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Drugs Acting on Pulmonary Hypertension and Pulmonary Thromboembolism: A Comprehensive Overview
Abstract
Pulmonary hypertension ( PH ) and pulmonary thrombo embolism ( PTE ) are serious medical conditions affecting the pulmonary circulation, often leading to significant morbidity and mortality if left untreated. Pulmonary hypertension is manifested by elevated by blood pressure in the pulmonary arteries, leading to increased workload on the right side of the heart. Numerous underlying conditions can lead to this syndrome, such as congenital heart diseases, idiopathic pulmonary arterial hypertension (IPAH), connective tissue diseases, and chronic thromboembolism pulmonary hypertension (CTEPH). Parameters of pulmonary hypertension include endothelial receptor antagonists ( REA s ), phospho diesterasec _ 5_ inhibitors ( PDE _ 5 inhibitors), Soluble granulated cycles stimulating, prosta cycling analogues and calcium channel blockers. Parameters of chronic thrombosis embolism pulmonary hypertension ( CTPEH ) include pulmonary endarterectomy, Ballon pulmonary angioplasty and Riocigual. Finally it is concluded that pharmaco therapy plays an important role in the management of pulmonary hypertension and chronic thrombosis embolism pulmonary hypertension.
References
Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RMF, Brida M, Carlsen J, Coats AJS, Escribano-Subias P, Ferrari P, Ferreira DS, Ghofrani HA, Giannakoulas G, Kiely DG, Mayer E, Meszaros G, Nagavci B, Olsson KM, Pepke-Zaba J, Quint JK, Rådegran G, Simonneau G, Sitbon O, Tonia T, Toshner M, Vachiery JL, Vonk Noordegraaf A, Delcroix M, Rosenkranz S., ESC/ERS Scientific Document Group. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022 Oct 11;43(38):3618-3731. [PubMed]
Condliffe R, Kovacs G. Identifying early pulmonary arterial hypertension in patients with systemic sclerosis. Eur Respir J. 2018 Apr;51(4) [PubMed]
Maron BA, Brittain EL, Choudhary G, Gladwin MT. Redefining pulmonary hypertension. Lancet Respir Med. 2018 Mar;6(3):168-170. [PubMed]
Maron BA, Wertheim BM, Gladwin MT. Under Pressure to Clarify Pulmonary Hypertension Clinical Risk. Am J Respir Crit Care Med. 2018 Feb 15;197(4):423-426. [PMC free article] [PubMed]
Kovacs G, Berghold A, Scheidl S, Olschewski H. Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review. Eur Respir J. 2009 Oct;34(4):888-94. [PubMed]
Simonneau G, Montani D, Celermajer DS, Denton CP, Gatzoulis MA, Krowka M, Williams PG, Souza R. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019 Jan;53(1) [PMC free article] [PubMed]
Bentley RF, Barker M, Esfandiari S, Wright SP, Valle FH, Granton JT, Mak S. Normal and Abnormal Relationships of Pulmonary Artery to Wedge Pressure During Exercise. J Am Heart Assoc. 2020 Nov 17;9(22):e016339. [PMC free article] [PubMed]
Eisman AS, Shah RV, Dhakal BP, Pappagianopoulos PP, Wooster L, Bailey C, Cunningham TF, Hardin KM, Baggish AL, Ho JE, Malhotra R, Lewis GD. Pulmonary Capillary Wedge Pressure Patterns During Exercise Predict Exercise Capacity and Incident Heart Failure. Circ Heart Fail. 2018 May;11(5):e004750. [PMC free article] [PubMed]
Garcia- Rivas G, Jerjes-Sánchez C, Rodriguez D, Garcia-Pelaez J, Trevino V. A systematic review of genetic mutations in pulmonary arterial hypertension. BMC Med Genet. 2017 Aug 02;18(1):82. [PMC free article] [PubMed]
Simonneau G, Galiè N, Rubin LJ, Langleben D, Seeger W, Domenighetti G, Gibbs S, Lebrec D, Speich R, Beghetti M, Rich S, Fishman A. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004 Jun 16;43(12 Suppl S):5S-12S. [PubMed]
