An Ephemeral Review on Pulmonary Arterial Hypertension
American Journal of Internal Medicine
Volume 7, Issue 4, July 2019, Pages: 81-85
Received: May 23, 2019; Accepted: Jun. 25, 2019; Published: Jul. 9, 2019
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Authors
Akshima Gupta, Department of Clinical Pharmacy, Vaageswari College of Pharmacy, Karimnagar, Telangana, India
Kanaparthi Sushrutha, Department of Clinical Pharmacy, Vaageswari College of Pharmacy, Karimnagar, Telangana, India
Nikhil Kumar Vanjari, Department of Clinical Pharmacy, Vaageswari College of Pharmacy, Karimnagar, Telangana, India
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Abstract
Pulmonary arterial hypertension [PAH] is a rare and potentially fatal disease whose management is usually restricted to a few specialized centers. The crucial vascular modifications in pulmonary arterial hypertension are endothelial-cell proliferation, vasoconstriction, thrombosis and smooth-muscle cell. As patients don’t essentially board in the neighborhood to those centers, daily care and emergencies ought to be delegated to the primary and second lines. Reduced contractility of mycardium, decreased venous return and abnormal rate of exchange of gases leads to deprivation of oxygen to cell and death. Diagnosing and management of pulmonary arterial hypertension is critical. There is no cure for PAH. Modern developments regarding cell biology, molecular genetics and of idiopathic pulmonary arterial hypertension create new insights and therapeutic targets in the management. However, there are several treatment options that aim to reduce symptoms, improve the quality of life, and slow disease progression. This short review provides an outline of our therapeutic protocols supported out there information. Based on the analysis of the reasons for death in the PAH population, a review of the main emergencies is provided. Drugs include vasodilators, anticoagulants, antiplatelet agents, antiinflammatory therapies, and vascular-remodeling therapies. Most of the drugs have pleiotropic effects.
Keywords
Pulmonary Arterial Hypertension, Nitric Oxide, Hypoxemia, Thromboembolism, Vasopressors
To cite this article
Akshima Gupta, Kanaparthi Sushrutha, Nikhil Kumar Vanjari, An Ephemeral Review on Pulmonary Arterial Hypertension, American Journal of Internal Medicine. Vol. 7, No. 4, 2019, pp. 81-85. doi: 10.11648/j.ajim.20190704.11
Copyright
Copyright © 2019 Authors retain the copyright of this article.
This article is an open access article distributed under the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
References
[1]
Hoeper, M. M.; Bogaard, H. J.; Condliffe, R.; Frantz, R.; Khanna, D.; Kurzyna, M.; Langleben, D.; Manes, A.; Satoh, T.; Torres, F. Definitions and diagnosis of pulmonary hypertension. J. Am. Coll. Cardiol. 2013, 62, D42–D50.
[2]
Moreira, E. M.; Gall, H.; Leaning, M. J.; Lahousse, L.; Loth, D. W.; Krijthe, B. P.; Kiefte-de Jong, J. C.; Brusselle, G. G.; Hofman, A.; Stricker, B. H. Prevalence of pulmonary hypertension in the general population: The Rotterdam study. PLoS ONE 2015, 10, e0130072.
[3]
Tuder, R. M.; Archer, S. L.; Dorfmüller, P.; Erzurum, S. C.; Guignabert, C.; Michelakis, E.; Rabinovitch, M.; Schermuly, R.; Stenmark, K. R.; Morrell, N. W. Relevant issues in the pathology and pathobiology of pulmonary hypertension. J. Am. Coll. Cardiol. 2013, 62, D4–D12.
[4]
Lundberg JO, Weitzberg E, Gladwin MT. The nitrate-nitrite-nitric oxide pathway in physiology and therapeutics. Nature Reviews Drug Discovery. 2008; 7: 156–167.
[5]
Giaid, A.; Saleh, D. Reduced expression of endothelial nitric oxide synthase in the lungs of patients with pulmonary hypertension. N. Engl. J. Med. 1995, 333, 214–221.
[6]
Zhao, Y.-Y.; Zhao, Y. D.; Mirza, M. K.; Huang, J. H.; Potula, H.-H. S.; Vogel, S. M.; Brovkovych, V.; Yuan, J. X.-J.; Wharton, J.; Malik, A. B. Persistent Enos activation secondary to caveolin-1 deficiency induces pulmonary hypertension in mice and humans through pkg nitration. J. Clin. Investig. 2009, 119, 2009–2018.
[7]
Mubarak KK. A review of prostaglandin analogs in the management of patients with pulmonary arterial hypertension. Respir Med 2010; 104: 9–21.
[8]
Christman BW, McPherson CD, Newman JH, King GA, Bernard GR, Groves BM, Loyd JE. An imbalance between the excretion of thromboxane and prostacyclin metabolites in pulmonary hypertension. New England Journal of Medicine. 1992 Jul 9; 327 [2]: 70-5.
[9]
Yanagisawa, M.; Kurihara, H.; Kimura, S.; Tomobe, Y.; Kobayashi, M.; Mitsui, Y.; Yazaki, Y.; Goto, K.; Masaki, T. A novel potent vasoconstrictor peptide produced by vascular endothelial cells. Nature 1988, 332, 411–415.
[10]
Galié N, Manes A, Branzi A. The endothelin system in pulmonary arterial hypertension. Cardiovascular research. 2004 Feb 1; 61 [2]: 227-37.
[11]
Hoeper MM. Definition, classification, and epidemiology of pulmonary arterial hypertension. seminars in respiratory and critical care medicine 2009 Aug [Vol. 30, No. 04, pp. 369-375].
