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Current Cardiology Reviews

Editor-in-Chief

ISSN (Print): 1573-403X
ISSN (Online): 1875-6557

Pulmonary Hypertension: Types and Treatments

Author(s): Lisa J. Rose-Jones and Vallerie V. Mclaughlin

Volume 11, Issue 1, 2015

Page: [73 - 79] Pages: 7

DOI: 10.2174/1573403X09666131117164122

Price: $65

Abstract

Pulmonary arterial hypertension (PAH) is a panvasculopathy that affects the distal pulmonary arteries and leads to restricted blood flow. This increased afterload leads to adaptive mechanisms of the right ventricle, with eventual failure once it can no longer compensate. Pulmonary hypertension from associated conditions, most importantly left heart disease, i.e. heart failure, can also lead to the same sequela. Patients often experience early vague symptoms of dyspnea and exercise intolerance, and thus PH can elude clinicians until right heart failure symptoms predominate. Evidence-based treatment options with pulmonary vasodilators are available for those with PAH and should be employed early. It is essential that patients be accurately categorized by their etiology of PH, as treatment strategies differ, and can potentially be dangerous if employed in the wrong clinical scenario.

Keywords: Pulmonary arterial hypertension, pulmonary hypertension, pulmonary vascular disease, pulmonary venous hypertension, right ventricular failure.

