肺動脈高壓的治療進展_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

徐婉婷 ^1,3 , 江曉琴 ² ,  朱濤 ¹

1. ;
2. ;
3. ;

Corresponding?author：

朱濤, Email: 739501155@qq.com

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DOI：

10.7507/1671-6205.202311023

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Citation： 徐婉婷, 江曉琴, 朱濤. 肺動脈高壓的治療進展. Chinese Journal of Respiratory and Critical Care Medicine, 2024, 23(4): 287-292. doi: 10.7507/1671-6205.202311023 Copy

1.	Maron BA, Hess E, Maddox TM, et al. Association of Borderline Pulmonary Hypertension With Mortality and Hospitalization in a Large Patient Cohort: Insights From the Veterans Affairs Clinical Assessment, Reporting, and Tracking Program. Circulation, 2016, 133(13): 1240-1248.
2.	Wissmüller M, Dohr J, Adler J, et al. Pulmonary hypertension associated with left heart disease. Herz, 2023, 48(4): 266-273.
3.	Lang IM, Delcroix M. Novel acquisitions in chronic thromboembolic pulmonary hypertension. Eur Heart J, 2023, 44(27): 2417-2419.
4.	Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J, 2019, 53(1): 1801913.
5.	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, 11(9): 804-819.
6.	Sitbon O, Humbert M, Ja?s X, et al. Long-Term Response to Calcium Channel Blockers in Idiopathic Pulmonary Arterial Hypertension. Circulation, 2005, 111(23): 3105-3111.
7.	Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J, 2023, 61(1): 2200879.
8.	Channick RN, Simonneau G, Sitbon O, et al. Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebocontrolled study. Lancet, 2001, 358(9288): 1119-1123.
9.	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, 117(23): 3010-3019.
10.	Pulido T, Adzerikho I, Channick RN, et al. Macitentan and Morbidity and Mortality in Pulmonary Arterial Hypertension. N Engl J Med, 2013, 369(9): 809-818.
11.	Shi Q, Wang Z, Yang N, et al. Sildenafil for adult Asian patients with pulmonary arterial hypertension: a systematic review and meta-analysis. Ann Palliat Med, 2022, 11(1): 339-351.
12.	Ghofrani H-A, Galiè N, Grimminger F, et al. Riociguat for the Treatment of Pulmonary Arterial Hypertension. N Engl J Med, 2013, 369(4): 330-340.
13.	Nakamura K, Saito Y, Akagi S, et al. Epoprostenol sodium for treatment of pulmonary arterial hypertension. Vasc Health Risk Manag, 2015: 265.
14.	Sitbon O, Channick R, Chin KM, et al. Selexipag for the Treatment of Pulmonary Arterial Hypertension. N Engl J Med, 2015, 373(26): 2522-2533.
15.	Lajoie AC, Lauzière G, Lega JC, et al. Combination therapy versus monotherapy for pulmonary arterial hypertension: a meta-analysis. Lancet Respir Med, 2016, 4(4): 291-305.
16.	Galiè N, Barberà JA, Frost AE, et al. Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension. N Engl J Med, 2015, 373(9): 834-844.
17.	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, 78(14): 1393-1403.
