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2024, Number 4

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Neumol Cir Torax 2024; 83 (4)

Mexican Clinical Practice Guideline for Pulmonary Hypertension

Zayas-Hernández NG, Espitia-Hernández G, Sandoval-Gutiérrez JL, Hernández-Oropeza JL, Cueto-Robledo G, Moreno-Hoyos JF, López-Estupiñán S, García-Aguilar H, Marín FJ, Palomar-Lever A, Salas-Domínguez J, Kimura E, Figueroa-Morales MA, Mayorga-Butrón JL, Rodríguez-Vega M, Sauza-Sosa JC, Portales-Castanedo AG, Pulido T, Gómez-González A

Language: Spanish
References: 100
Page: 256-301
PDF size: 860.78 Kb.


ABSTRACT

Introduction: pulmonary hypertension (PH) is a diagnostic challenge because of its nonspecific symptoms and diverse etiologies. Diagnosis should follow an order, using right heart catheterization. Treatment is determined according to the cause and severity of the disease. Objective: to update the guidelines for the classification, diagnosis, treatment, and referral to specialized centers of patients with PH, focusing on the Mexican scenario. Material and methods: the existing clinical practice guidelines on PH, evaluated by AGREE II, were updated and adapted. The development group comprised a core group and an extended group. The core group developed a scope document, and conducted PI(E)CO questions, leading to systematic literature searches using PubMed and EMBASE databases. The core group evaluated the literature with the best quality of evidence and prepared a document with clinical questions and proposed recommendations. Using a modified Delphi panel consensus methodology, the extended group approved the recommendations in terms of content and wording, and then, the level of scientific evidence and grade of recommendation was added, according to the SIGN methodology. Results: 45 recommendations were made for classification, diagnosis, treatments [non-pharmacological, pharmacological (non-specific and specific), interventional, and surgical], end-of-life care, and referral criteria to the specialist. Conclusions: we provide an updated document, useful for physicians caring for patients with PH.


REFERENCES

  1. Jerjes-Sánchez C, Ramírez-Rivera A, Hernandez NZ, Cueto-Robledo G, García-Aguilar H, Gutiérrez-Fajardo P, et al. Demographic, hemodynamic characteristics, and therapeutic trends of pulmonary hypertension patients: The Pulmonary Hypertension Mexican registry (REMEHIP). Pulm Circ. 2024;14(2):e12395. Available in: https://doi.org/10.1002/pul2.12395

  2. CENETEC. Diagnóstico y tratamiento de la hipertensión arterial pulmonar primario en el adulto. Catálogo Maestro Guías Práctica Clínica. 2010; México.

  3. Benza RL, Lohmueller LC, Kraisangka J, Kanwar M. Risk assessment in pulmonary arterial hypertension patients: the long and short of it. Adv Pulm Hypertens. 2018;16(3):125-135. Available in: https://doi.org/10.21693/1933-088x-16.3.125

  4. Pulido T, Sandoval J, Jerjes-Sanchez C, Ramirez A, Beltrán M, Seoane M, et al. The phenotype of the adult mexican patient with pulmonary arterial hypertension: Baseline characteristics of the REMEHIP Registry. The REMEHIP Investigators. Eur Respir J. 2018;52(Suppl 62):PA3086. doi: 10.1183/13993003.congress-2018.PA3086.

  5. George MG, Schieb LJ, Ayala C, Talwalkar A, Levant S. Pulmonary hypertension surveillance: United States, 2001 to 2010. Chest. 2014;146(2):476-495. Available in: https://doi.org/10.1378/chest.14-1440.

  6. Morales-Blanhir JE, Baloira-Villar A. Trabajando juntos en la hipertensión pulmonar: España y Latinoamérica. Neumol Cir Torax. 2012;71(2):139-140.

