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Current Respiratory Medicine Reviews

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ISSN (Print): 1573-398X
ISSN (Online): 1875-6387

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Review Article

A Literature Review of Pulmonary Arterial Hypertension (PAH)

Author(s): Ashima Panchal*, Jigar Panchal, Sonika Jain and Jaya Dwivedi

Volume 18, Issue 2, 2022

Published on: 29 April, 2022

Page: [104 - 114] Pages: 11

DOI: 10.2174/1573398X18666220217151152

Price: $65

Abstract

In 1891, German doctor E. Romberg was the first to report PAH. It is widespread throughout the world, but it is particularly problematic in India and other developing countries. Pulmonary arterial hypertension (PAH) is characterised by an increase in pulmonary arterial pressure as well as the emergence of progressive symptoms, such as a loss of functional ability, shortness of breath, and fatigue.

Blood flows from the right side of the heart to the lungs through the pulmonary arteries. Pulmonary arterial pressure refers to the pressure in the arteries of the lungs (PAH). It necessitates immediate treatment because high blood pressure in the lungs causes the right side of the heart to work much harder, increasing the risk of heart failure. This article aimed to provide brief information about the prevalence, pathology, classification, and different therapies of PAH.

Keywords: Pulmonary arterial hypertension (PAH), Pulmonary capillary wedge pressure (PCWP), pathology, pathogenesis, endothelin, blood pressure.

