1379-91. [Erratum in: JAMA. 2022 Sep 6;328(9):892.] Abstract

73. 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

74. 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

75. Barnes H, Brown Z, Burns A, et al. Phosphodiesterase 5 inhibitors for pulmonary hypertension. Cochrane Database Syst Rev. 2019 Jan 31;1(1):CD012621.Full text  Abstract

76. 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

77. 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

78. 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

79. Chin KM, Sitbon O, Doelberg M, et al. Three- versus two-drug therapy for patients with newly diagnosed pulmonary arterial hypertension. J Am Coll Cardiol. 2021 Oct 5;78(14):1393-403.Full text  Abstract

80. Haarman MG, Lévy M, Roofthooft MTR, et al. Upfront triple combination therapy in severe paediatric pulmonary arterial hypertension. Eur Respir J. 2021 Jan;57(1):2001120.Full text  Abstract

81. Sitbon O, Jaïs X, Savale L, et al. Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study. Eur Respir J. 2014 Jun;43(6):1691-7.Full text  Abstract

82. D'Alto M, Badagliacca R, Argiento P, et al. Risk reduction and right heart reverse remodeling by upfront triple combination therapy in pulmonary arterial hypertension. Chest. 2020 Feb;157(2):376-83. Abstract

83. Hoeper MM, Al-Hiti H, Benza RL, et al; REPLACE investigators. Switching to riociguat versus maintenance therapy with phosphodiesterase-5 inhibitors in patients with pulmonary arterial hypertension (REPLACE): a multicentre, open-label, randomised controlled trial. Lancet Respir Med. 2021 Jun;9(6):573-84. Abstract

84. Wardle AJ, Seager MJ, Wardle R, et al. Guanylate cyclase stimulators for pulmonary hypertension. Cochrane Database Syst Rev. 2016 Aug 2;2016(8):CD011205.Full text  Abstract

85. 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

86. Preston IR, Roberts KE, Miller DP, et al. Effect of warfarin treatment on survival of patients with pulmonary arterial hypertension (PAH) in the registry to evaluate early and long-term PAH disease management (REVEAL). Circulation. 2015 Dec 22;132(25):2403-11.Full text  Abstract

87. Humbert M, McLaughlin V, Gibbs JSR, et al; PULSAR Trial Investigators. Sotatercept for the treatment of pulmonary arterial hypertension. N Engl J Med. 2021 Apr 1;384(13):1204-15.Full text  Abstract

88. Hoeper MM, Badesch DB, Ghofrani HA, et al. Phase 3 trial of sotatercept for treatment of pulmonary arterial hypertension. N Engl J Med. 2023 Mar 6 [online ahead of print]. Abstract

89. 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 Mar 12;127(10):1128-38.Full text  Abstract

90. 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

91. 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

92. Spiekerkoetter E, Sung YK, Sudheendra D, et al. Randomised placebo-controlled safety and tolerability trial of FK506 (tacrolimus) for pulmonary arterial hypertension. Eur Respir J. 2017 Sep;50(3):1602449.Full text  Abstract

93. 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

94. 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

95. 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

96. 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

97. 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

98. 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

99. 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

100. 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

101. 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

102. 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

103. 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

104. 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

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106. Rajagopal S, Ruetzler K, Ghadimi K, et al. Evaluation and management of pulmonary hypertension in noncardiac surgery: A Scientific Statement from the American Heart Association. Circulation. 2023 Mar 16 [epub ahead of print].Full text  Abstract

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