特发性肺动脉高压

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.全文  摘要

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.]全文  摘要

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

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.] 摘要

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

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.全文  摘要

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

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.全文  摘要

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.]全文  摘要

4. Leber L, Beaudet A, Muller A. Epidemiology of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: identification of the most accurate estimates from a systematic literature review. Pulm Circ. 2021 Jan-Mar;11(1):1-12.全文  摘要

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

6. Lau EMT, Giannoulatou E, Celermajer DS, et al. Epidemiology and treatment of pulmonary arterial hypertension. Nat Rev Cardiol. 2017 Oct;14(10):603-14. 摘要

7. 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.全文  摘要

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. 摘要

9. 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. 摘要

10. Ling Y, Johnson MK, Kiely DG, 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 Oct 15;186(8):790-6.全文  摘要

11. 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. 摘要

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

13. 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.全文  摘要

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

15. 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. 摘要

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

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

18. 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. 摘要

19. 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. 摘要

20. 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. 摘要

21. 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. 摘要

22. 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. 摘要

23. Chester AH, Yacoub MH, Moncada S. Nitric oxide and pulmonary arterial hypertension. Glob Cardiol Sci Pract. 2017 Jun 30;2017(2):14.全文  摘要

24. 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. 摘要

25. 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. 摘要

26. 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. 摘要

27. 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. 摘要

28. 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. 摘要

29. 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. 摘要

30. 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.全文  摘要

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

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

33. 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.全文  摘要

34. 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.] 摘要

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

36. Rich JD, Rich S. Clinical diagnosis of pulmonary hypertension. Circulation. 2014 Nov 11;130(20):1820-30.全文  摘要

37. American College of Radiology. ACR appropriateness criteria: suspected pulmonary hypertension. 2022 [internet publication].全文

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

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

40. 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. 摘要

41. 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.全文  摘要

42. 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. 摘要

43. 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.全文  摘要

44. 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.全文  摘要

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

46. 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.全文  摘要

47. 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.]全文  摘要

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

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

50. 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.全文  摘要

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

52. 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.全文  摘要

53. 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.全文  摘要

54. 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.全文  摘要

55. 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.全文  摘要

56. 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.全文  摘要

57. 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. 摘要

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

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

60. 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.全文  摘要

61. 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.全文  摘要

62. 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.全文  摘要

63. 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.全文  摘要

64. 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.全文  摘要

65. 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.全文  摘要

66. 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.] 摘要

67. 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.全文  摘要

68. 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.全文  摘要

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

70. 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.全文  摘要

71. 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.全文  摘要

72. 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.全文  摘要

73. 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.全文  摘要

74. 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.全文  摘要

75. 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.全文  摘要

76. 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. 摘要

77. 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. 摘要

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

79. 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.全文  摘要

80. 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.全文  摘要

81. 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.全文  摘要

82. 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]. 摘要

83. 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.全文  摘要

84. 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.全文  摘要

85. 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. 摘要

86. 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.全文  摘要

87. 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. 摘要

88. 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. 摘要

89. 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.]全文  摘要

90. 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. 摘要

91. 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.全文  摘要

92. 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. 摘要

93. 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.全文  摘要

94. 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.全文  摘要

95. 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. 摘要

96. 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.全文  摘要

97. 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. 摘要

98. 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.全文  摘要

99. Zeiger T, Cobo GC, Dillingham C, et al. Prevalence of sodium and fluid restriction recommendations for patients with pulmonary hypertension. Healthcare (Basel). 2015 Jul 28;3(3):630-6.全文  摘要

100. 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].全文  摘要