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Graphical Abstract

[1]
Matura LA, Carroll DL. Human responses to pulmonary arterial hypertension: review of the literature. J Cardiovasc Nurs 2010; 25(5): 420-7.
[http://dx.doi.org/10.1097/JCN.0b013e3181d25458] [PMID: 20714240]
[2]
Simonneau G, Galiè N, Rubin LJ, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol 2004; 43(12)(Suppl. S): 5S-12S.
[http://dx.doi.org/10.1016/j.jacc.2004.02.037] [PMID: 15194173]
[3]
Larrabee WF, Parker RL, Edwards JE. Pathology of intrapulmonary arteries and arterioles in mitral stenosis. Proc Staff Meet Mayo Clin 1949; 24(12): 316-26.
[PMID: 18150460]
[4]
Fishman AP. Respiratory gases in the regulation of the pulmonary circulation. Physiol Rev 1961; 41(1): 214-80.
[http://dx.doi.org/10.1152/physrev.1961.41.1.214] [PMID: 13700012]
[5]
Fritts HW Jr, Harris P, Clauss RH, Odell JE, Cournand A. The effect of acetylcholine on the human pulmonary circulation under normal and hypoxic conditions. J Clin Invest 1958; 37(1): 99-110.
[http://dx.doi.org/10.1172/JCI103590] [PMID: 13491717]
[6]
Wood P, Besterman EM, Towers MK, McIlroy MB. The effect of acetylcholine on pulmonary vascular resistance and left atrial pressure in mitral stenosis. Br Heart J 1957; 19(2): 279-86.
[http://dx.doi.org/10.1136/hrt.19.2.279] [PMID: 13413016]
[7]
Gurtner HP. Aminorex and pulmonary hypertension. A review. Cor Vasa 1985; 27(2-3): 160-71.
[PMID: 3928246]
[8]
Fishman AP. Primary pulmonary arterial hypertension: a look back. J Am Coll Cardiol 2004; 43(12)(Suppl. S): 2S-4S.
[http://dx.doi.org/10.1016/j.jacc.2004.03.019] [PMID: 15194172]
[9]
Hatano S, Strasser T. World Health Organization Primary pulmonary hypertension: report on a WHO meeting World Health Organization Geneva 197515-17 October 1973.
[10]
Fishman AP. The Pulmonary Circulation, Normal and Abnormal: Mechanisms, Management, and the National Registry. University of Pennsylvania Press 2016.
[11]
Pietra GG, Edwards WD, Kay JM, et al. Histopathology of primary pulmonary hypertension. A qualitative and quantitative study of pul-monary blood vessels from 58 patients in the National Heart, Lung, and Blood Institute, Primary Pulmonary Hypertension Registry. Circulation 1989; 80(5): 1198-206.
[http://dx.doi.org/10.1161/01.CIR.80.5.1198] [PMID: 2805258]
[12]
Badesch DB, Raskob GE, Elliott CG, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest 2010; 137(2): 376-87.
[http://dx.doi.org/10.1378/chest.09-1140] [PMID: 19837821]
[13]
Kumar U, Ramteke R, Yadav R, Ramam M, Handa R, Kumar A. Prevalence and predictors of pulmonary artery hypertension in systemic sclerosis. J Assoc Physicians India 2008; 56: 413-7.
[PMID: 18822619]
[14]
Chopra S, Badyal DK, Baby PC, Cherian D. Pulmonary arterial hypertension: advances in pathophysiology and management. Indian J Pharmacol 2012; 44(1): 4-11.
[http://dx.doi.org/10.4103/0253-7613.91858] [PMID: 22345861]
[15]
Thomson JR, Machado RD, Pauciulo MW, et al. Sporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR-II, a receptor member of the TGF-β family. J Med Genet 2000; 37(10): 741-5.
[http://dx.doi.org/10.1136/jmg.37.10.741] [PMID: 11015450]
[16]
Shao D, Park JE, Wort SJ. The role of endothelin-1 in the pathogenesis of pulmonary arterial hypertension. Pharmacol Res 2011; 63(6): 504-11.
[http://dx.doi.org/10.1016/j.phrs.2011.03.003] [PMID: 21419223]
[17]
Machado RD, Aldred MA, James V, et al. Mutations of the TGF-β type II receptor BMPR2 in pulmonary arterial hypertension. Hum Mutat 2006; 27(2): 121-32.
[http://dx.doi.org/10.1002/humu.20285] [PMID: 16429395]
[18]