Hoeper MM, Humbert M, Souza R, Idrees M, Kawut SM, Sliwa-Hahnle K, Jing ZC, Gibbs JS. A global view of pulmonary hypertension. Lancet Respir Med. 2016 Apr;4(4):306-22. [PubMed]
Leber L, Beaudet A, Muller A. Epidemiology of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: identification of the most accurate estimates from a systematic literature review. Pulm Circ. 2021 Jan-Mar;11(1):2045894020977300. [PMC free article] [PubMed]
Montani D, Girerd B, Jaïs X, Laveneziana P, Lau EMT, Bouchachi A, Hascoët S, Günther S, Godinas L, Parent F, Guignabert C, Beurnier A, Chemla D, Hervé P, Eyries M, Soubrier F, Simonneau G, Sitbon O, Savale L, Humbert M. Screening for pulmonary arterial hypertension in adults carrying a BMPR2 mutation. Eur Respir J. 2021 Jul;58(1) [PMC free article] [PubMed]
Lau EMT, Giannoulatou E, Celermajer DS, Humbert M. Epidemiology and treatment of pulmonary arterial hypertension. Nat Rev Cardiol. 2017 Oct;14(10):603-614. [PubMed]
Rosenkranz S, Gibbs JS, Wachter R, De Marco T, Vonk-Noordegraaf A, Vachiéry JL. Left ventricular heart failure and pulmonary hypertension. Eur Heart J. 2016 Mar 21;37(12):942-54. [PMC free article] [PubMed]
Lam CS, Roger VL, Rodeheffer RJ, Borlaug BA, Enders FT, Redfield MM. Pulmonary hypertension in heart failure with preserved ejection fraction: a community-based study. J Am Coll Cardiol. 2009 Mar 31;53(13):1119-26. [PMC free article] [PubMed]
Tichelbäcker T, Dumitrescu D, Gerhardt F, Stern D, Wissmüller M, Adam M, Schmidt T, Frerker C, Pfister R, Halbach M, Baldus S, Rosenkranz S. Pulmonary hypertension and valvular heart disease. Herz. 2019 Sep;44(6):491-501. [PubMed]
Weber L, Rickli H, Haager PK, Joerg L, Weilenmann D, Brenner R, Taramasso M, Baier P, Maisano F, Maeder MT. Haemodynamic mechanisms and long-term prognostic impact of pulmonary hypertension in patients with severe aortic stenosis undergoing valve replacement. Eur J Heart Fail. 2019 Feb;21(2):172-181. [PubMed]
Nathan SD, Barbera JA, Gaine SP, Harari S, Martinez FJ, Olschewski H, Olsson KM, Peacock AJ, Pepke-Zaba J, Provencher S, Weissmann N, Seeger W. Pulmonary hypertension in chronic lung disease and hypoxia. Eur Respir J. 2019 Jan;53(1) [PMC free article] [PubMed]
Hurdman J, Condliffe R, Elliot CA, Swift A, Rajaram S, Davies C, Hill C, Hamilton N, Armstrong IJ, Billings C, Pollard L, Wild JM, Lawrie A, Lawson R, Sabroe I, Kiely DG. Pulmonary hypertension in COPD: results from the ASPIRE registry. Eur Respir J. 2013 Jun;41(6):1292-301. [PubMed]
Delcroix M, Torbicki A, Gopalan D, Sitbon O, Klok FA, Lang I, Jenkins D, Kim NH, Humbert M, Jais X, Vonk Noordegraaf A, Pepke-Zaba J, Brénot P, Dorfmuller P, Fadel E, Ghofrani HA, Hoeper MM, Jansa P, Madani M, Matsubara H, Ogo T, Grünig E, D'Armini A, Galie N, Meyer B, Corkery P, Meszaros G, Mayer E, Simonneau G. ERS statement on chronic thromboembolic pulmonary hypertension. Eur Respir J. 2021 Jun;57(6) [PubMed]
Kramm T, Wilkens H, Fuge J, Schäfers HJ, Guth S, Wiedenroth CB, Weingard B, Huscher D, Pittrow D, Cebotari S, Hoeper MM, Mayer E, Olsson KM. Incidence and characteristics of chronic thromboembolic pulmonary hypertension in Germany. Clin Res Cardiol. 2018 Jul;107(7):548-553. [PMC free article] [PubMed]
Shlobin OA, Kouranos V, Barnett SD, Alhamad EH, Culver DA, Barney J, Cordova FC, Carmona EM, Scholand MB, Wijsenbeek M, Ganesh S, Lower EE, Engel PJ, Wort J, Price L, Wells AU, Nathan SD, Baughman RP. Physiological predictors of survival in patients with sarcoidosis-associated pulmonary hypertension: results from an international registry. Eur Respir J. 2020 May;55(5) [PubMed]
