There are several classifications of Pulmonary Hypertension, one of them being Pulmonary Arterial Hypertension (PAH). In cases in which the cause of PAH is unknown, the disease is referred to as Idiopathic Pulmonary Arterial Hypertension (IPAH). IPAH is an extremely rare disease, making it difficult to treat. However, over the past few decades’ treatments have expanded and improved. Treatments commonly used in clinical practice include one or more of the following, calcium channel blockers, prostacyclin derivatives, endothelin receptor antagonists, phosphodiesterase 5 inhibitors, guanylate cyclase stimulators, and in extreme cases lung transplants (single or double) or heart and lung transplants. The aim of this paper is to review the treatments and therapies available for IPAH because new information has been added after FDA approval of new medications. This article also raises ideas of possible future clinical trials to look at how these medications would work in a combination therapy.
1. Baliga RS, MacAllister RJ, Hobbs AJ. New perspectives for the treatment of pulmonary hypertension. British journal of pharmacology. 2011;163(1):125-40. https://doi.org/10.1111/j.1476-5381.2010.01164.x
2.Holland K. What is Idiopathic Pulmonary Arterial Hypertension. 2014.
3. Waxman AB, Zamanian RT. Pulmonary arterial hypertension: new insights into the optimal role of current and emerging prostacyclin therapies. The American journal of cardiology. 2013;111(5 Suppl):1A-16A; quiz 7A-9A. https://doi.org/10.1016/j.amjcard.2012.12.003
4. Lai YC, Potoka KC, Champion HC, Mora AL, Gladwin MT. Pulmonary arterial hypertension: the clinical syndrome. Circulation research. 2014;115(1):115-30.
5. Bazan IS, Fares WH. Pulmonary hypertension: diagnostic and therapeutic challenges. Therapeutics and clinical risk management. 2015;11:1221-33. https://doi.org/10.2147/tcrm.s74881
6. Idiopathic Pulmonary Arterial Hypertension. Drugscom. 2016.
7. Raed A. Dweik GAH, Omar A. Minai, Adriano R. Tonelli. Pulmonary Hypertension 2011. Available from: http://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/pulmonary/pulmonary-hypertension/.
8. Todd Hirschtritt MKS, Nicholas S Hill Pulmonary Hypertension2008.
9. Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. The New England journal of medicine. 2004;351(14):1425-36.
10. Zhang Y, He LS, Zhang G, Xu Y, Lee OO, Matsumura K, et al. The regulatory role of the NO/cGMP signal transduction cascade during larval attachment and metamorphosis of the barnacle Balanus (=Amphibalanus) amphitrite. The Journal of experimental biology. 2012;215(Pt 21):3813-22. https://doi.org/10.1242/jeb.070235
11. Burger CD, D'Albini L, Raspa S, Pruett JA. The evolution of prostacyclins in pulmonary arterial hypertension: from classical treatment to modern management. The American journal of managed care. 2016;22(1 Suppl):S3-15.
12. Ventetuolo CE, Klinger JR. WHO Group 1 pulmonary arterial hypertension: current and investigative therapies. Progress in cardiovascular diseases. 2012;55(2):89-103. https://doi.org/10.1016/j.pcad.2012.07.002
13. Mitchell JA, Ahmetaj-Shala B, Kirkby NS, Wright WR, Mackenzie LS, Reed DM, et al. Role of prostacyclin in pulmonary hypertension. Global cardiology science & practice. 2014;2014(4):382-93. https://doi.org/10.5339/gcsp.2014.53
14. Medicine SSo. FDA-approved Treatments for Pulmonary Hypertension. Stanford Medicine. 2016.
15. Walsh S. FDA approves new orphan drug to treat pulmonary arterial hypertension. FDA. 2015.
16. Kowalczyk A, Kleniewska P, Kolodziejczyk M, Skibska B, Goraca A. The role of endothelin-1 and endothelin receptor antagonists in inflammatory response and sepsis. Archivum immunologiae et therapiae experimentalis. 2015;63(1):41-52. https://doi.org/10.1007/s00005-014-0310-1
17. Kuntz M, Leiva-Juarez MM, Luthra S. Systematic Review of Randomized Controlled Trials of Endothelin ReceptorAntagonists for Pulmonary Arterial Hypertension. Lung. 2016;194(5):723-32. https://doi.org/10.1007/s00408-016-9928-6
18. Dhillon S. Macitentan: a review of its use in patients with pulmonary arterial hypertension. Drugs. 2014;74(13):1495-507. https://doi.org/10.1007/s40265-014-0266-9
19. Matsubara H, Ogawa A. Treatment of idiopathic/hereditary pulmonary arterial hypertension. Journal of cardiology. 2014;64(4):243-9. https://doi.org/10.1016/j.jjcc.2014.06.009
20. Galie N, Brundage BH, Ghofrani HA, Oudiz RJ, Simonneau G, Safdar Z, et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation. 2009;119(22):2894-903. https://doi.org/10.1161/circulationaha.108.839274
21. FDA approves Adempas to treat pulmonary hypertension. FDA. 2013.
22. Ghofrani HA, Galie N, Grimminger F, Grunig E, Humbert M, Jing ZC, et al. Riociguat for the treatment of pulmonary arterial hypertension. The New England journal of medicine. 2013;369(4):330-40.
23. George Ruiz M, MBA; Gary M. Besinque, PharmD; Cassandra A. Lickert, MD; Susan Raspa, RN, BSN Combination Therapy in Pulmonary Arterial Hypertension: Is This the New Standard of Care? . 2015.
24. Schaffer JM, Singh SK, Joyce DL, Reitz BA, Robbins RC, Zamanian RT, et al. Transplantation for idiopathic pulmonary arterial hypertension: improvement in the lung allocation score era. Circulation. 2013;127(25):2503-13. https://doi.org/10.1161/circulationaha.112.001080
25. Simonneau G, Rubin LJ, Galie N, Barst RJ, Fleming TR, Frost AE, 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(8):521-30. https://doi.org/10.7326/0003-4819-149-8-200810210-00004
26. Galie N, Barbera JA, Frost AE, Ghofrani HA, Hoeper MM, McLaughlin VV, et al. Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension. The New England journal of medicine. 2015;373(9):834-44.
Available at: http://works.bepress.com/fuad-zeid/1/