Du L, Sullivan CC, Chu D, et al. Signaling molecules in nonfamilial pulmonary hypertension. N Engl J Med 2003; 348(6): 500-9.
[http://dx.doi.org/10.1056/NEJMoa021650] [PMID: 12571257]
[19]
Rudarakanchana N, Flanagan JA, Chen H, et al. Functional analysis of bone morphogenetic protein type II receptor mutations underlying primary pulmonary hypertension. Hum Mol Genet 2002; 11(13): 1517-25.
[http://dx.doi.org/10.1093/hmg/11.13.1517] [PMID: 12045205]
[20]
Jeffery TK, Morrell NW. Molecular and cellular basis of pulmonary vascular remodeling in pulmonary hypertension. Prog Cardiovasc Dis 2002; 45(3): 173-202.
[http://dx.doi.org/10.1053/pcad.2002.130041] [PMID: 12525995]
[21]
Christman BW, McPherson CD, Newman JH, et al. An imbalance between the excretion of thromboxane and prostacyclin metabolites in pulmonary hypertension. N Engl J Med 1992; 327(2): 70-5.
[http://dx.doi.org/10.1056/NEJM199207093270202] [PMID: 1603138]
[22]
Humbert M, Morrell NW, Archer SL, et al. Cellular and molecular pathobiology of pulmonary arterial hypertension. J Am Coll Cardiol 2004; 43(12)(Suppl. S): 13S-24S.
[http://dx.doi.org/10.1016/j.jacc.2004.02.029] [PMID: 15194174]
[23]
Morrell NW, Adnot S, Archer SL, et al. Cellular and molecular basis of pulmonary arterial hypertension. J Am Coll Cardiol 2009; 54(1)(Suppl.): S20-31.
[http://dx.doi.org/10.1016/j.jacc.2009.04.018] [PMID: 19555855]
[24]
Petkov V, Mosgoeller W, Ziesche R, et al. Vasoactive intestinal peptide as a new drug for treatment of primary pulmonary hypertension. J Clin Invest 2003; 111(9): 1339-46.
[http://dx.doi.org/10.1172/JCI17500] [PMID: 12727925]
[25]
Omland T, Lie RT, Aakvaag A, Aarsland T, Dickstein K. Plasma endothelin determination as a prognostic indicator of 1-year mortality after acute myocardial infarction. Circulation 1994; 89(4): 1573-9.
[http://dx.doi.org/10.1161/01.CIR.89.4.1573] [PMID: 8149523]
[26]
Giaid A, Yanagisawa M, Langleben D, et al. Expression of endothelin-1 in the lungs of patients with pulmonary hypertension. N Engl J Med 1993; 328(24): 1732-9.
[http://dx.doi.org/10.1056/NEJM199306173282402] [PMID: 8497283]
[27]
Gabbay E, Fraser J, McNeil K. Review of bosentan in the management of pulmonary arterial hypertension. Vasc Health Risk Manag 2007; 3(6): 887-900.
[PMID: 18200808]
[28]
Masaki T. Historical review: Endothelin. Trends Pharmacol Sci 2004; 25(4): 219-24.
[http://dx.doi.org/10.1016/j.tips.2004.02.008] [PMID: 15063086]
[29]
Clozel M, Gray GA, Breu V, Löffler B-M, Osterwalder R. The endothelin ETB receptor mediates both vasodilation and vasoconstriction in vivo. Biochem Biophys Res Commun 1992; 186(2): 867-73.
[http://dx.doi.org/10.1016/0006-291X(92)90826-7] [PMID: 1323294]
[30]
Levin ER. Endothelins. N Engl J Med 1995; 333(6): 356-63.
[http://dx.doi.org/10.1056/NEJM199508103330607] [PMID: 7609754]
[31]
Miwa S, Iwamuro Y, Zhang X-F, et al. Ca2+ entry channels in rat thoracic aortic smooth muscle cells activated by endothelin-1. Jpn J Pharmacol 1999; 80(4): 281-8.
[http://dx.doi.org/10.1254/jjp.80.281] [PMID: 10496327]
[32]
Hervé P, Launay J-M, Scrobohaci M-L, et al. Increased plasma serotonin in primary pulmonary hypertension. Am J Med 1995; 99(3): 249-54.
[33]
Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med 2004; 351(14): 1425-36.
[http://dx.doi.org/10.1056/NEJMra040291] [PMID: 15459304] [http://dx.doi.org/10.1016/S0002-9343(99)80156-9] [PMID: 7653484]
[34]
Eddahibi S, Chaouat A, Morrell N, et al. Polymorphism of the serotonin transporter gene and pulmonary hypertension in chronic obstruc-tive pulmonary disease. Circulation 2003; 108(15): 1839-44.