Boucly A, Cottin V, Nunes H, Jaïs X, Tazi A, Prévôt G, Reynaud-Gaubert M, Dromer C, Viacroze C, Horeau-Langlard D, Pison C, Bergot E, Traclet J, Weatherald J, Simonneau G, Valeyre D, Montani D, Humbert M, Sitbon O, Savale L. Management and long-term outcomes of sarcoidosis-associated pulmonary hypertension. Eur Respir J. 2017 Oct;50(4) [PubMed]
Meyer T, Binder L, Hruska N, Luthe H, Buchwald AB. Cardiac troponin I elevation in acute pulmonary embolism is associated with right ventricular dysfunction. J Am Coll Cardiol. 2000 Nov 01;36(5):1632-6. [PubMed]
Konstantinides S, Geibel A, Olschewski M, Kasper W, Hruska N, Jäckle S, Binder L. Importance of cardiac troponins I and T in risk stratification of patients with acute pulmonary embolism. Circulation. 2002 Sep 03;106(10):1263-8. [PubMed]
Horlander KT, Leeper KV. Troponin levels as a guide to treatment of pulmonary embolism. Curr Opin Pulm Med. 2003 Sep;9(5):374-7. [PubMed]
Righini M, Van Es J, Den Exter PL, Roy PM, Verschuren F, Ghuysen A, Rutschmann OT, Sanchez O, Jaffrelot M, Trinh-Duc A, Le Gall C, Moustafa F, Principe A, Van Houten AA, Ten Wolde M, Douma RA, Hazelaar G, Erkens PM, Van Kralingen KW, Grootenboers MJ, Durian MF, Cheung YW, Meyer G, Bounameaux H, Huisman MV, Kamphuisen PW, Le Gal G. Age-adjusted D-dimer cutoff levels to rule out pulmonary embolism: the ADJUST-PE study. JAMA. 2014 Mar 19;311(11):1117-24. [PubMed]
Rodger M, Makropoulos D, Turek M, Quevillon J, Raymond F, Rasuli P, Wells PS. Diagnostic value of the electrocardiogram in suspected pulmonary embolism. Am J Cardiol. 2000 Oct 01;86(7):807-9, A10. [PubMed]
Worsley DF, Alavi A, Aronchick JM, Chen JT, Greenspan RH, Ravin CE. Chest radiographic findings in patients with acute pulmonary embolism: observations from the PIOPED Study. Radiology. 1993 Oct;189(1):133-6. [PubMed]
Ghaye B, Szapiro D, Mastora I, Delannoy V, Duhamel A, Remy J, Remy-Jardin M. Peripheral pulmonary arteries: how far in the lung does multi-detector row spiral CT allow analysis? Radiology. 2001 Jun;219(3):629-36. [PubMed]
Stein PD, Fowler SE, Goodman LR, Gottschalk A, Hales CA, Hull RD, Leeper KV, Popovich J, Quinn DA, Sos TA, Sostman HD, Tapson VF, Wakefield TW, Weg JG, Woodard PK., PIOPED II Investigators. Multidetector computed tomography for acute pulmonary embolism. N Engl J Med. 2006 Jun 01;354(22):2317-27. [PubMed]
Becattini C, Agnelli G, Vedovati MC, Pruszczyk P, Casazza F, Grifoni S, Salvi A, Bianchi M, Douma R, Konstantinides S, Lankeit M, Duranti M. Multidetector computed tomography for acute pulmonary embolism: diagnosis and risk stratification in a single test. Eur Heart J. 2011 Jul;32(13):1657-63. [PubMed]
Reid JH, Coche EE, Inoue T, Kim EE, Dondi M, Watanabe N, Mariani G., International Atomic Energy Agency Consultants' Group. Is the lung scan alive and well? Facts and controversies in defining the role of lung scintigraphy for the diagnosis of pulmonary embolism in the era of MDCT. Eur J Nucl Med Mol Imaging. 2009 Mar;36(3):505-21. [PubMed]
Glaser JE, Chamarthy M, Haramati LB, Esses D, Freeman LM. Successful and safe implementation of a trinary interpretation and reporting strategy for V/Q lung scintigraphy. J Nucl Med. 2011 Oct;52(10):1508-12. [PubMed]
Anderson DR, Kahn SR, Rodger MA, Kovacs MJ, Morris T, Hirsch A, Lang E, Stiell I, Kovacs G, Dreyer J, Dennie C, Cartier Y, Barnes D, Burton E, Pleasance S, Skedgel C, O'Rouke K, Wells PS. Computed tomographic pulmonary angiography vs ventilation-perfusion lung scanning in patients with suspected pulmonary embolism: a randomized controlled trial. JAMA. 2007 Dec 19;298(23):2743-53. [PubMed]