  7. Gob.mx. [citado el 21 de febrero de 2024]. Disponible en: https://infosen.senado.gob.mx/sgsp/gaceta/65/1/2021-09-13-1/assets/documentos/Dict_Salud_Himpertencion_Arterial_Pulmonar.pdf

  8. Malenfant S, Neyron AS, Paulin R, Potus F, Meloche J, Provencher S, et al. Signal transduction in the development of pulmonary arterial hypertension. Pulm Circ. 2013;3(2):278-293. Available in: https://doi.org/10.4103/2045-8932.114752

  9. Brouwers MC, Kho ME, Browman GP, Burgers JS, Cluzeau F, Feder G, et al. AGREE II: advancing guideline development, reporting and evaluation in health care. CMAJ.2010;182(18):E839-42. Available in: https://doi.org/10.1503/cmaj.090449

  10. Murphy MK, Black NA, Lamping DL, McKee CM, Sanderson CF, Askham J, et al. Consensus development methods, and their use in clinical guideline development. Health Technol Assess. 1998;2(3):i-iv, 1-88. Available in: http://www.ncbi.nlm.nih.gov/pubmed/9561895

  11. (SIGN), SIGN 50: A guideline developer's handbook. Edinburgh: SIGN. SIGN Publ. No.50. 2019. [Internet]. 2019. Available in: http://www.sign.ac.uk

  12. Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RMF, Brida M, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;43(38):3618-3731. doi: 10.1093/eurheartj/ehac237. Erratum in: Eur Heart J. 2023;44(15):1312.

  13. Kovacs G, Berghold A, Scheidl S, Olschewski H. Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review. Eur Respir J. 2009;34(4):888-894. Available in: https://doi.org/10.1183/09031936.00145608

  14. Simonneau G, Montani D, Celermajer DS, Denton CP, Gatzoulis MA, Krowka M, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53(1):1801913. Available in: https://doi.org/10.1183/13993003.01913-2018

  15. Kovacs G, Dumitrescu D, Barner A, Greiner S, Grünig E, Hager A, et al. Definition, clinical classification and initial diagnosis of pulmonary hypertension: Updated recommendations from the Cologne Consensus Conference 2018. Int J Cardiol. 2018;272:11-19. Available in: https://doi.org/10.1016/j.ijcard.2018.08.083

  16. Galiè N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, 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 Respir J. 2015;46(4):903-975. doi: 10.1183/13993003.51032-2015.

  17. Taichman DB, McGoon MD, Harhay MO, Archer-Chicko C, Sager JS, Murugappan M, et al. Wide variation in clinicians' assessment of New York Heart Association/World Health Organization functional class in patients with pulmonary arterial hypertension. Mayo Clin Proc. 2009;84(7):586-592. Available in: https://doi.org/10.1016/s0025-6196(11)60747-7

  18. Barst RJ, Chung L, Zamanian RT, Turner M, McGoon MD. Functional class improvement and 3-year survival outcomes in patients with pulmonary arterial hypertension in the REVEAL Registry. Chest. 2013;144(1):160-168. Available in: https://doi.org/10.1378/chest.12-2417

  19. Galiè N, Channick RN, Frantz RP, Grünig E, Jing ZC, Moiseeva O, et al. Risk stratification and medical therapy of pulmonary arterial hypertension. Eur Respir J. 2019;53(1):1801889. Available in: https://doi.org/10.1183/13993003.01889-2018

  20. Scott JV, Garnett CE, Kanwar MK, Stockbridge NL, Benza RL. Enrichment benefits of risk algorithms for pulmonary arterial hypertension clinical trials. Am J Respir Crit Care Med. 2021;203(6):726-736. Available in: https://doi.org/10.1164/rccm.202002-0357OC

  21. Humbert M, Sitbon O, Yaici A, Montani D, O'Callaghan DS, Jais X, et al. Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension. Eur Respir J. 2010;36(3):549-555. Available in: https://doi.org/10.1183/09031936.00057010

  22. Benza RL, Gomberg-Maitland M, Miller DP, Frost A, Frantz RP, Foreman AJ, et al. The REVEAL Registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension. Chest. 2012;141(2):354-362. Available in: https://doi.org/10.1378/chest.11-0676