[http://dx.doi.org/10.1161/01.CIR.0000091409.53101.E8] [PMID: 14530202]
[35]
Raja SG, Raja SM. Treating pulmonary arterial hypertension: current treatments and future prospects. Ther Adv Chronic Dis 2011; 2(6): 359-70.
[http://dx.doi.org/10.1177/2040622311420773] [PMID: 23251761]
[36]
Rubin LJ. Primary pulmonary hypertension. N Engl J Med 1997; 336(2): 111-7.
[http://dx.doi.org/10.1056/NEJM199701093360207] [PMID: 8988890]
[37]
Humbert M, Nunes H, Sitbon O, Parent F, Hervé P, Simonneau G. Risk factors for pulmonary arterial hypertension. Clin Chest Med 2001; 22(3): 459-75.
[http://dx.doi.org/10.1016/S0272-5231(05)70284-7] [PMID: 11590841]
[38]
Davie N, Haleen SJ, Upton PD, et al. ET(A) and ET(B) receptors modulate the proliferation of human pulmonary artery smooth muscle cells. Am J Respir Crit Care Med 2002; 165(3): 398-405.
[http://dx.doi.org/10.1164/ajrccm.165.3.2104059] [PMID: 11818328]
[39]
Hirata Y, Emori T, Eguchi S, et al. Endothelin receptor subtype B mediates synthesis of nitric oxide by cultured bovine endothelial cells. J Clin Invest 1993; 91(4): 1367-73.
[http://dx.doi.org/10.1172/JCI116338] [PMID: 7682570]
[40]
Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002; 346(12): 896-903.
[http://dx.doi.org/10.1056/NEJMoa012212] [PMID: 11907289]
[41]
Rivera-Lebron BN, Risbano MG. Ambrisentan: a review of its use in pulmonary arterial hypertension. Ther Adv Respir Dis 2017; 11(6): 233-44.
[http://dx.doi.org/10.1177/1753465817696040] [PMID: 28425346]
[42]
Galié N, Badesch D, Oudiz R, et al. Ambrisentan therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2005; 46(3): 529-35.
[http://dx.doi.org/10.1016/j.jacc.2005.04.050] [PMID: 16053970]
[43]
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(3): 736-45.
[http://dx.doi.org/10.1124/jpet.108.142976] [PMID: 18780830]
[44]
Lau EMT, Giannoulatou E, Celermajer DS, Humbert M. Epidemiology and treatment of pulmonary arterial hypertension. Nat Rev Cardiol 2017; 14(10): 603-14.
[http://dx.doi.org/10.1038/nrcardio.2017.84] [PMID: 28593996]
[45]
Cohen H, Chahine C, Hui A, Mukherji R. Bosentan therapy for pulmonary arterial hypertension. Am J Health Syst Pharm 2004; 61(11): 1107-19.
[http://dx.doi.org/10.1093/ajhp/61.11.1107] [PMID: 15237563]
[46]
Kholdani CA, Fares WH, Trow TK. Macitentan for the treatment of pulmonary arterial hypertension. Vasc Health Risk Manag 2014; 10: 665-73.
[PMID: 25473292]
[47]
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(5): 296-301.
[http://dx.doi.org/10.1056/NEJM199602013340504] [PMID: 8532025]
[48]
Tapson VF, Gomberg-Maitland M, McLaughlin VV, et al. Safety and efficacy of IV treprostinil for pulmonary arterial hypertension: a prospective, multicenter, open-label, 12-week trial. Chest 2006; 129(3): 683-8.
[http://dx.doi.org/10.1378/chest.129.3.683] [PMID: 16537868]
[49]
Olschewski H, Simonneau G, Galiè N, et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 2002; 347(5): 322-9.
[http://dx.doi.org/10.1056/NEJMoa020204] [PMID: 12151469]
[50]
Okano Y, Yoshioka T, Shimouchi A, Satoh T, Kunieda T. Orally active prostacyclin analogue in primary pulmonary hypertension. Lancet 1997; 349(9062): 1365-5.
[http://dx.doi.org/10.1016/S0140-6736(97)24019-5] [PMID: 9149701]
[51]
Galiè N, Ghofrani HA, Torbicki A, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005; 353(20): 2148-57.
[http://dx.doi.org/10.1056/NEJMoa050010] [PMID: 16291984]
[52]
Galiè N, Brundage BH, Ghofrani HA, et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation 2009; 119(22): 2894-903.
[http://dx.doi.org/10.1161/CIRCULATIONAHA.108.839274] [PMID: 19470885]
[53]