18.	Zhang Q, Zhu F, Shi G, et al. Maternal Outcomes Among Pregnant Women With Congenital Heart Disease-Associated Pulmonary Hypertension. Circulation, 2023, 147(7): 549-561.
19.	Luna-Lopez R, Segura De La Cal T, Sarnago Cebada F, et al. Triple vasodilator therapy in pulmonary arterial hypertension associated with congenital heart disease. Heart, 2023: heartjnl-2023-323015.
20.	Humbert M, McLaughlin V, Gibbs J S R, et al. Sotatercept for the Treatment of Pulmonary Arterial Hypertension. N Engl J Med, 2021, 384(13): 1204-1215.
21.	Hoeper MM, Badesch DB, Ghofrani HA, et al. Phase 3 Trial of Sotatercept for Treatment of Pulmonary Arterial Hypertension. N Engl J Med, 2023, 388(16): 1478-1490.
22.	Carvalho T. Merck bets on sotatercept in pulmonary arterial hypertension. Nat Med, 2023, 29(1): 2-3.
23.	Olsson KM, Fuge J, Park DH, et al. Effects of sotatercept on lung diffusion capacity and blood gases in patients with pulmonary arterial hypertension. Eur Respir J, 2023, 62(2): 2301070.
24.	Souza R, Badesch DB, Ghofrani HA, et al. Effects of sotatercept on haemodynamics and right heart function: analysis of the STELLAR trial. Eur Respir J, 2023, 62(3): 2301107.
25.	Zheng R, Xu T, Wang X, et al. Stem cell therapy in pulmonary hypertension: current practice and future opportunities. Eur Respir Rev, 2023, 32(169): 230112.
26.	Granton J, Langleben D, Kutryk MB, et al. Endothelial NO-Synthase Gene-Enhanced Progenitor Cell Therapy for Pulmonary Arterial Hypertension: The PHACeT Trial. Circ Res, 2015, 117(7): 645-654.
27.	Samaranayake CB, Kempny A, Naeije R, et al. Beta-blockade improves right ventricular diastolic function in exercising pulmonary arterial hypertension. Eur Respir J, 2023, 61(5): 2300144.
28.	Ryanto GRT, Suraya R, Nagano T. Mitochondrial Dysfunction in Pulmonary Hypertension. Antioxidants, 2023, 12(2): 372.
29.	Galkin A, Sitapara R, Clemons B, et al. Inhaled seralutinib exhibits potent efficacy in models of pulmonary arterial hypertension. Eur Respir J, 2022, 60(6): 2102356.
30.	Cullivan S, Gaine S, Sitbon O. New trends in pulmonary hypertension. Eur Respir Rev, 2023, 32(167): 220211.
31.	Morris NR, Kermeen FD, Jones AW, et al. Exercise-based rehabilitation programmes for pulmonary hypertension. Cochrane Airways Group. Cochrane Database Syst Rev, 2023, 2023(3).
32.	Mocumbi A, Humbert M, Saxena A, et al. Pulmonary hypertension. Nat Rev Dis Primers. 2024 Jan 4;10(1): 1.
33.	Bermejo J, Yotti R, García-Orta R, et al. Sildenafil for improving outcomes in patients with corrected valvular heart disease and persistent pulmonary hypertension: a multicenter, double-blind, randomized clinical trial. Eur Heart J, 2018 Apr 14;39(15): 1255-1264.
34.	Purvey M, Allen G. Managing acute pulmonary oedema. Aust Prescr, 2017, 40(2): 59-63.
35.	Ciarka A, Doan V, Velez-Roa S, et al. Prognostic significance of sympathetic nervous system activation in pulmonary arterial hypertension. Am J Respir Crit Care Med, 2010, 181(11): 1269-1275.