  23. Mathai SC, Hassoun PM. Pulmonary arterial hypertension in connective tissue diseases. Heart Fail Clin. 2012;8(3):413-425. Available in: https://doi.org/10.1016/j.hfc.2012.04.001

  24. Evans JDW, Girerd B, Montani D, Wang X-J, Galiè N, Austin ED, et al. BMPR2 mutations and survival in pulmonary arterial hypertension: an individual participant data meta-analysis. Lancet Respir Med. 2016;4(2):129-137. Available in: https://doi.org/10.1016/S2213-2600(15)00544-5

  25. Gabler NB, French B, Strom BL, Palevsky HI, Taichman DB, Kawut SM, et al. Validation of 6-minute walk distance as a surrogate end point in pulmonary arterial hypertension trials. Circulation. 2012;126(3):349-356. Available in: https://doi.org/10.1161/CIRCULATIONAHA.112.105890

  26. Frantz RP, Farber HW, Badesch DB, Elliott CG, Frost AE, McGoon MD, et al. Baseline and serial brain natriuretic peptide level predicts 5-year overall survival in patients with pulmonary arterial hypertension: data from the REVEAL Registry. Chest. 2018;154(1):126-135. Available in: https://doi.org/10.1016/j.chest.2018.01.009

  27. Burger CD, Long PK, Shah MR, McGoon MD, Miller DP, Romero AJ, et al. Characterization of first-time hospitalizations in patients with newly diagnosed pulmonary arterial hypertension in the REVEAL registry. Chest. 2014;146(5):1263-1273. Available in: https://doi.org/10.1378/chest.14-0193

  28. D'Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med. 1991;115(5):343-349. Available in: https://doi.org/10.7326/0003-4819-115-5-343

  29. Humbert M, Sitbon O, Chaouat A, Bertocchi M, Habib G, Gressin V, et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation. 2010;122(2):156-163. Available in: https://doi.org/10.1161/CIRCULATIONAHA.109.911818

  30. Thenappan T, Glassner C, Gomberg-Maitland M. Validation of the pulmonary hypertension connection equation for survival prediction in pulmonary arterial hypertension. Chest. 2012;141(3):642-650. Available in: https://doi.org/10.1378/chest.11-0969

  31. Lee WT, Ling Y, Sheares KK, Pepke-Zaba J, Peacock AJ, Johnson MK. Predicting survival in pulmonary arterial hypertension in the UK. Eur Respir J. 2012;40(3):604-611. Available in: https://doi.org/10.1183/09031936.00196611

  32. Benza RL, Miller DP, Foreman AJ, Frost AE, Badesch DB, Benton WW, et al. Prognostic implications of serial risk score assessments in patients with pulmonary arterial hypertension: a Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) analysis. J Heart Lung Transplant. 2015;34(3):356-361. Available in: https://doi.org/10.1016/j.healun.2014.09.016

  33. Olsson KM, Delcroix M, Ghofrani HA, Tiede H, Huscher D, Speich R, 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;129(1):57-65. Available in: https://doi.org/10.1161/CIRCULATIONAHA.113.004526

  34. Benza RL, Kanwar MK, Raina A, Scott JV, Zhao CL, Selej M, et al. Development and validation of an abridged version of the REVEAL 2.0 risk score calculator, REVEAL Lite 2, for use in patients with pulmonary arterial hypertension. chest. 2021;159(1):337-346. Available in: https://doi.org/10.1016/j.chest.2020.08.2069

  35. Boucly A, Weatherald J, Savale L, Jais X, Cottin V, Prevot G, et al. Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension. Eur Respir J. 2017;50(2):1700889. Available in: https://doi.org/10.1183/13993003.00889-2017

  36. Braganza M, Shaw J, Solverson K, Vis D, Janovcik J, Varughese RA, et al. A prospective evaluation of the diagnostic accuracy of the physical examination for pulmonary hypertension. Chest. 2019;155(5):982-990. Available in: https://doi.org/10.1016/j.chest.2019.01.035

