Hostname: page-component-8448b6f56d-cfpbc Total loading time: 0 Render date: 2024-04-24T17:04:32.488Z Has data issue: false hasContentIssue false

Conventional and targeted medical therapies

Published online by Cambridge University Press:  01 May 2009

Robyn J. Barst*
Affiliation:
Professor Emeritus of Pediatrics, Columbia UniversityNew York, New York, United States of America
Duncan Stewart
Affiliation:
The Ottawa Hospital, Ottawa, Ontario, Canada
*
Correspondence to: Robyn J. Barst, MD, 31 Murray Hill Road, Scarsdale, NY 10583, USA. E-mail: robyn.barst@gmail.com

Abstract

Image of the first page of this content. For PDF version, please use the ‘Save PDF’ preceeding this image.'
Type
Original Article
Copyright
Copyright © Cambridge University Press 2009

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1.Gatzoulis, MA, Barst, R, Fineman, J, Galie, N. Eisenmenger syndrome and pulmonary arterial hypertension in adults with congenital heart disease. Curr Med Res Opin 2007; 23 (Suppl 2): S1S7.CrossRefGoogle Scholar
2.D’Alonzo, GE, Barst, RJ, Ayres, SM, et al. Survival in Patients with Primary Pulmonary Hypertension: Results of a National Prospective Registry. Annals Int Med 1991; 115: 343349.CrossRefGoogle ScholarPubMed
3.Badesch, DB, Abman, SH, Simonneau, G, Rubin, LJ, McLaughlin, VV. Medical Therapy for Pulmonary Arterial Hypertension. Updated ACCP Evidence-Based Clinical Practice Guidelines. Chest 2007; 131: 19171928.CrossRefGoogle ScholarPubMed
4.Humbert, M, Nunes, H, Sitbon, O, Parent, F, Herve, P, Simonneau, G. Risk factors for pulmonary arterial hypertension. Clin Chest Med 2001; 22: 459475.CrossRefGoogle ScholarPubMed
5.Recommendations on the management of pulmonary hypertension in clinical practice. Heart 2001; 86 (Suppl 1): I1I13.Google Scholar
6.Humbert, M, Sitbon, O, Simonneau, G. Treatment of pulmonary arterial hypertension. N Engl J Med 2004; 351: 14251436.CrossRefGoogle ScholarPubMed
7.Bowyer, JJ, Busst, CM, Denison, DM, Shinebourne, EA. Effect of long term oxygen treatment at home in children with pulmonary vascular disease. Br Heart J 1986; 55: 385390.CrossRefGoogle ScholarPubMed
8.Sandoval, J, Aguirre, JS, Pulido, T, et al. Nocturnal oxygen therapy in patients with the Eisenmenger syndrome. Am J Respir Crit Care Med 2001; 164: 16821687.CrossRefGoogle ScholarPubMed
9.Rich, S, Seidlitz, M, Dodin, E, et al. The short-term effects of digoxin in patients with right ventricular dysfunction from pulmonary hypertension. Chest 1998; 114: 787792.CrossRefGoogle ScholarPubMed
10.Fuster, V, Steele, PM, Edwards, WD, Gersh, BJ, McGoon, MD, Frye, RL. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation 1984; 70: 580587.CrossRefGoogle ScholarPubMed
11.Rich, S, Kaufmann, E, Levy, PS. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med 1992; 327: 7681.CrossRefGoogle ScholarPubMed
12.Niwa, K, Perloff, JK, Kaplan, S, Child, JS, Miner, PD. Eisenmenger syndrome in adults: ventricular septal defect, truncus arteriosus, univentricular heart. J Am Coll Cardiol 1999; 34: 223232.CrossRefGoogle ScholarPubMed
13.Rich, S. In: World Symposium on Primary pulmonary Hypertension; 1998; Evian, France; 1998.Google Scholar
14.Lockshin, M, Tenedios, F, Petri, M, et al. Cardiac disease in the antiphospholipid syndrome: recommendations for treatment. Committee consensus report. Lupus 2003; 12: 518523.CrossRefGoogle ScholarPubMed
15.Rosenzweig, EB, Widlitz, AC, Barst, RJ. Pulmonary arterial hypertension in children. Pediatr Pulmonol 2004; 38: 222.CrossRefGoogle ScholarPubMed
16.Gaine, S. Pulmonary hypertension. Jama 2000; 284: 31603168.CrossRefGoogle ScholarPubMed
17.Sitbon, O, Humbert, M, JaÔs, X, et al. Long-Term Response to Calcium Channel Blockers in Idiopathic Pulmonary Arterial Hypertension. Circulation 2005; 111: 31053111.CrossRefGoogle ScholarPubMed
