Pulmonary arterial hypertension: A current review of pharmacological management

Advances in Respiratory Medicine

Volumn 85, Issue 1, 2016, Pages 47-61

  Sahni, Sonu ^a   Ojrzanowski, Marcin ^b   Majewski, Sebastian ^c   Talwar, Arunabh ^a  

^a Northwell Health   (United States)

^b Medical University of Lodz Bieganski Hospital   (Poland)

^c MEDICAL UNIVERSITY OF LODZ   (Poland)

Author keywords

Endothelin receptor antagonist; Phosphodiesterase type 5 inhibitors; Prostacyclin analogues; Pulmonary arterial hypertension; Right heart catheterization

Indexed keywords

ANTIHYPERTENSIVE AGENT; ENDOTHELIN RECEPTOR ANTAGONIST; PHOSPHODIESTERASE V INHIBITOR; PROSTACYCLIN; VASODILATOR AGENT;

COMBINATION DRUG THERAPY; DISEASE MANAGEMENT; ECHOCARDIOGRAPHY; EXERCISE TOLERANCE; HEART CATHETERIZATION; HUMAN; HYPERTENSION, PULMONARY; QUALITY OF LIFE;

ANTIHYPERTENSIVE AGENTS; CARDIAC CATHETERIZATION; DISEASE MANAGEMENT; DRUG THERAPY, COMBINATION; ECHOCARDIOGRAPHY; ENDOTHELIN RECEPTOR ANTAGONISTS; EPOPROSTENOL; EXERCISE TOLERANCE; HUMANS; HYPERTENSION, PULMONARY; PHOSPHODIESTERASE 5 INHIBITORS; QUALITY OF LIFE; VASODILATOR AGENTS;

EID: 84955614692     PISSN: 24514934     EISSN: 25436031     Source Type: Journal    
DOI: 10.5603/PiAP.a2015.0084     Document Type: Review

References (68)