  37. Brown LM, Chen H, Halpern S, Taichman D, McGoon MD, Farber HW, et al. Delay in recognition of pulmonary arterial hypertension: factors identified from the REVEAL Registry. Chest. 2011;140(1):19-26. Available in: https://doi.org/10.1378/chest.10-1166

  38. Frost A, Badesch D, Gibbs JSR, Gopalan D, Khanna D, Manes A, et al. Diagnosis of pulmonary hypertension. Eur Respir J. 2019;53(1):1801904. Available in: https://doi.org/10.1183/13993003.01904-2018

  39. Flores-Franco RA, Frías-Fierro DA. La clínica en la hipertensión pulmonar del adulto. Neumol Cir Torax. 2019;78(3):324-333. Available in: https://dx.doi.org/10.35366/NT193J

  40. Rubin L. Clinical features and diagnosis of pulmonary hypertension of unclear etiology in adults. In: UpToDate, Post TW (Ed), UpToDate, Waltham, MA. 2021.

  41. Kovacs G, Avian A, Foris V, Tscherner M, Kqiku X, Douschan P, et al. Use of ECG and other simple non-invasive tools to assess pulmonary hypertension. PLoS One. 2016;11(12):e0168706. Available in: https://doi.org/10.1371/journal.pone.0168706

  42. Rajaram S, Swift AJ, Telfer A, Hurdman J, Marshall H, Lorenz E, et al. 3D contrast-enhanced lung perfusion MRI is an effective screening tool for chronic thromboembolic pulmonary hypertension: results from the ASPIRE registry. Thorax. 2013;68(7):677-678. Available in: https://doi.org/10.1136/thoraxjnl-2012-203020

  43. Reiter G, Reiter U, Kovacs G, Olschewski H, Fuchsjager M. Blood flow vortices along the main pulmonary artery measured with MR imaging for diagnosis of pulmonary hypertension. Radiology. 2015;275(1):71-79. Available in: https://doi.org/10.1148/radiol.14140849

  44. Jilwan FN, Escourrou P, Garcia G, Jaïs X, Humbert M, Roisman G. High occurrence of hypoxemic sleep respiratory disorders in precapillary pulmonary hypertension and mechanisms. Chest. 2013;143(1):47-55. Available in: https://doi.org/10.1378/chest.11-3124

  45. Opitz CF, Rubin LJ. Acute vasodilator testing in idiopathic pulmonary arterial hypertension: must we take NO for the answer? Eur Respir J. 2009;33(6):1247-1249. Available in: https://doi.org/10.1183/09031936.00033809

  46. Morrell NW, Aldred MA, Chung WK, Elliott CG, Nichols WC, Soubrier F, et al. Genetics and genomics of pulmonary arterial hypertension. Eur Respir J. 2019;53(1):1801899. Available in: https://doi.org/10.1183/13993003.01899-2018

  47. Leuchte HH, Halank M, Held M, Borst M, Ewert R, Klose H, et al. [Differential diagnosis of pulmonary hypertension using the example of collagenosis-associated PAH in the context of chronic lung and left heart disease]. Pneumologie. 2021;75(2):122-137. Available in: https://doi.org/10.1055/a-1204-3248

  48. Nogueira-Ferreira R, Moreira-Goncalves D, Santos M, Trindade F, Ferreira R, Henriques-Coelho T. Mechanisms underlying the impact of exercise training in pulmonary arterial hypertension. Respir Med. 2018;134:70-78. Available in: https://doi.org/10.1016/j.rmed.2017.11.022

  49. Pandey A, Garg S, Khunger M, Garg S, Kumbhani DJ, Chin KM, et al. Efficacy and Safety of Exercise Training in Chronic Pulmonary Hypertension: Systematic Review and Meta-Analysis. Circ Heart Fail. 2015;8(6):1032-1043. Available in: https://doi.org/10.1161/CIRCHEARTFAILURE.115.002130

