Macitentan in the Treatment of Pulmonary Arterial Hypertension

References

• Simonneau G , MontaniD, CelermajerDSet al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur. Respir. J., 53, 1801913 (2019).
   PubMed Web of Science ®Google Scholar
• Hoeper M , GibbsS. The changing landscape of pulmonary arterial hypertension and implications for patient care. Eur. Respir. Rev., 23, 450–457 (2014).
   PubMedGoogle Scholar
• Naeije R , ManesA. The right ventricle in pulmonary arterial hypertension. Eur. Respir. Rev., 23, 476–487 (2014).
   PubMedGoogle Scholar
• Christman B , McPhersonC, NewmanJet al. An imbalance between the excretion of thromboxane and prostacyclin metabolites in pulmonary hypertension. N. Engl. J. Med., 327, 70–75 (1992).
   PubMed Web of Science ®Google Scholar
• Giaid A , SalehD. Reduced expression of endothelial nitric oxide synthase in the lungs of patients with pulmonary hypertension. N. Engl. J. Med., 333, 214–221 (1995).
   PubMed Web of Science ®Google Scholar
• Giaid A , YanagisawaM, LanglebenDet al. Expression of endothelin-1 in the lungs of patients with pulmonary hypertension. N. Engl. J. Med., 328, 1732–1739 (1993).
   PubMed Web of Science ®Google Scholar
• Giaid A . Nitric oxide and endothelin-1 in pulmonary hypertension. Chest, 114, S208–S212 (1998).
   PubMed Web of Science ®Google Scholar
• Schiffrin EL , TouyzRM. Vascular biology of endothelin. J. Cardiovasc. Pharmacol., 32, S2–S13 (1998).
   PubMed Web of Science ®Google Scholar
• FD Davenport AP . Secretory pathways in endothelin synthesis. Br. J. Pharmacol., 126, 391–398 (1999).
   PubMed Web of Science ®Google Scholar
• Bacon C , CaryN, DavenportA. Endothelin peptide and receptors in human atherosclerotic coronary artery and aorta. Circ. Res., 79, 794–801 (1996).
   PubMed Web of Science ®Google Scholar
• Kurihara H , YoshizumiM, SugiyamaTet al. The possible role of endothelin-1 in the pathogenesis of coronary vasospasm. J. Cardiovasc. Pharmacol., 13, S132–S137 (1989).
   PubMed Web of Science ®Google Scholar
• Cohn J . Is there a role for endothelin in the natural history of heart failure?Circulation, 94, 604–606 (1996).
   PubMed Web of Science ®Google Scholar
• Russell FD , SkepperJN, DavenportAP. Evidence using immunoelectron microscopy for regulated and constitutive pathways in the transport and release of endothelin. J. Cardiovasc. Pharmacol., 31, 424–430 (1998).
   PubMed Web of Science ®Google Scholar
• Montani D , ChaumaisM, GuignabertCet al. Targeted therapies in pulmonary arterial hypertension. Pharmacol. Ther., 141, 172–191 (2014).
   PubMed Web of Science ®Google Scholar
• Yamashita K , DischerDJ, HuJ, BishopricNH, WebsterKA. Molecular regulation of the endothelin-1 gene by hypoxia. Contributions of hypoxia-inducible factor-1, activator protein-1, GATA-2 and p300/CBP. J. Biol. Chem., 276, 12645–12653 (2001).
   PubMed Web of Science ®Google Scholar
• Kanse SM , TakahashiK, WarrenJB, GhateiM, BloomSR. Glucocorticoids induce endothelin reléase from vascular smooth muscle cells but not endothelial cells. Eur. J. Pharmacol., 199, 99–101 (1991).
   PubMed Web of Science ®Google Scholar
• Rodríguez-Pascual F , ReimundeFM, Redondo-HorcajoM, LamasS. Transforming growth factor-beta induces endothelin-1 expression through activation of the Smad signaling pathway. J. Cardiovasc. Pharmacol., 44, S39–S42 (2004).
   PubMed Web of Science ®Google Scholar
• Malek AM , GreeneAL, IzumoS. Regulation of endothelin-1 gene by fluid shear stress is transcriptionally mediated and independent of protein kinase C and cAMP. Proc. Natl Acad. Sci. USA, 90, 5999–6003 (1993).
   PubMed Web of Science ®Google Scholar
• Malek AM , ZhangJ, JiangJ, AlpertSL, IzumoS. Endothelin-1 gene suppression by shear stress: pharmacological evaluation of the role of tyrosine kinase, intracellular calcium, cytoskeleton, and mechanosensitive channels. J. Mol. Cell. Cardiol., 31, 387–399 (1999).