Sostman HD, Stein PD, Gottschalk A, Matta F, Hull R, Goodman L. Acute pulmonary embolism: sensitivity and specificity of ventilation-perfusion scintigraphy in PIOPED II study. Radiology. 2008 Mar;246(3):941-6. [PubMed]
PIOPED Investigators. Value of the ventilation/perfusion scan in acute pulmonary embolism. Results of the prospective investigation of pulmonary embolism diagnosis (PIOPED). JAMA. 1990 May 23-30;263(20):2753-9. [PubMed]
Wittram C, Waltman AC, Shepard JA, Halpern E, Goodman LR. Discordance between CT and angiography in the PIOPED II study. Radiology. 2007 Sep;244(3):883-9. [PubMed]
Stein PD, Chenevert TL, Fowler SE, Goodman LR, Gottschalk A, Hales CA, Hull RD, Jablonski KA, Leeper KV, Naidich DP, Sak DJ, Sostman HD, Tapson VF, Weg JG, Woodard PK., PIOPED III (Prospective Investigation of Pulmonary Embolism Diagnosis III) Investigators. Gadolinium-enhanced magnetic resonance angiography for pulmonary embolism: a multicenter prospective study (PIOPED III). Ann Intern Med. 2010 Apr 06;152(7):434-43, W142-3. [PMC free article] [PubMed]
Grifoni S, Olivotto I, Cecchini P, Pieralli F, Camaiti A, Santoro G, Conti A, Agnelli G, Berni G. Short-term clinical outcome of patients with acute pulmonary embolism, normal blood pressure, and echocardiographic right ventricular dysfunction. Circulation. 2000 Jun 20;101(24):2817-22. [PubMed]
Torbicki A, Kurzyna M, Ciurzynski M, Pruszczyk P, Pacho R, Kuch-Wocial A, Szulc M. Proximal pulmonary emboli modify right ventricular ejection pattern. Eur Respir J. 1999 Mar;13(3):616-21. [PubMed]
Bova C, Greco F, Misuraca G, Serafini O, Crocco F, Greco A, Noto A. Diagnostic utility of echocardiography in patients with suspected pulmonary embolism. Am J Emerg Med. 2003 May;21(3):180-3. [PubMed]
Wolfe MW, Lee RT, Feldstein ML, Parker JA, Come PC, Goldhaber SZ. Prognostic significance of right ventricular hypokinesis and perfusion lung scan defects in pulmonary embolism. Am Heart J. 1994 May;127(5):1371-5. [PubMed]
Kurzyna M, Torbicki A, Pruszczyk P, Burakowska B, Fijałkowska A, Kober J, Oniszh K, Kuca P, Tomkowski W, Burakowski J, Wawrzyńska L. Disturbed right ventricular ejection pattern as a new Doppler echocardiographic sign of acute pulmonary embolism. Am J Cardiol. 2002 Sep 01;90(5):507-11. [PubMed]
Pruszczyk P, Goliszek S, Lichodziejewska B, Kostrubiec M, Ciurzyński M, Kurnicka K, Dzikowska-Diduch O, Palczewski P, Wyzgal A. Prognostic value of echocardiography in normotensive patients with acute pulmonary embolism. JACC Cardiovasc Imaging. 2014 Jun;7(6):553-60. [PubMed]
Hull RD, Hirsh J, Carter CJ, Jay RM, Dodd PE, Ockelford PA, Coates G, Gill GJ, Turpie AG, Doyle DJ, Buller HR, Raskob GE. Pulmonary angiography, ventilation lung scanning, and venography for clinically suspected pulmonary embolism with abnormal perfusion lung scan. Ann Intern Med. 1983 Jun;98(6):891-9. [PubMed]
Kearon C, Ginsberg JS, Hirsh J. The role of venous ultrasonography in the diagnosis of suspected deep venous thrombosis and pulmonary embolism. Ann Intern Med. 1998 Dec 15;129(12):1044-9. [PubMed]
Le Gal G, Righini M, Sanchez O, Roy PM, Baba-Ahmed M, Perrier A, Bounameaux H. A positive compression ultrasonography of the lower limb veins is highly predictive of pulmonary embolism on computed tomography in suspected patients. Thromb Haemost. 2006 Jun;95(6):963-6. [PubMed]
van Rossum AB, van Houwelingen HC, Kieft GJ, Pattynama PM. Prevalence of deep vein thrombosis in suspected and proven pulmonary embolism: a meta-analysis. Br J Radiol. 1998 Dec;71(852):1260-5. [PubMed]
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