  50. Buys R, Avila A, Cornelissen VA. Exercise training improves physical fitness in patients with pulmonary arterial hypertension: a systematic review and meta-analysis of controlled trials. BMC Pulm Med. 2015;15:40. Available in: https://doi.org/10.1186/s12890-015-0031-1

  51. Waller L, Krüger K, Conrad K, Weiss A, Alack K. Effects of different types of exercise training on pulmonary arterial hypertension: a systematic review. J Clin Med. 2020;9(6):1689. Available in: https://doi.org/10.3390/jcm9061689

  52. González-Saiz L, Fiuza-Luces C, Sanchis-Gomar F, Santos-Lozano A, Quezada-Loaiza CA, Flox-Camacho A, et al. Benefits of skeletal-muscle exercise training in pulmonary arterial hypertension: The WHOLEi + 12 trial. Int J Cardiol. 2017;231:277-283. Available in: https://dx.doi.org/10.1016/j.ijcard.2016.12.026

  53. Klinger JR, Elliott CG, Levine DJ, Bossone E, Duvall L, Fagan K, et al. Therapy for pulmonary arterial hypertension in adults: update of the CHEST guideline and expert panel report. Chest. 2019;155(3):565-586. Available in: https://doi.org/10.1016/j.chest.2018.11.030

  54. Ezedunukwe I, Enuh H, Nfonoyim J, Enuh CU. Anticoagulation therapy versus placebo for pulmonary hypertension. Cochrane Database Syst Rev. 2014;2014(6):CD010695. Available in: https://doi.org/10.1002/14651858.cd010695.pub2

  55. Caldeira D, Loureiro MJ, Costa J, Pinto FJ, Ferreira JJ. Oral anticoagulation for pulmonary arterial hypertension: Systematic review and meta-analysis. Can J Cardiol. 2014;30(8):879-887. http://dx.doi.org/10.1016/j.cjca.2014.04.016

  56. Preston IR, Roberts KE, Miller DP, Sen GP, Selej M, Benton WW, 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;132(25):2403-2411. Available in: https://doi.org/10.1161/CIRCULATIONAHA.115.018435

  57. Calderone A, Stevens W, Prior D, Nandurkar H, Gabbay E, Proudman SM, et al. Multicentre randomised placebo-controlled trial of oral anticoagulation with apixaban in systemic sclerosis-related pulmonary arterial hypertension: the SPHInX study protocol. BMJ Open. 2016;6(12):e011028. Available in: https://doi.org/10.1136/bmjopen-2016-011028

  58. Ulrich S, Keusch S, Hildenbrand FF, Lo Cascio C, Huber LC, Tanner FC, et al. Effect of nocturnal oxygen and acetazolamide on exercise performance in patients with pre-capillary pulmonary hypertension and sleep-disturbed breathing: randomized, double-blind, cross-over trial. Eur Heart J. 2015;36(10):615-623. Available in: https://doi.org/10.1093/eurheartj/eht540

  59. Weitzenblum E, Sautegeau A, Ehrhart M, Mammosser M, Pelletier A. Long-term oxygen therapy can reverse the progression of pulmonary hypertension in patients with chronic obstructive pulmonary disease. Am Rev Respir Dis. 1985;131(4):493-498. Available in: https://doi.org/10.1164/arrd.1985.131.4.493

  60. Lacasse Y, Bernard S, Sériès F, Nguyen VH, Bourbeau J, Aaron S, et al. Multi-center, randomized, placebo-controlled trial of nocturnal oxygen therapy in chronic obstructive pulmonary disease: a study protocol for the INOX trial. BMC Pulm Med. 2017;17(1):8. Available in: https://doi.org/10.1186/s12890-016-0343-9

  61. Ulrich S, Hasler ED, Saxer S, Furian M, Müller-Mottet S, Keusch S, et al. Effect of breathing oxygen-enriched air on exercise performance in patients with precapillary pulmonary hypertension: randomized, sham-controlled cross-over trial. Eur Heart J. 2017;38(15):1159-1168. Available in: https://doi.org/10.1093/eurheartj/ehx099