18.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: 7075.CrossRefGoogle ScholarPubMed
19.Adatia, I, Haworth, SG. Circulating endothelin in children with congenital heart disease. Br Heart J 1993; 69: 233236.CrossRefGoogle ScholarPubMed
20.Tuder, RM, Cool, CD, Geraci, MW, et al. Prostacyclin synthase expression is decreased in lungs from patients with severe pulmonary hypertension. Am J Respir Crit Care Med 1999; 159: 19251932.CrossRefGoogle ScholarPubMed
21.Barst, RJ, Rubin, LJ, Long, WA, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group. N Engl J Med 1996; 334: 296302.CrossRefGoogle Scholar
22.Shapiro, SM, Oudiz, RJ, Cao, T, et al. Primary pulmonary hypertension: improved long-term effects and survival with continuous intravenous epoprostenol infusion. J Am Coll Cardiol 1997; 30: 343349.CrossRefGoogle ScholarPubMed
23.McLaughlin, VV, Genthner, DE, Panella, MM, Rich, S. Reduction in pulmonary vascular resistance with long-term epoprostenol (prostacyclin) therapy in primary pulmonary hypertension. N Engl J Med 1998; 338: 273277.CrossRefGoogle ScholarPubMed
24.Sitbon, O, Humbert, M, Nunes, H, et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol 2002; 40: 780788.CrossRefGoogle ScholarPubMed
25.Badesch, DB, Tapson, VF, McGoon, MD, et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med 2000; 132: 425434.CrossRefGoogle Scholar
26.McLaughlin, VV, Genthner, DE, Panella, MM, Hess, DM, Rich, S. Compassionate use of continuous prostacyclin in the management of secondary pulmonary hypertension: a case series. Ann Intern Med 1999; 130: 730733.CrossRefGoogle ScholarPubMed
27.Rosenzweig, EB, Kerstein, D, Barst, RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999; 99: 18581865.CrossRefGoogle ScholarPubMed
28.McLaughlin, VV, Gaine, SP, Barst, RJ, et al. Efficacy and safety of treprostinil: an epoprostenol analog for primary pulmonary hypertension. J Cardiovasc Pharmacol 2003; 41: 293299.CrossRefGoogle ScholarPubMed
29.Simonneau, G, Barst, RJ, Galie, N, et al. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary hypertension. Am J Respir Crit Care Med 2002; 165: 15.CrossRefGoogle Scholar
30.Higenbottam, TW, Butt, AY, Dinh-Xaun, AT, Takao, M, Cremona, G, Akamine, S. Treatment of pulmonary hypertension with the continuous infusion of a prostacyclin analogue, iloprost. Heart 1998; 79: 175179.CrossRefGoogle ScholarPubMed
31.Higenbottam, T, Butt, AY, McMahon, A, Westerbeck, R, Sharples, L. Long-term intravenous prostaglandin (epoprostenol or iloprost) for treatment of severe pulmonary hypertension. Heart 1998; 80: 151155.CrossRefGoogle ScholarPubMed
32.Olschewski, H, Simonneau, G, Galie, N, et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 2002; 347: 322329.CrossRefGoogle ScholarPubMed
33.Hoeper, MM, Schwarze, M, Ehlerding, S, et al. Long-term treatment of primary pulmonary hypertension with aerosolized iloprost, a prostacyclin analogue. N Engl J Med 2000; 342: 18661870.CrossRefGoogle ScholarPubMed
34.Galie, N, Humbert, M, Vachiery, JL, et al. Effects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled trial. J Am Coll Cardiol 2002; 39: 14961502.CrossRefGoogle ScholarPubMed
35.Barst, RJ, McGoon, M, McLaughlin, V, et al. Beraprost therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2003; 41: 21192125.CrossRefGoogle ScholarPubMed
36.Clozel, M. Effects of bosentan on cellular processes involved in pulmonary arterial hypertension: do they explain the long-term benefit? Ann Med 2003; 35: 605613.CrossRefGoogle ScholarPubMed
37.Stewart, DJ, Levy, RD, Cernacek, P, Langleben, D. Increased plasma endothelin-1 in pulmonary hypertension: marker or mediator of disease? Ann Intern Med 1991; 114: 464469.CrossRefGoogle ScholarPubMed