   PubMed Web of Science ®Google Scholar
• Woods M , MitchellJA, WoodEGet al. Endothelin-1 is induced by cytokines in human vascular smooth muscle cells: evidence for intracellular endothelin-converting enzyme. Mol. Pharmacol., 55, 902–909 (1999).
   PubMed Web of Science ®Google Scholar
• Bouallegue A , DaouGB, SrivastavaAK. Endothelin-1 induced signaling pathways in vascular smooth muscle cells. Curr. Vasc. Pharmacol., 5, 45–52 (2007).
   PubMed Web of Science ®Google Scholar
• Maguire J , KucR, PellVet al. Comparison of human ETA and ETB receptor signaling via G-protein and β-arrestin pathways. Life Sci., 91, 544–549 (2012).
   PubMed Web of Science ®Google Scholar
• Maguire J , DavenportA. Endothelin receptors and their antagonists. Semin. Nephrol., 35(2), 125–136 (2015).
   PubMed Web of Science ®Google Scholar
• European Medicines Agency . Thelin (sitaxentan) to be withdrawn due to cases of unpredictable serious liver injury. Press release (2010).
   Google Scholar
• Kingman M , RuggieroR, TorresF. Ambrisentan, an endothelin receptor type A-selective endothelin receptor antagonist, for the treatment of pulmonary arterial hypertension. Expert. Opin. Pharmacother., 10(11), 1847–1858 (2009).
   PubMed Web of Science ®Google Scholar
• Scott LJ . Sitaxentan: in pulmonary arterial hypertension. Drugs, 67(5), 761–770 (2007).
   PubMed Web of Science ®Google Scholar
• Chen SJ , ChenYF, MengQC, DurandJ, DicarloVS, OparilS. Endothelin-receptor antagonist bosentan prevents and reverses hypoxic pulmonary hypertension in rats. J. Appl. Physiol., 79(6), 2122–2131 (1995).
   PubMed Web of Science ®Google Scholar
• Chen SJ , ChenYF, OpgenorthTJet al. The orally active nonpeptide endothelin A-receptor antagonist A-127722 prevents and reverses hypoxia-induced pulmonary hypertension and pulmonary vascular remodeling in Sprague–Dawley rats. J. Cardiovasc. Pharmacol., 29(6), 713–725 (1997).
   PubMed Web of Science ®Google Scholar
• Kim H , YungGL, MarshJJet al. Endothelin mediates pulmonary vascular remodeling in a canine model of chronic embolic pulmonary hypertension. Eur. Respir. J., 15(4), 640–648 (2000).
   PubMed Web of Science ®Google Scholar
• Tilton RG , MunschCL, SherwoodSJet al. Attenuation of pulmonary vascular hypertension and cardiac hypertrophy with sitaxsentan sodium, an orally active ET(A) receptor antagonist. Pulm. Pharmacol. Ther., 13(2), 87–97 (2000).
   PubMed Web of Science ®Google Scholar
• Rondelet B , KerbaulF, MotteSet al. Bosentan for the prevention of overcirculation-induced experimental pulmonary arterial hypertension. Circulation, 107, 1329–1335 (2003).
   PubMed Web of Science ®Google Scholar
• Hong I , CoeH, CatanzaroL. Macitentan for the treatment of pulmonary arterial hypertension. Ann. Pharmacother., 48(4), 538–547 (2014).
   PubMed Web of Science ®Google Scholar
• Sidharta PN , van GiersbergenPL, DingemanseJ. Safety, tolerability, pharmacokinetics, and pharmacodynamics of macitentan, an endothelin receptor antagonist, in an ascending multiple-dose study in healthy subjects. J. Clin. Pharmacol., 53, 1131–1138 (2013).
   PubMed Web of Science ®Google Scholar
• Opsumit , (Macitentan) tablets [product information]. Actelion Pharmaceuticals US, Inc, CA, USA (2013).
   Google Scholar
• Bruderer S , HopfgartnerG, SeiberlingMet al. Absorption, distribution, metabolism, and excretion of macitentan, a dual endothelin receptor antagonist, in humans. Xenobiotica. 42, 901–910 (2012).
   Google Scholar
• Gatfield J , GrandjeanCM, SasseT, ClozelM, NaylerO. Slow receptor dissociation kinetics differentiate macitentan from other endothelin receptor antagonists in pulmonary arterial smooth muscle cells. PLoS ONE, 7(10), e47662 (2012).
   PubMed Web of Science ®Google Scholar
• Iglarz M , BinkertC, MorrisonKet al. Pharmacology of macitentan, an orally active tissue-targeting dual endothelin receptor antagonist. J. Pharmacol. Exp. Ther., 327(3), 736–745 (2008).