  62. Ulrich S, Saxer S, Hasler ED, Schwarz EI, Schneider SR, Furian M, et al. Effect of domiciliary oxygen therapy on exercise capacity and quality of life in patients with pulmonary arterial or chronic thromboembolic pulmonary hypertension: A randomised, placebo-controlled trial. Eur Respir J. 2019;54(2):1900276. Available in: https://doi.org/10.1183/13993003.002762019

  63. Alajaji W, Baydoun A, Al-Kindi SG, Henry L, Hanna MA, Oliveira GH. Digoxin therapy for cor pulmonale: a systematic review. Int J Cardiol. 2016;223:320-324. Available in: https://doi.org/10.1016/j.ijcard.2016.08.018

  64. Correale M, Zicchino S, Monaco I, Di Biase M, Brunetti ND. Angiotensin-converting enzyme inhibitors, angiotensin II receptors antagonists, beta-blockers and ivabradine as supportive therapy in pulmonary hypertension: drug safety and tolerability. Eur J Intern Med. 2017;44:e24-e27. Available in: https://doi.org/10.1016/j.ejim.2017.07.016

  65. Correale M, Monaco I, Ferraretti A, Tricarico L, Padovano G, Formica ES, et al. Hospitalization cost reduction with sacubitril-valsartan implementation in a cohort of patients from the Daunia Heart Failure Registry. Int J Cardiol Heart Vasc. 2019;22:102-104. Available in: https://doi.org/10.1016/j.ijcha.2018.12.009

  66. Correale M, Mallardi A, Mazzeo P, Tricarico L, Diella C, Romano V, et al. Sacubitril/valsartan improves right ventricular function in a real-life population of patients with chronic heart failure: the Daunia Heart Failure Registry. Int J Cardiol Heart Vasc. 2020;27:100486. Available in: https://doi.org/10.1016/j.ijcha.2020.100486

  67. Provencher S, Herve P, Jais X, Lebrec D, Humbert M, Simonneau G, et al. Deleterious effects of beta-blockers on exercise capacity and hemodynamics in patients with portopulmonary hypertension. Gastroenterology. 2006;130(1):120-126. Available in: https://doi.org/10.1053/j.gastro.2005.10.013

  68. Galiè N, Torbicki A, Barst R, Dartevelle P, Haworth S, Higenbottam T, et al. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The task force on diagnosis and treatment of pulmonary arterial hypertension of the European Society of Cardiology. Eur Heart J. 2004;25(24):2243-2278. Available in: https://doi.org/10.1016/j.ehj.2004.09.014

  69. Fan Z, Chen Y, Liu H. Calcium channel blockers for pulmonary arterial hypertension. Cochrane Database Syst Rev. 2015;2015(9):CD010066. Available in: https://doi.org/10.1002/14651858.CD010066.pub2

  70. Horinouchi T, Terada K, Higashi T, Miwa S. Endothelin receptor signaling: new insight into its regulatory mechanisms. J Pharmacol Sci. 2013;123(2):85-101. Available in: https://doi.org/10.1254/jphs.13r02cr

  71. Thenappan T, Ormiston ML, Ryan JJ, Archer SL. Pulmonary arterial hypertension: pathogenesis and clinical management. BMJ. 2018;360:j5492. Available in: https://doi.org/10.1136/bmj.j5492

  72. Liu C, Chen J, Gao Y, Deng B, Liu K. Endothelin receptor antagonists for pulmonary arterial hypertension. Cochrane Database Syst Rev. 2021;3(3):CD004434. Available in: https://doi.org/10.1002/14651858.cd004434.pub6

  73. Ghofrani HA, Simonneau G, D'Armini AM, Fedullo P, Howard LS, Jais X, 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;5(10):785-794. Available in: https://doi.org/0.1016/S2213-2600(17)30305-3