Graphical Abstract
[1]
McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA. ACCF/AHA 2009 expert consensus document on pulmonary hypertension. J Am Coll Cardiol 2009; 119(16): 2250-94.
[2]
Thenappan T, Shah SJ, Rich S, et al. A USA-based registry for pulmonary arterial hypertension: 1982-2006. Eur Respir J 2007; 30: 1103-20.
[3]
Ghio S, Gavazzi A, Campana C, et al. Independent and additive prognostic value of right ventricular systolic function and pulmonary artery pressure in patients with chronic heart failure. J Am Coll Cardiol 2001; 37: 183-8.
[4]
Humbert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 2006; 173: 1023-30.
[5]
Peacock AJ, Murphy NF, McMurray JJV, et al. An epidemiological study of pulmonary arterial hypertension. Eur Respir J 2007; 30: 104-9.
[6]
McGoon M, Guetterman D, Steen V, et al. Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004; 126: 14S-34S.
[7]
Archer S, Rich S. Primary pulmonary hypertension: a vascular biology and translation research “Work in progress”. Circulation 2000; 102: 2781-91.
[8]
Christmas 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: 70-5.
[9]
Oudiz RJ. Pulmonary hypertension associated with left-sided heart disease. Clin Chest Med 2007; 28: 233-41.
[10]
Guglin M, Khan H. Pulmonary hypertension in heart failure. J Card Fail 2010; 16(6): 461-74.
[11]
Tryka AF, Godleski JJ, Shoen FJ, Vandevater SH. Pulmonary vascular disease and hypertension after valve surgery for mitral stenosis. Hum Pathol 1985; 16(1): 65-71.
[12]
Thabut G, Dauriat G, Stern JB, et al. Pulmonary hemodynamics in advanced COPD candidates for lung volume reduction surgery or lung transplantation. Chest 2005; 127: 1531-6.
[13]
Pengo V, Lensing AW, Prins MG, et al. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med 2004; 350: 2257-64.
[14]
Hoeper MM, Mayer E, Simmonneau G, et al. Chronic thromboembolic pulmonary hypertension. Circulation 2006; 113: 2011-20.
[15]
Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension: a national prospective study. Ann Intern Med 1987; 107: 216-23.
[16]
Simonneau G, Robbins IM, Beghetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009; 54(1)(Suppl.): S43-54.
[17]
Barst RJ, McGoon M, Torbicki A, et al. Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol 2004; 43: 40S-7S.
[18]
Thenappan T, Shah SJ, Gomberg-Maitland M, et al. Clinical characteristics of pulmonary hypertension in patients with heart failure and preserved ejection fraction. Circ Heart Fail 2011; 4: 257-65.
[19]
Lopez-Candales A, Dohi K, Iliescu A, et al. An abnormal right ventricular apical angle is indiciative of global right ventricular impairment. Echocardiography 2006; 23: 361-8.
[20]
Forfia PR. Forfia PR. Separating the ‘right from the left’ in patients with pulmonary hypertension. 2013 Feb Available from: http://mdprimer. com/opinioupdate/2010_1.htm.
[21]
Miyamoto S, Nagaya N, Satoh T, et al. Clinical correlates and prognostic significance of six-minute walk test in patients with primary pulmonary hypertension. Am J Respir Crit Care Med 2000; 161: 487-92.
[22]
Mathier MA. The nuts and bolts of interpreting hemodynamics in pulmonary hypertension associated with diastolic heart failure. Adv Pulmonary Hypertens 2011; 10: 33049.
[23]
Hemnes AR, Forfia PR, Champion HC. Assessment of pulmonary vasculature and right heart by invasive haemodynamics and echocardiography. Int J Clin Pract Suppl 2009; 63: 4-19.
[24]
Halpern SD, Taichman DB. Misclassification of pulmonary hypertension due to reliance on pulmonary capillary wedge pressure rather than left ventricular end-diastolic pressure. Chest 2009; 136: 37-43.
[25]
Hoeper MM, Lee S, Voswinckel R, et al. Complications of Right Heart Catherization Procedures in Patients with Pulmonary Hypertension in Experienced Centers. J Am Coll Cardiol 2006; 48: 2546-52.
[26]
Badesch DB, Abman SH, Simonneau G, et al. Medical therapy for pulmonary arterial hypertension: updated ACCP evidenced-based clinical practice guidelines. Chest 2007; 131: 1917-28.
[27]
Sitbon O, Humbert M, Jagot J-L, et al. Inhaled nitric oxide as a screening agent for safely identifying responders to oral calcium-channel blockers in primary pulmonary hypertension. Eur Respir J 1998; 12: 265-70.
[28]
Bocchi EA, Bacal F, Auler Junior JO, et al. Inhaled nitric oxide leading to pulmonary edema in stable severe heart failure. Am J Cardiol 1994; 74: 70-2.
[29]
Rich , et al. In Harrison’s Principles of Internal Medicine 15th edition. 2001; pp. 1506-7.
[30]
McGoon M, Frost A, Miller D, et al. Four-year outcomes of patients with pulmonary arterial hypertension: risk, prognosis, and the disease duration continuum. Chest October 2011; 140(4_Meeting Abstracts):. 724A.724A.
[http://dx.doi.org/10.1378/chest.1119437]
[31]
McLaughlin VV, McGoon M. Pulmonary arterial hypertension. Circulation 2006; 114: 1417-31.
[32]
Paul R. Forfia and Jean-Luc Vachiery. Echocardiography in pulmonary arterial hypertension. Am J Cardiol 2012; 6: S16-24.
[33]
Maruitz GJ, Kind T, Marcus JT, et al. Progressive changes in right ventricular geometric shortening and long-term survival in pulmonary arterial hypertension. Chest 2012; 141(4): 935-43.
[34]
Sitbon O, Humbert M, Jais X, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005; 111: 3105-11.
[35]
Gaile N, Ghofrani H, Torbicki A, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005; 353: 2148-57.
[36]
Gaile N, Brundage BH, Ghofrani H, et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation 2009; 119: 2894-903.
[37]
Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002; 346: 896-903.
[38]
Gaile N, Ribin LJ, Hoeper M, et al. Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial. Lancet 2008; 371: 2093-100.
[39]
Gaile N, Olschewski H, Oudiz RJ, et al. Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation 2008; 117: 3010-9.
[40]
United States Food and Drug Administration drug labeling. Medication Guide-Tracleer. Available from: http://www.fda.gov/ downloads/ Drugs/DrugSafety/UCM089801.pdf.
[41]
United States Food and Drug Administration drug labeling. Medication Guide - Letaris.Available from: http://www.fda.gov/ downloads/Drugs/DrugSafety/ucm088617.pdf.
[42]
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: 296-301.
[43]
Dadfarmay S, Berkowitz R, Kim B, et al. Differentiating pulmonary arterial and pulmonary venous hypertension and the implications for therapy. Congest Heart Fail 2010; 16: 287-91.
[44]
Reichenberger F, Pepke-Zaba J, McNeil K, et al. Atrial septostomy in the treatment of severe pulmonary arterial hypertension. Thorax 2003; 58: 797-800.
[45]
Rothman A, Sklansky MS, Lucas VW, et al. Atrial septostomy as a bridge to lung transplantation in patients with severe pulmonary hypertension. Am J Cardiol 1999; 84: 682-6.
[46]
Simonneau G, Robbins IM, Beghetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009; 54: S43-54.
[47]
Oudiz RJ. Pulmonary hypertension associated with left-sided heart disease. Clin Chest Med 2007; 28: 233-41.
[48]
Teerlink JR. Recent heart failure trials of neurohormonal modulation (OVERTURE and ENABLE): approaching the asymptome of efficacy? J Card Fail 2003; 8: 124-7.
[49]
Califf RM, Adams KF, McKenna WJ, et al. A randomized controlled trial of epoprostenol therapy for severe congestive heart failure: the Flolan International Randomized Survival Trial (FIRST). Am Heart J 1997; 134: 44-54.
[50]
Ontkean M, Gay R, Greenberg B. Diminished endothelium-derived relaxing factor activity in an experimental model of chronic heart failure. Circ Res 1991; 69: 1088-96.
[51]
Porter TR, Taylor DO, Cycan A, et al. Endothelium-dependent pulmonary artery responses in chronic heart failure: influence of pulmonary hypertension. J Am Coll Cardiol 1993; 22: 1418-24.
[52]
Guazzi M, Vicenzi M, Arena R, Guazzi MD. Pulmonary hypertension in heart failure with preserved ejection fraction. Circulation 2011; 124: 164-74.
[53]
M.M Redfield, H.H Chen, B.A Borlaug, et al. Effect of phosphodiesterase-5 inhibition on exercise capacity and clinical status in heart failure with preserved ejection fraction: a randomized clinical trial. JAMA 2013; 309(12): 1268-77.
[54]
Guazzi M, Samaja M, Arena R, et al. Long-term use of sildenafil in the therapeutic management of heart failure. J Am Coll Cardiol 2007; 50: 2136-44.
[55]
Lewis GD, Lachmann J, Camuso J, et al. Sildenafil improves exercise hemodynamics and oxygen uptake in patients with systolic heart failure. Circulation 2007; 115: 59-66.

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