36.	Zhang H, Kan J, Zhang J, et al. 3-Year Outcome in Patients With Combined Precapillary and Postcapillary Pulmonary Hypertension: Results From PADN-5 Trial. JACC Heart Fail. 2023 Jun 12: S2213-1779(23)00252-4.
37.	Zhang H, Wei Y, Zhang C, et al. Pulmonary Artery Denervation for Pulmonary Arterial Hypertension: A Sham-Controlled Randomized PADN-CFDA Trial. JACC Cardiovasc Interv. 2022 Dec 12;15(23): 2412-2423.
38.	Shi Y, Liu J, Zhang R, et al. Targeting Endothelial ENO1 (Alpha-Enolase) -PI3K-Akt-mTOR Axis Alleviates Hypoxic Pulmonary Hypertension. Hypertension, 2023, 80(5): 1035-1047.
39.	Sugarman J, Weatherald J. Management of Pulmonary Hypertension Due to Chronic Lung Disease. Methodist Debakey Cardiovasc J. 2021 Jul 1;17(2): 124-133.
40.	Chaouat A, Naeije R, Weitzenblum E. Pulmonary hypertension in COPD. Eur Respir J. 2008 Nov;32(5): 1371-85.
41.	Vitulo P, Stanziola A, Confalonieri M, et al. Sildenafil in severe pulmonary hypertension associated with chronic obstructive pulmonary disease: A randomized controlled multicenter clinical trial. J Heart Lung Transplant. 2017 Feb;36(2): 166-174.
42.	Raghu G, Behr J, Brown KK, et al. Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomized trial. Ann Intern Med. 2013 May 7;158(9): 641-9.
43.	Nathan SD, Behr J, Collard HR, et al. Riociguat for idiopathic interstitial pneumonia-associated pulmonary hypertension (RISE-IIP): a randomised, placebo-controlled phase 2b study. Lancet Respir Med. 2019 Sep;7(9): 780-790.
44.	Barnes H, Brown Z, Burns A, et al. Phosphodiesterase 5 inhibitors for pulmonary hypertension. Cochrane Database Syst Rev. 2019 Jan 31;1(1): CD012621.
45.	Waxman A, Restrepo-Jaramillo R, Thenappan T, et al. Inhaled Treprostinil in Pulmonary Hypertension Due to Interstitial Lung Disease. N Engl J Med. 2021 Jan 28;384(4): 325-334.
46.	Hsieh WC, Jansa P, Huang WC, et al. Residual pulmonary hypertension after pulmonary endarterectomy: A meta-analysis. J Thorac Cardiovasc Surg. 2018 Sep;156(3): 1275-1287.
47.	Humbert M, Simonneau G, Pittrow D, et al. Oral anticoagulants (NOAC and VKA) in chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant. 2022 Jun;41(6): 716-721.
48.	Simonneau G, D'Armini AM, Ghofrani HA, et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension: a long-term extension study (CHEST-2). Eur Respir J. 2015 May;45(5): 1293-302.
49.	Sadushi-Kolici R, Jansa P, Kopec G, et al. Subcutaneous treprostinil for the treatment of severe non-operable chronic thromboembolic pulmonary hypertension (CTREPH): a double-blind, phase 3, randomised controlled trial. Lancet Respir Med. 2019 Mar;7(3): 239-248.
50.	Ghofrani HA, Simonneau G, D'Armini AM, et al. Macitentan for the treatment of inoperable chronic thromboembolic pulmonary hypertension (MERIT-1): results from the multicentre, phase 2, randomised, double-blind, placebo-controlled study. Lancet Respir Med. 2017 Oct;5(10): 785-794.