  74. Stasch JP, Pacher P, Evgenov OV. Soluble guanylate cyclase as an emerging therapeutic target in cardiopulmonary disease. Circulation. 2011;123(20):2263-2273. Available in: https://doi.org/10.1161/CIRCULATIONAHA.110.981738

  75. Wardle AJ, Seager MJ, Wardle R, Tulloh RMR, Gibbs JSR. Guanylate cyclase stimulators for pulmonary hypertension. Cochrane Database Syst Rev. 2016;2016(8):CD011205. Available in: https://doi.org/10.1002/14651858.CD011205.pub2

  76. Sim JY. Nitric oxide and pulmonary hypertension. Korean J Anesthesiol. 2010;58(1):4-14. Available in: https://doi.org/10.4097/kjae.2010.58.1.4

  77. Ghofrani HA, Pepke-Zaba J, Barbera JA, Channick R, Keogh AM, Gomez-Sanchez MA, et al. Nitric oxide pathway and phosphodiesterase inhibitors in pulmonary arterial hypertension. J Am Coll Cardiol. 2004;43(12 Suppl S):68S-72S. Available in: https://doi.org/10.1016/j.jacc.2004.02.031

  78. Barnes H, Brown Z, Burns A, Williams T. Phosphodiesterase 5 inhibitors for pulmonary hypertension. Cochrane Database Syst Rev. 2019;1(1):CD012621. Available in: https://doi.org/10.1002/14651858.CD012621.pub2

  79. Vane J, Corin RE. Prostacyclin: a vascular mediator. Eur J Vasc Endovasc Surg. 2003;26(6):571-578. Available in: https://doi.org/10.1016/s1078-5884(03)00385-x

  80. Dorris SL, Peebles RS Jr. PGI2 as a regulator of inflammatory diseases. Mediators Inflamm. 2012;2012:926968. Available in: https://doi.org/10.1155/2012/926968

  81. Doran AK, Ivy DD, Barst RJ, Hill N, Murali S, Benza RL; 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;(160):5-9. Available in: https://doi.org/10.1111/j.1742-1241.2008.01811.x

  82. Saji T, Myoishi M, Sugimura K, Tahara N, Takeda Y, Fukuda K, et al. Efficacy and safety of inhaled iloprost in japanese patients with pulmonary arterial hypertension-insights from the IBUKI and AIR studies. Circ J. 2016;80(4):835-842. Available in: https://doi.org/10.1253/circj.CJ-16-0097

  83. Tapson VF, Jing Z-C, Xu K-F, Pan L, Feldman J, Kiely DG, et al. Oral treprostinil for the treatment of pulmonary arterial hypertension in patients receiving background endothelin receptor antagonist and phosphodiesterase type 5 inhibitor therapy (the FREEDOM-C2 study): a randomized controlled trial. Chest. 2013;144(3):952-958. Available in: https://doi.org/10.1378/chest.12-2875

  84. Olschewski H, Rose F, Schermuly R, Ghofrani HA, Enke B, Olschewski A, et al. Prostacyclin and its analogues in the treatment of pulmonary hypertension. Pharmacol Ther. 2004;102(2):139-153. Available in: https://doi.org/10.1016/j.pharmthera.2004.01.003

  85. Sitbon O, Channick R, Chin KM, Frey A, Gaine S, Galiè N, et al. Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med. 2015;373(26):2522-2533. Available in: https://doi.org/10.1056/NEJMoa1503184

  86. Jain S, Khera R, Girotra S, Badesch D, Wang Z, Murad MH, et al. Comparative effectiveness of pharmacologic interventions for pulmonary arterial hypertension: a systematic review and network meta-analysis. Chest. 2017;151(1):90-105. Available in: https://doi.org/10.1016/j.chest.2016.08.1461

  87. Shivanna B, Gowda S, Welty SE, Barrington KJ, Pammi M. Prostanoids and their analogues for the treatment of pulmonary hypertension in neonates. Cochrane Database Syst Rev. 2019;10(10):CD012963. Available in: https://doi.org/10.1002/14651858.CD012963.pub2