   PubMed Web of Science ®Google Scholar
• Kummer O , HaschkeM, HammannFet al. Comparison of the dissolution and pharmacokinetic profiles of two galenical formulations of the endothelin receptor antagonist macitentan. Eur. J. Pharm. Sci., 38(4), 384–388 (2009).
   PubMed Web of Science ®Google Scholar
• Bolli M , BossC, BinkertC, BuchmannS, BurD, HessP. The discovery of N-[5-(4-bromophenyl)-6-[2-[(5-bromo-2-pyrimidinyl)oxy]ethoxy]-4-pyrimidinyl]-N’-propylsulfamide (macitentan), an orally active, potent dual endothelin receptor antagonist. J. Med. Chem., 55(17), 7849–7861 (2012).
   PubMed Web of Science ®Google Scholar
• Temple IP , MonfrediO, QuigleyGet al. Macitentan treatment retards the progression of established pulmonary arterial hypertension in an animal model. Int. J. Cardiol., 177, 423–428 (2014).
   PubMed Web of Science ®Google Scholar
• Sidharta P , van GiersbergenP, HalabiA, DingemanseJ. Macitentan: entry-into-humans study with a new endothelin receptor antagonist. Eur. J. Clin. Pharmacol., 67(10), 977–984 (2011).
   PubMed Web of Science ®Google Scholar
• Pulido T , AdzerikhoI, ChannickRNet al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N. Eng. J. Med., 369, 809–818 (2013).
   PubMed Web of Science ®Google Scholar
• Gatzoulis MA , LandzbergM, BeghettiMet al. Evaluation of macitentan in patients with Eisenmenger syndrome. Circulation, 139(1), 51–63 (2019).
   PubMed Web of Science ®Google Scholar
• Rosenkranz S , GalièN, ChannickRet al. Effects of macitentan onright ventricular remodeling in pulmonary arterial hypertension: results from the REPAIR study interim analysis. JAAC, 73, 1898 (2019).
   Google Scholar
• Ghofrani AH , SimonneauG, D’ArminiAet al. Macitentan for the treatment of inoperable chronic thromboembolic pulmonary hypertension (MERIT-1): results from the multicenter, Phase II, randomized, double-blind, placebo-controlled study. Lancet Respir. Med., 5, 785–794 (2017).
   PubMed Web of Science ®Google Scholar
• Sitbon O , BoschJ, CottreelEet al. Macitentan for the treatment of portopulmonary hypertension (PORTICO): a multicentre, randomised, double-blind, placebo-controlled, Phase IV trial. Lancet Respir. Med., 7(7), 594–604 (2019).
   PubMed Web of Science ®Google Scholar
• Channick RN , DelacroixM, GhofraniHAet al. Effect of macitentan on hospitalizations, results from the SERAPHIN trial. J. Am. Coll. Cardiol., 2(1), 1–8 (2015).
   Google Scholar
• ClinicalTrials.gov . Long-term extension study of the SERAPHIN study, to assess the safety and tolerability of ACT 064992 in patients with symptomatic pulmonary arterial hypertension (SERAPHIN OL). (2015). https://clinicaltrials.gov/ct2/show/NCT00667823
   Google Scholar
• Simonneau G , ChannickR, DelcroixMet al. Incident and prevalent cohorts with pulmonary arterial hypertension: insight from SERAPHIN. Eur. Respir. J., 46, 1711–1720 (2015).
   PubMed Web of Science ®Google Scholar
• Raghu G , Million-RousseauR, MorgantiA, PerchenetL, BehrJ, MUSICstudy group. Macitentan for the treatment of idiopathic pulmonary fibrosis: the randomised controlled MUSIC trial. Eur. Respir. J., 42(6), 1622–1632 (2013).
   PubMed Web of Science ®Google Scholar
• Zhang Y , WangN, GuZet al. A network meta-analysis for safety of endothelin receptor antagonists in pulmonary arterial hypertension. Cardiovasc. Diagn. Ther., 9(3), 239–249 (2019).
   PubMed Web of Science ®Google Scholar
• Wei A , GuZ, LiJet al. Clinical adverse effects of endothelin receptor antagonists: insights from the meta-analysis of 4894 patients from 24 randomized double-blind placebo-controlled clinical trials. J. Am. Heart Assoc., 5, e003896 doi:10.1161/JAHA.116.003896 (2016) ( Epub ahead of print).
   PubMed Web of Science ®Google Scholar
• Provencher S , GrantonJ. Current treatment approaches to pulmonary arterial hypertension. Can. J. Cardiol., 31, 460–477 (2015).