1. Maron BA, Hess E, Maddox TM, et al. Association of Borderline Pulmonary Hypertension With Mortality and Hospitalization in a Large Patient Cohort: Insights From the Veterans Affairs Clinical Assessment, Reporting, and Tracking Program. Circulation, 2016, 133(13): 1240-1248.
2. Wissmüller M, Dohr J, Adler J, et al. Pulmonary hypertension associated with left heart disease. Herz, 2023, 48(4): 266-273.
3. Lang IM, Delcroix M. Novel acquisitions in chronic thromboembolic pulmonary hypertension. Eur Heart J, 2023, 44(27): 2417-2419.
4. Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J, 2019, 53(1): 1801913.
5. 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, 11(9): 804-819.
6. Sitbon O, Humbert M, Ja?s X, et al. Long-Term Response to Calcium Channel Blockers in Idiopathic Pulmonary Arterial Hypertension. Circulation, 2005, 111(23): 3105-3111.
7. Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J, 2023, 61(1): 2200879.
8. Channick RN, Simonneau G, Sitbon O, et al. Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebocontrolled study. Lancet, 2001, 358(9288): 1119-1123.
9. 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, 117(23): 3010-3019.
10. Pulido T, Adzerikho I, Channick RN, et al. Macitentan and Morbidity and Mortality in Pulmonary Arterial Hypertension. N Engl J Med, 2013, 369(9): 809-818.
11. Shi Q, Wang Z, Yang N, et al. Sildenafil for adult Asian patients with pulmonary arterial hypertension: a systematic review and meta-analysis. Ann Palliat Med, 2022, 11(1): 339-351.
12. Ghofrani H-A, Galiè N, Grimminger F, et al. Riociguat for the Treatment of Pulmonary Arterial Hypertension. N Engl J Med, 2013, 369(4): 330-340.
13. Nakamura K, Saito Y, Akagi S, et al. Epoprostenol sodium for treatment of pulmonary arterial hypertension. Vasc Health Risk Manag, 2015: 265.
14. Sitbon O, Channick R, Chin KM, et al. Selexipag for the Treatment of Pulmonary Arterial Hypertension. N Engl J Med, 2015, 373(26): 2522-2533.
15. Lajoie AC, Lauzière G, Lega JC, et al. Combination therapy versus monotherapy for pulmonary arterial hypertension: a meta-analysis. Lancet Respir Med, 2016, 4(4): 291-305.
16. Galiè N, Barberà JA, Frost AE, et al. Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension. N Engl J Med, 2015, 373(9): 834-844.
17. 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, 78(14): 1393-1403.
18. Zhang Q, Zhu F, Shi G, et al. Maternal Outcomes Among Pregnant Women With Congenital Heart Disease-Associated Pulmonary Hypertension. Circulation, 2023, 147(7): 549-561.
19. Luna-Lopez R, Segura De La Cal T, Sarnago Cebada F, et al. Triple vasodilator therapy in pulmonary arterial hypertension associated with congenital heart disease. Heart, 2023: heartjnl-2023-323015.
20. Humbert M, McLaughlin V, Gibbs J S R, et al. Sotatercept for the Treatment of Pulmonary Arterial Hypertension. N Engl J Med, 2021, 384(13): 1204-1215.
21. Hoeper MM, Badesch DB, Ghofrani HA, et al. Phase 3 Trial of Sotatercept for Treatment of Pulmonary Arterial Hypertension. N Engl J Med, 2023, 388(16): 1478-1490.
22. Carvalho T. Merck bets on sotatercept in pulmonary arterial hypertension. Nat Med, 2023, 29(1): 2-3.
23. Olsson KM, Fuge J, Park DH, et al. Effects of sotatercept on lung diffusion capacity and blood gases in patients with pulmonary arterial hypertension. Eur Respir J, 2023, 62(2): 2301070.
24. Souza R, Badesch DB, Ghofrani HA, et al. Effects of sotatercept on haemodynamics and right heart function: analysis of the STELLAR trial. Eur Respir J, 2023, 62(3): 2301107.
25. Zheng R, Xu T, Wang X, et al. Stem cell therapy in pulmonary hypertension: current practice and future opportunities. Eur Respir Rev, 2023, 32(169): 230112.
26. Granton J, Langleben D, Kutryk MB, et al. Endothelial NO-Synthase Gene-Enhanced Progenitor Cell Therapy for Pulmonary Arterial Hypertension: The PHACeT Trial. Circ Res, 2015, 117(7): 645-654.
27. Samaranayake CB, Kempny A, Naeije R, et al. Beta-blockade improves right ventricular diastolic function in exercising pulmonary arterial hypertension. Eur Respir J, 2023, 61(5): 2300144.
28. Ryanto GRT, Suraya R, Nagano T. Mitochondrial Dysfunction in Pulmonary Hypertension. Antioxidants, 2023, 12(2): 372.