1. Farber HW, Loscalzo J. Pulmonary arterial hypertension. N Engl J Med 2004; 351: 1655-1665
2. Simonneau G, Gatzoulis MA, Adatia I et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2013; 62: D34-41. doi: 10.1016/j.jacc.2013.10.029
3. Galie 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 Respir J, 2015. doi: 10.1183/13993003.01032-2015
4. McLaughlin VV, Gaine SP, Howard LS et al. Treatment goals of pulmonary hypertension. J Am Coll Cardiol 2013; 62: D73-81. doi: 10.1016/j.jacc.2013.10.034
5. Sandoval J, Aguirre JS, Pulido T et al. Nocturnal oxygen therapy in patients with the Eisenmenger syndrome. Am J Respir Crit Care Med 2001; 164: 1682-1687
6. Cohn JN. Optimal diuretic therapy for heart failure. Am J Med 2001; 111: 577
7. Hoeper, MM, Sosada M, Fabel H. Plasma coagulation profiles in patients with severe primary pulmonary hypertension. Eur Respir J 1998; 12: 1446-1449
8. 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; 129: 57-65. doi: 10.1161/CIRCULATIONAHA.113.004526
9. Rhodes CJ, Howard LS, Busbridge M et al. Iron deficiency and raised hepcidin in idiopathic pulmonary arterial hypertension: clinical prevalence, outcomes, and mechanistic insights. J Am Coll Cardiol 2011; 58: 300-309. doi: 10.1016/j.jacc.2011.02.057
10. Sitbon O, Humbert M, Jaïs X et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005; 111: 3105-3111
11. Barst RJ, McGoon M, Torbicki A et al. Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol 2004; 43: 40S-47S.
12. Malhotra R, Hess D, Lewis GD, Bloch KD, Waxman AB, Semigran MJ. Vasoreactivity to inhaled nitric oxide with oxygen predicts long-term survival in pulmonary arterial hypertension. Pulm Circ 2011; 1: 250-258
13. McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation 2002; 106: 1477-1482
14. Badesch DB, Tapson VF, McGoon MD et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med 2000; 132: 425-434
15. Barst RJ, Rubin LJ, Long WA et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med 1996; 334: 296-301
16. Fuentes A, Coralic A, Dawson KL A new epoprostenol formulation for the treatment of pulmonary arterial hypertension. Am J Health Syst Pharm 2012; 69: 1389-1393. doi: 10.2146/ajhp110687
17. 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; 165: 800-804
18. 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; 55: 1915-1922. doi: 10.1016/j.jacc.2010.01.027
19. Hiremath J, Thanikachalam S, Parikh K et al. Exercise improvement and plasma biomarker changes with intravenous treprostinil therapy for pulmonary arterial hypertension: a placebo-controlled trial. J Heart Lung Transplant 2010; 29: 137-149. doi: 10.1016/j.healun.2009.09.005
20. Jing ZC, Parikh K, Pulido T et al. Efficacy and safety of oral treprostinil monotherapy for the treatment of pulmonary arterial hypertension: a randomized, controlled trial. Circulation 2013; 127: 624-633. doi: 10.1161/CIRCULATIONAHA.112.124388
21. Olschewski H, Simonneau G, Galiè N et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 2002; 347: 322-329
22. Sitbon O, Badesch DB, Channick RN et al. Effects of the dual endothelin receptor antagonist bosentan in patients with pulmonary arterial hypertension: a 1-year follow-up study. Chest 2003; 124: 247-254
23. Galie N, Rubin LJ, Hoeper M et al. Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial. Lancet 2008; 371: 2093-2100. doi: 10.1016/S0140-6736(08)60919-8
24. Channick RN, Simonneau G, Sitbon O et al. Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study. Lancet 2001; 358: 1119-1123
25. Oudiz RJ, Galiè N, Olschewski H et al. Long-term ambrisentan therapy for the treatment of pulmonary arterial hypertension. J Am Coll Cardiol 2009; 54: 1971-1981. doi: 10.1016/j.jacc.2009.07.033
26. Galie N, Badesch D, Oudiz R et al. Ambrisentan therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2005; 46: 529-535
27. Galie 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: 3010-3019. doi: 10.1161/CIRCULATIONAHA.107.742510
28. Montani D, Günther S, Dorfmüller P et al. Pulmonary arterial hypertension. Orphanet J Rare Dis 2013; 8: 97. doi: 10.1186/1750-1172-8-97
29. Iglarz M, Binkert C, Morrison K et al. Pharmacology of macitentan, an orally active tissue-targeting dual endothelin receptor antagonist. J Pharmacol Exp Ther 2008; 327: 736-745. doi: 10.1124/jpet.108.142976
30. Pulido T, Adzerikho I, Channick RN et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013; 369: 809-818. doi: 10.1056/NEJMoa1213917
31. Kass DA, Takimoto E, Nagayama T, Champion HC. Phosphodiesterase regulation of nitric oxide signaling. Cardiovasc Res 2007; 75: 303-314
32. Sastry BK, Narasimhan C, Reddy NK, Raju BS. Clinical efficacy of sildenafil in primary pulmonary hypertension: a randomized, placebo-controlled, double-blind, crossover study. J Am Coll Cardiol 2004; 43: 1149-1153
33. Galie N, Ghofrani HA, Torbicki A et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005; 353: 2148-2157
34. Galie N, Brundage BH, Ghofrani HA et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation 2009; 119: 2894-2903. doi: 10.1161/CIRCULATIONAHA.108.839274
35. Evgenov OV, Pacher P, Schmidt PM, Haskó G, Schmidt HH, Stasch JP. NO-independent stimulators and activators of soluble guanylate cyclase: discovery and therapeutic potential. Nat Rev Drug Discov 2006; 5: 755-768
36. Stasch JP, Hobbs AJ. NO-independent, haem-dependent soluble guanylate cyclase stimulators. Handb Exp Pharmacol 2009; 277-308. doi: 10.1007/978-3-540-68964-5_13
37. Grimminger F, Weimann G, Frey R et al. First acute haemodynamic study of soluble guanylate cyclase stimulator riociguat in pulmonary hypertension. Eur Respir J 2009. 33: 785-792. doi: 10.1183/09031936.00039808
38. Ghofrani HA, Galiè N, Grimminger F et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med 2013; 369: 330-340. doi: 10.1056/NEJMoa1209655