   PubMed Web of Science ®Google Scholar
• Mehta S , SastryBKS, SouzaRet al. Macitentan improves health-related quality of life in pulmonary arterial hypertension: results from the randomized controlled SERAPHIN trial. Chest, 151(1), 106–118 (2017).
   PubMed Web of Science ®Google Scholar
• Ghofrani HA , HumbertM. The role of combination therapy in managing pulmonary arterial hypertension. Eur. Respir. Rev., 23, 469–475 (2014).
   PubMedGoogle Scholar
• McLaughlin V , ChannikR, GhofraniHAet al. Effect of bosentan and sildenafil combination therapy on morbidity and mortality in pulmonary arterial hypertension (PAH): results from the COMPASS-2 study. Chest, 146, 860A (2014).
   Google Scholar
• Ghofrani HA , GalièN, GrimmingerFet al. Riociguat for the treatment of pulmonary arterial hypertension. N. Engl. J. Med., 369, 330–340 (2013).
   PubMed Web of Science ®Google Scholar
• Tapson VF , TorresF, KermeenFet al. Oral treprostinil for the treatment of pulmonary arterial hypertension in patients on background endothelin receptor antagonist and/or phosphodiesterase type 5 inhibitor therapy (the FREEDOM C study): a randomized controlled trial. Chest, 142, 1383–1390 (2012).
   PubMed Web of Science ®Google Scholar
• Tapson VF , JingZC, XuKFet 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, 144, 952–958 (2013).
   PubMed Web of Science ®Google Scholar
• Vizza C , JansaP, TealS, DombiT, ZhouD. Sildenafil dosed concomitantly with bosentan for adult pulmonary arterial hypertension in a randomized controlled trial. BMC Cardiovasc. Disord., 17, 239 (2017).
   PubMed Web of Science ®Google Scholar
• Galiè N , BarberàJA, FrostAEet al. Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension. N. Engl. J. Med., 373, 834–844 (2015).
   PubMed Web of Science ®Google Scholar
• FDA news release . FDA approves Opsumit to treat pulmonary arterial hypertension. (2013). http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm371362.htm
   Google Scholar
• Weiss J , TheileD, RuppellMA, SpeckT, SpalwiszA, HaefeliWE. Interaction profile of macitentan, a new non-selective endothelin-1 receptor antagonist, in vitro. Eur. J. Pharmacol., 701, 168–175 (2013).
   PubMed Web of Science ®Google Scholar
• Bruderer S , ÄänismaaP, HomeryMet al. Effect of cyclosporine and rifampin on the pharmacokinetics of macitentan, a tissue-targeting dual endothelin receptor antagonist. AAPS J., 14(1), 68–78 (2012).
   PubMed Web of Science ®Google Scholar
• Atsmon J , DingemanseJ, ShaikevichD, VolokhovI, SiddhartaPN. Investigations of the effects of ketoconazole on the pharmacokinetics of macitentan, a novel dual endothelin receptor antagonist, in healthy subjects. Clin. Pharmacokinet., 52(8), 685–692 (2013).
   PubMed Web of Science ®Google Scholar
• Corallo C , PecettiG, IglarzMet al. Macitentan slows down the dermal fibrotic process in systemic sclerosis: in vitro findings. J. Biol. Regul. Homeost. Agents, 27, 455–462 (2013).
   PubMed Web of Science ®Google Scholar
• Khanna D , DentonCP, MerkelPAet al. Effect of macitentan on the development of new ischemic digital ulcers in patients with systemic sclerosis: DUAL-1 and DUAL-2 randomized clinical trials. JAMA, 10, 1975–1988 (2016).
   Google Scholar
• Sen S , ChenS, FengB, IglarzM, ChakrabartiS. Renal, retinal and cardiac changes in Type 2 diabetes are attenuated by macitentan, a dual endothelin receptor antagonist. Life Sci., 91, 658–668 (2012).
   PubMed Web of Science ®Google Scholar
• Coffman L , MooneyC, LimJet al. Endothelin receptor-A is required for the recruitment of antitumor T cells and modulates chemotherapy induction of cancer stem cells. Cancer Biol. Ther., 14, 184–192 (2013).
   PubMed Web of Science ®Google Scholar
• Kim SJ , KimJS, KimSWet al. Macitentan (ACT-064992), a tissue-targeting endothelin receptor antagonist, enhances therapeutic efficacy of paclitaxel by modulating survival pathways in orthotopic models of metastatic human ovarian cancer. Neoplasia, 13, 167–179 (2011).
   PubMed Web of Science ®Google Scholar
• Kim SJ , KimJS, KimSWet al. Antivascular therapy for multidrug-resistant ovarian tumors by macitentan, a dual endothelin receptor antagonist. Transl. Oncol., 5, 39–47 (2012).
   PubMed Web of Science ®Google Scholar