29. Galkin A, Sitapara R, Clemons B, et al. Inhaled seralutinib exhibits potent efficacy in models of pulmonary arterial hypertension. Eur Respir J, 2022, 60(6): 2102356.
30. Cullivan S, Gaine S, Sitbon O. New trends in pulmonary hypertension. Eur Respir Rev, 2023, 32(167): 220211.
31. Morris NR, Kermeen FD, Jones AW, et al. Exercise-based rehabilitation programmes for pulmonary hypertension. Cochrane Airways Group. Cochrane Database Syst Rev, 2023, 2023(3).
32. Mocumbi A, Humbert M, Saxena A, et al. Pulmonary hypertension. Nat Rev Dis Primers. 2024 Jan 4;10(1): 1.
33. Bermejo J, Yotti R, García-Orta R, et al. Sildenafil for improving outcomes in patients with corrected valvular heart disease and persistent pulmonary hypertension: a multicenter, double-blind, randomized clinical trial. Eur Heart J, 2018 Apr 14;39(15): 1255-1264.
34. Purvey M, Allen G. Managing acute pulmonary oedema. Aust Prescr, 2017, 40(2): 59-63.
35. Ciarka A, Doan V, Velez-Roa S, et al. Prognostic significance of sympathetic nervous system activation in pulmonary arterial hypertension. Am J Respir Crit Care Med, 2010, 181(11): 1269-1275.
36. Zhang H, Kan J, Zhang J, et al. 3-Year Outcome in Patients With Combined Precapillary and Postcapillary Pulmonary Hypertension: Results From PADN-5 Trial. JACC Heart Fail. 2023 Jun 12: S2213-1779(23)00252-4.
37. Zhang H, Wei Y, Zhang C, et al. Pulmonary Artery Denervation for Pulmonary Arterial Hypertension: A Sham-Controlled Randomized PADN-CFDA Trial. JACC Cardiovasc Interv. 2022 Dec 12;15(23): 2412-2423.
38. Shi Y, Liu J, Zhang R, et al. Targeting Endothelial ENO1 (Alpha-Enolase) -PI3K-Akt-mTOR Axis Alleviates Hypoxic Pulmonary Hypertension. Hypertension, 2023, 80(5): 1035-1047.
39. Sugarman J, Weatherald J. Management of Pulmonary Hypertension Due to Chronic Lung Disease. Methodist Debakey Cardiovasc J. 2021 Jul 1;17(2): 124-133.
40. Chaouat A, Naeije R, Weitzenblum E. Pulmonary hypertension in COPD. Eur Respir J. 2008 Nov;32(5): 1371-85.
41. Vitulo P, Stanziola A, Confalonieri M, et al. Sildenafil in severe pulmonary hypertension associated with chronic obstructive pulmonary disease: A randomized controlled multicenter clinical trial. J Heart Lung Transplant. 2017 Feb;36(2): 166-174.
42. Raghu G, Behr J, Brown KK, et al. Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomized trial. Ann Intern Med. 2013 May 7;158(9): 641-9.
43. Nathan SD, Behr J, Collard HR, et al. Riociguat for idiopathic interstitial pneumonia-associated pulmonary hypertension (RISE-IIP): a randomised, placebo-controlled phase 2b study. Lancet Respir Med. 2019 Sep;7(9): 780-790.
44. Barnes H, Brown Z, Burns A, et al. Phosphodiesterase 5 inhibitors for pulmonary hypertension. Cochrane Database Syst Rev. 2019 Jan 31;1(1): CD012621.
45. Waxman A, Restrepo-Jaramillo R, Thenappan T, et al. Inhaled Treprostinil in Pulmonary Hypertension Due to Interstitial Lung Disease. N Engl J Med. 2021 Jan 28;384(4): 325-334.
46. Hsieh WC, Jansa P, Huang WC, et al. Residual pulmonary hypertension after pulmonary endarterectomy: A meta-analysis. J Thorac Cardiovasc Surg. 2018 Sep;156(3): 1275-1287.
47. Humbert M, Simonneau G, Pittrow D, et al. Oral anticoagulants (NOAC and VKA) in chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant. 2022 Jun;41(6): 716-721.
48. Simonneau G, D'Armini AM, Ghofrani HA, et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension: a long-term extension study (CHEST-2). Eur Respir J. 2015 May;45(5): 1293-302.
49. Sadushi-Kolici R, Jansa P, Kopec G, et al. Subcutaneous treprostinil for the treatment of severe non-operable chronic thromboembolic pulmonary hypertension (CTREPH): a double-blind, phase 3, randomised controlled trial. Lancet Respir Med. 2019 Mar;7(3): 239-248.
50. Ghofrani HA, Simonneau G, D'Armini AM, et al. Macitentan for the treatment of inoperable chronic thromboembolic pulmonary hypertension (MERIT-1): results from the multicentre, phase 2, randomised, double-blind, placebo-controlled study. Lancet Respir Med. 2017 Oct;5(10): 785-794.

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肺動脈高壓的治療進展

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