Chaumais Marie-Camille . Macari Elise Artaud, Sitbon Olivier Calcium-channel blockers in pulmonary arterial hypertension Pharmacotherapy of Pulmonary Hypertension 2013; 161-75.
[http://dx.doi.org/10.1007/978-3-662-45805-1_7]
[54]
Sitbon O, Humbert M, Ioos V, Jais X, Parent F, Garcia G. Who benefits from long-term calcium-channel blocker therapy in primary pul-monary hypertension. Am J Respir Crit Care Med 2003; 167: A440.
[55]
Stasch J-P, Pacher P, Evgenov OV. Soluble guanylate cyclase as an emerging therapeutic target in cardiopulmonary disease. Circulation 2011; 123(20): 2263-73.
[http://dx.doi.org/10.1161/CIRCULATIONAHA.110.981738] [PMID: 21606405]
[56]
Stasch J-P, Becker EM, Alonso-Alija C, et al. NO-independent regulatory site on soluble guanylate cyclase. Nature 2001; 410(6825): 212-5.
[http://dx.doi.org/10.1038/35065611] [PMID: 11242081]
[57]
Humbert M, Coghlan JG, Ghofrani H-A, et al. Riociguat for the treatment of pulmonary arterial hypertension associated with connective tissue disease: results from PATENT-1 and PATENT-2. Ann Rheum Dis 2017; 76(2): 422-6.
[http://dx.doi.org/10.1136/annrheumdis-2015-209087] [PMID: 27457511]
[58]
Ghofrani H-A, Galiè N, Grimminger F, et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med 2013; 369(4): 330-40.
[http://dx.doi.org/10.1056/NEJMoa1209655] [PMID: 23883378]
[59]
Humbert M, Barst RJ, Robbins IM, et al. Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2. Eur Respir J 2004; 24(3): 353-9.
[http://dx.doi.org/10.1183/09031936.04.00028404] [PMID: 15358690]
[60]
Galiè N, Rubin Lj, Hoeper M, et al. Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EAR-LY study): a double-blind, randomised controlled trial. Lancet 2008; 371(9630): 2093-100.
[http://dx.doi.org/10.1016/S0140-6736(08)60919-8] [PMID: 18572079]
[61]
Hoeper MM, Leuchte H, Halank M, et al. Combining inhaled iloprost with bosentan in patients with idiopathic pulmonary arterial hyper-tension. Eur Respir J 2006; 28(4): 691-4.
[http://dx.doi.org/10.1183/09031936.06.00057906] [PMID: 17012628]
[62]
Marcos E, Adnot S, Pham MH, et al. Serotonin transporter inhibitors protect against hypoxic pulmonary hypertension. Am J Respir Crit Care Med 2003; 168(4): 487-93.
[http://dx.doi.org/10.1164/rccm.200210-1212OC] [PMID: 12773327]
[63]
Wang X-X, Zhang F-R, Shang Y-P, et al. Transplantation of autologous endothelial progenitor cells may be beneficial in patients with idio-pathic pulmonary arterial hypertension: a pilot randomized controlled trial. J Am Coll Cardiol 2007; 49(14): 1566-71.
[http://dx.doi.org/10.1016/j.jacc.2006.12.037] [PMID: 17418297]
[64]
Ghofrani HA, Hoeper MM, Halank M, et al. Riociguat for chronic thromboembolic pulmonary hypertension and pulmonary arterial hy-pertension: a phase II study. Eur Respir J 2010; 36(4): 792-9.
[http://dx.doi.org/10.1183/09031936.00182909] [PMID: 20530034]
[65]
Ghofrani HA, Morrell NW, Hoeper MM, et al. Imatinib in pulmonary arterial hypertension patients with inadequate response to estab-lished therapy. Am J Respir Crit Care Med 2010; 182(9): 1171-7.
[http://dx.doi.org/10.1164/rccm.201001-0123OC] [PMID: 20581169]
[66]
Schermuly RT, Dony E, Ghofrani HA, et al. Reversal of experimental pulmonary hypertension by PDGF inhibition. J Clin Invest 2005; 115(10): 2811-21.
[http://dx.doi.org/10.1172/JCI24838] [PMID: 16200212]
[67]
Dumitrascu R, Kulcke C, Königshoff M, et al. Terguride ameliorates monocrotaline-induced pulmonary hypertension in rats. Eur Respir J 2011; 37(5): 1104-18.
[http://dx.doi.org/10.1183/09031936.00126010] [PMID: 20947677]

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