39. Khaybullina D, Patel A, Zerilli T. Riociguat (adempas): a novel agent for the treatment of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. P T 2014; 39: 749-758
40. Ghofrani HA, D’Armini AM, Grimminger F et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med 2013; 369: 319-329. doi: 10.1056/NEJMoa1209657
41. McGoon MD, Barst RJ, Doyle RL et al. Reveal registry: Treatment history and treatment at baseline. Chest 2007; 132: 631-631
42. Humbert M, Barst RJ, Robbins IM et al. Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2. Eur Respir J 2004; 24: 353-359
43. Galie N, Barberà JA, Frost AE et al. Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension. N Engl J Med 2015; 373: 834-44. doi: 10.1056/NEJMoa1413687
44. Fox BD, Shimony A, Langleben D. Meta-analysis of monotherapy versus combination therapy for pulmonary arterial hypertension. Am J Cardiol 2011; 108: 1177-1182. doi: 10.1016/j.amjcard.2011.06.021
45. 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; 174: 1257-1263
46. McLaughlin VV, Channick R, Ghofrani HA et al. Effect of bosentan and sildenafil combination therapy on morbidity and mortality in pulmonary arterial hypertension (pah): Results from the compass-2 study. Chest 2014; 146: 860A-860A.
47. Paul GA, Gibbs JS, Boobis AR, Abbas A, Wilkins MR. Bosentan decreases the plasma concentration of sildenafil when coprescribed in pulmonary hypertension. Br J Clin Pharmacol 2005; 60: 107-112
48. Galie N, Müller K, Scalise AV, Grünig E. PATENT PLUS: a blinded, randomised and extension study of riociguat plus sildenafil in pulmonary arterial hypertension. Eur Respir J 2015; 45: 1314-1322. doi: 10.1183/09031936.00105914
49. Simonneau G, Torbicki A, Hoeper MM. Selexipag: an oral, selective prostacyclin receptor agonist for the treatment of pulmonary arterial hypertension. Eur Respir J 2012; 40: 874-880
50. McLaughlin VV, Channick R, Chin K et al. Effect of selexipag on morbidity/mortality in pulmonary arterial hypertension: results of the griphon study. J Am Coll Cardiol 2015; 65 (10_S).
51. Jing ZC, Yu ZX, Shen JY et al. Vardenafil in pulmonary arterial hypertension: a randomized, double-blind, placebo-controlle study. Am J Respir Crit Care Med 2011; 183: 1723-1729. doi: 10.1164/rccm.201101-0093OC.
52. Chin MP, Reisman SA, Bakris GL et al. Mechanisms contributing to adverse cardiovascular events in patients with type 2 diabetes mellitus and stage 4 chronic kidney disease treated with bardoxolone methyl. Am J Nephrol 2014; 39: 499-508. doi: 10.1159/000362906
53. Oudiz R, Meyer C, Chin M et al. Initial data report from “LARIAT”: A phase 2 study of bardoxolone methyl in pah patients on stable background therapy. Chest 2015; 148: 639A-639A.
54. Sahni S, Capozzi B, Iftikhar A, Sgouras V, Ojrzanowski M, Talwar A. Pulmonary rehabilitation and exercise in pulmonary arterial hypertension: An underutilized intervention. J Exerc Rehabil 2015; 11: 74-79. doi: 10.12965/jer.150190
55. Grunig E, Ehlken N, Ghofrani A et al. Effect of exercise and respiratory training on clinical progression and survival in patients with severe chronic pulmonary hypertension. Respiration 2011; 81: 394-401. doi: 10.1159/000322475
56. Grunig E, Lichtblau M, Ehlken N et al. Safety and efficacy of exercise training in various forms of pulmonary hypertension. Eur Respir J 2012; 40: 84-92. doi: 10.1183/09031936.00123711
57. Talwar A, Sahni S, John S, Verma S, Cárdenas-Garcia J, Kohn N. Effects of pulmonary rehablitation on Fatigue Severity Scale in patients with lung disease. Pneumonol Alergol Pol 2014; 82: 534-540. doi: 10.5603/PiAP.2014.0070
58. Keogh AM, Mayer E, Benza RL et al. Interventional and surgical modalities of treatment in pulmonary hypertension. J Am Coll Cardiol 2009; 54: S67-77. doi: 10.1016/j.jacc.2009.04.016
59. Szturmowicz M, Kacprzak A, Błasińska-Przerwa K, Kuś J. Pulmonary hypertension in the course of diffuse parenchymal lung diseases — state of art and future considerations. Pneumonol Alergol Pol. 2015; 83: 312-323. doi: 10.5603/PiAP.2015.0051
60. Higenbottam T, Butt AY, McMahon A, Westerbeck R, Sharples L. Long-term intravenous prostaglandin (epoprostenol or iloprost) for treatment of severe pulmonary hypertension. Heart 1998; 80: 151-155
61. Galie N, Humbert M, Vachiéry JL et al. Effects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: a randomized, double-blind, placebo- controlled trial. J Am Coll Cardiol 2002; 39: 1496-1502
62. Sitbon O, Jaïs X, Savale L et al. Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study. Eur Respir J 2014; 43: 1691-1697. doi: 10.1183/09031936.00116313
63. Kemp K, Savale L, O’Callaghan DS et al. Usefulness of first -line combination therapy with epoprostenol and bosentan in pulmonary arterial hypertension: an observational study. J Heart Lung Transplant 2012; 31: 150-158. doi: 10.1016/j.healun.2011.11.002
64. Simonneau G, Rubin LJ, Galiè N et al. Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial. Ann Intern Med 2008; 149: 521-530
65. Badesch DB, Feldman J, Keogh A et al. ARIES-3: ambrisentan therapy in a diverse population of patients with pulmonary hypertension. Cardiovasc Ther 2012; 30: 93-99. doi: 10.1111/j.1755-5922.2011.00279.x.
66. Provencher S, Sitbon O, Humbert M, Cabrol S, Jaïs X, Simonneau G. Long-term outcome with first-line bosentan therapy in idiopathic pulmonary arterial hypertension. Eur Heart J 2006; 27: 589-595
67. McLaughlin VV, Channick RN, Ghofrani HA et al. Bosentan added to sildenafil therapy in patients with pulmonary arterial hypertension. Eur Respir J 2015; 46: 405-413. doi: 10.1183/13993003.02044-2014
68. Dardi F, Manes A, Palazzini M et al. Combining bosentan and sildenafil in pulmonary arterial hypertension patients failing monotherapy: real-world insights. Eur Respir J 2015; 46: 414-421. doi: 10.1183/09031936.00209914.