  88. Galiè N, Barberà JA, Frost AE, Ghofrani H-A, Hoeper MM, McLaughlin VV, et al. Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension. N Engl J Med. 2015;373(9):834-844. Available in: https://doi.org/10.1056/NEJMoa1413687

  89. McLaughlin V, Channick RN, Ghofrani H-A, Lemarié J-C, Naeije R, Packer M, et al. Bosentan added to sildenafil therapy in patients with pulmonary arterial hypertension. Eur Respir J. 2015;46(2):405-413. Available in: https://doi.org/10.1183/13993003.02044-2014

  90. Zhuang Y, Jiang B, Gao H, Zhao W. Randomized study of adding tadalafil to existing ambrisentan in pulmonary arterial hypertension. Hypertens Res. 2014;37(6):507-512. Available in: https://doi.org/10.1038/hr.2014.28

  91. Channick RN, Delcroix M, Ghofrani H-A, Hunsche E, Jansa P, Le Brun F-O, et al. Effect of macitentan on hospitalizations: results from the SERAPHIN trial. JACC Heart Fail. 2015;3(1):1-8. Available in: https://doi.org/10.1016/j.jchf.2014.07.013

  92. Critser PJ, Evers PD, McGovern E, Cash M, Hirsch R. Balloon atrial septostomy as initial therapy in pediatric pulmonary hypertension. Pulm Circ. 2020;10(4):2045894020958970. Available in: https://doi.org/10.1177/2045894020958970

  93. Sandoval J, Gaspar J, Peña H, Santos LE, Córdova J, del Valle K, et al. Effect of atrial septostomy on the survival of patients with severe pulmonary arterial hypertension. Eur Respir J. 2011;38(6):1343-1348. Available in: https://doi.org/10.1183/09031936.00072210

  94. Khan MS, Memon MM, Amin E, Yamani N, Khan SU, Figueredo VM, et al. Use of balloon atrial septostomy in patients with advanced pulmonary arterial hypertension: a systematic review and meta-analysis. Chest. 2019;156(1):53-63. Available in: https://doi.org/10.1016/j.chest.2019.03.003

  95. Micheletti A, Hislop AA, Lammers A, Bonhoeffer P, Derrick G, Rees P, et al. Role of atrial septostomy in the treatment of children with pulmonary arterial hypertension. Heart. 2006;92(7):969-972. Available in: https://doi.org/10.1136/hrt.2005.077669

  96. Khan IY, Singer LG, de Perrot M, Granton JT, Keshavjee S, Chau C, et al. Survival after lung transplantation in systemic sclerosis. A systematic review. Respir Med. 2013;107(12):2081-2087. Available in: https://doi.org/10.1016/j.rmed.2013.09.015

  97. Christiansen D, Porter S, Hurlburt L, Weiss A, Granton J, Wentlandt K. Pulmonary arterial hypertension: a palliative medicine review of the disease, its therapies, and drug interactions. J Pain Symptom Manage. 2020;59(4):932-943. Available in: https://doi.org/10.1016/j.jpainsymman.2019.11.023

  98. Anand V, Vallabhajosyula S, Cheungpasitporn W, Frantz RP, Cajigas HR, Strand JJ, et al. Inpatient palliative care use in patients with pulmonary arterial hypertension: temporal trends, predictors, and outcomes. Chest. 2020;158(6):2568-2578. Available in: https://doi.org/10.1016/j.chest.2020.07.079

  99. Kovacs G, Bartolome S, Denton CP, Gatzoulis MA, Gu S, Khanna D, et al. Definition, classification and diagnosis of pulmonary hypertension. Eur Respir J. 2024;64(4):2401324. doi: 10.1183/13993003.01324-2024.

  100. Bhatnagar A, Wiesen J, Dweik R, Chaisson NF. Evaluating suspected pulmonary hypertension: a structured approach. Cleve Clin J Med. 2018;85(6):468-480. doi: 10.3949/ccjm.85a.17065.




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