[12]
Humbert M, Nunes H, Sitbon O, Parent F, Hervé P, Simonneau G. Risk factors for pulmonary arterial hypertension. Clinics in chest medicine. 2001 Sep 1; 22 [3]: 459-75.
[13]
Simonneau G, Galiè N, Rubin LJ, Langleben D, Seeger W, Domenighetti G, Gibbs S, Lebrec D, Speich R, Beghetti M, Rich S. Clinical classification of pulmonary hypertension. Journal of the American College of Cardiology. 2004 Jun 16; 43 [12 Supplement]: S5-12.
[14]
Bazan IS, Fares WH. Pulmonary hypertension: diagnostic and therapeutic challenges. Therapeutics and clinical risk management. 2015; 11: 1221.
[15]
Naeije R. Treatment of right heart failure on pulmonary arterial hypertension: is going left a step in the right direction? Eur Respir Rev 2010; 19: 4–6.
[16]
Galie N, Palazzini M, Manes A. Pulmonary arterial hypertension: from the kingdom of the near-dead to multiple clinical trial meta-analyses. Eur Heart J 2010; [Epub ahead of print DOI: 10.1093/ our heart/ehq152].
[17]
Minai OA, Yared JP, Kaw R, et al. Perioperative risk and management in patients with pulmonary hypertension. Chest. 2013; 144: 329-340.
[18]
Brown LM, Chen H, Halpern S, et al. Delay in recognition of pulmonary arterial hypertension: factors identified from the REVEAL registry. Chest. 2011; 140: 19-26.
[19]
Dela Cruz M, Devey JS. Emergency department diagnosis of pulmonary hypertension in a patient with left atrial sarcoma. Int J Emerg Med. 2014; 7: 32-014-0032-5.
[20]
Devaraj A, Wells AU, Meister MG, et al. Detection of pulmonary hypertension with multidetector CT and echocardiography alone and in combination. Radiology. 2010; 254: 609-616.
[21]
Grubstein A, Benjaminov O, Dayan DB, et al. Computed tomography angiography in pulmonary hypertension. Isr Med Assoc J. 2008; 10: 117-120.
[22]
Raman SV, Shah M, McCarthy B, et al. Multi-detector row cardiac computed tomography accurately quantifies right and left ventricular size and function compared with cardiac magnetic resonance. Am Heart J. 2006; 151: 736-744.
[23]
Quiroz R, Kucher N, Schoepf UJ, et al. Right ventricular enlargement on chest computed tomography: prognostic role in acute pulmonary embolism. Circulation. 2004; 109: 2401-2404.
[24]
Schoepf UJ, Kucher N, Kipfmueller F, et al. Right ventricular enlargement on chest computed tomography: a predictor of early death in acute pulmonary embolism. Circulation. 2004; 110: 3276-3280.
[25]
Maxwell BG, Pearl RG, Kudelko KT, et al. Case 7-2012. Airway management and perioperative decision making in the patient with severe pulmonary hypertension who requires emergency noncardiac surgery. J Cardiothorac Vasc Anesth. 2012; 26: 940-944.
[26]
De Backer D, Boston P, Devriendt J, et al. Comparison of dopamine and norepinephrine in the treatment of shock. N Engl J Med. 2010; 362: 779-789.
[27]
De Backer D, Aldecoa C, Njimi H, et al. Dopamine versus norepinephrine in the treatment of septic shock: a meta-analysis. Crit Care Med. 2012; 40: 725-730.
[28]
Dellinger RP, Levy MM, Rhodes A, et al. Surviving Sepsis Campaign: international guidelines for the management of severe sepsis and septic shock: 2012. Crit Care Med. 2013; 41: 580-637.
[29]
Rich S, Gubin S, Hart K. The effects of phenylephrine on right ventricular performance in patients with pulmonary hypertension. Chest. 1990; 98: 1102-1106.
[30]
Kwak YL, Lee CS, Park YH, et al. The effect of phenylephrine and norepinephrine in patients with chronic pulmonary hypertension. Anesthesia. 2002; 57: 9-14.
[31]
Price LC, Wort SJ, Finney SJ, et al. Pulmonary vascular and right ventricular dysfunction in adult critical care: current and emerging options for management: a systematic literature review. Crit Care. 2010; 14: R169.
[32]
Currigan DA, Hughes RJ, Wright CE, et al. Vasoconstrictor responses to vasopressor agents in human pulmonary and radial arteries: an in vitro study. Anesthesiology. 2014; 121: 930-936.
[33]
Hoeper MM, Granton J. Intensive care unit management of patients with severe pulmonary hypertension and right heart failure. Am J Respir Crit Care Med. 2011; 184: 1114-1124.
[34]
Lahm T, McCaslin CA, Wozniak TC, et al. Medical and surgical treatment of acute right ventricular failure. J Am Coll Cardiol. 2010; 56: 1435-1446.
[35]
Lopez BL, Christopher TA, Griswold SK, et al. Bench to bedside: nitric oxide in emergency medicine. Acad Emerg Med. 2000; 7: 285-293.
[36]
Frostell CG, Blomqvist H, Hedenstierna G, et al. Inhaled nitric oxide selectively reverses human hypoxic pulmonary vasoconstriction without causing systemic vasodilation. Anesthesiology. 1993; 78: 427-435.
[37]
Ismail K, Roberts K, Manning P, et al. OSA and pulmonary hypertension: time for a new look. Chest. 2015; 147: 847-861.
[38]
Maxwell BG, Pearl RG, Kudelko KT, et al. Case 7-2012. Airway management and perioperative decision making in the patient with severe pulmonary hypertension who requires emergency noncardiac surgery. J Cardiothorac Vasc Anesth. 2012; 26: 940-944.
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