38.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: 17321739.CrossRefGoogle ScholarPubMed
39.Vancheeswaran, R, Magoulas, T, Efrat, G, et al. Circulating endothelin-1 levels in systemic sclerosis subsets – a marker of fibrosis or vascular dysfunction? J Rheumatol 1994; 21: 18381844.Google ScholarPubMed
40.Jia, B, Zhang, S, Chen, Z, et al. Plasma endothelin 1 concentrations in children with congenital heart defects. Minerva Pediatr 1998; 50: 99103.Google ScholarPubMed
41.Allen, SW, Chatfield, BA, Koppenhafer, SA, Schaffer, MS, Wolfe, RR, Abman, SH. Circulating immunoreactive endothelin-1 in children with pulmonary hypertension. Association with acute hypoxic pulmonary vasoreactivity. Am Rev Respir Dis 1993; 148: 519522.CrossRefGoogle ScholarPubMed
42.Lutz, J, Gorenflo, M, Habighorst, M, Vogel, M, Lange, PE, Hocher, B. Endothelin-1- and endothelin-receptors in lung biopsies of patients with pulmonary hypertension due to congenital heart disease. Clin Chem Lab Med 1999; 37: 423428.CrossRefGoogle ScholarPubMed
43.Rubens, C, Ewert, R, Halank, M, et al. Big endothelin-1 and endothelin-1 plasma levels are correlated with the severity of primary pulmonary hypertension. Chest 2001; 120: 15621569.CrossRefGoogle ScholarPubMed
44.Galiè, N, Grigioni, F, Bacchi-Reggiani, L, et al. Relation of endothelin-1 to survival in patients with primary pulmonary hypertension. Eur J Clin Invest 1996; 26 (Supp 1): 273.Google Scholar
45.Channick, RN, Simonneau, G, Sitbon, O, et al. Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study. Lancet 2001; 358: 11191123.CrossRefGoogle ScholarPubMed
46.Rubin, LJ, Badesch, DB, Barst, RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002; 346: 896903.CrossRefGoogle ScholarPubMed
47.McLaughlin, VV, Sitbon, O, Badesch, DB, et al. Survival with first-line bosentan in patients with primary pulmonary hypertension. Eur Respir J 2005; 25: 244249.CrossRefGoogle ScholarPubMed
48.Galiè, N, Beghetti, M, Gatzoulis, MA, et al. Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 (BREATHE-5) Investigators. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation 2006; 114: 4854.CrossRefGoogle ScholarPubMed
49.Barst, RJ, Langleben, D, Frost, A, et al. Sitaxsentan therapy for pulmonary arterial hypertension. Am J Respir Crit Care Med 2004; 169: 441447.CrossRefGoogle ScholarPubMed
50.Barst, RJ, Langleben, D, Badesch, D, et al. STRIDE-2 Study Group. Treatment of pulmonary arterial hypertension with the selective endothelin-A receptor antagonist sitaxsentan. J Am Coll Cardiol 2006; 47: 20562059.CrossRefGoogle Scholar
51.Oudiz, R, Torres, F, Frost, A, et al. ARIES-1: A placebo-controlled efficacy and safety study of ambrisentan in patients with pulmonary arterial hypertension. Chest 2006; 130: 121S.CrossRefGoogle Scholar
52.Olschewski, H, Galie, N, Ghofrani, HA, et al. Ambrisentan improves exercise capacity and time to clinical worsening in patients with pulmonary arterial hypertension: Results of the ARIES-2 study. Proc Am Thorac Soc 2006; 3: A728.Google Scholar
53.Palmer, RM, Ferrige, AG, Moncada, S. Nitric oxide release accounts for the biological activity of endothelium-derived relaxing factor. Nature 1987; 327: 524526.CrossRefGoogle ScholarPubMed
54.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: 214221.CrossRefGoogle ScholarPubMed
55.Kuhn, KP, Wickersham, NE, Robbins, IM, Byrne, DW. Acute effects of sildenafil in patients with primary pulmonary hypertension receiving epoprostenol. Exp Lung Res 2004; 30: 135145.CrossRefGoogle ScholarPubMed
56.Galie, N, Ghofrani, HA, Torbicki, A, et al. Sildenafil Citrate Therapy for Pulmonary Arterial Hypertension. N Engl J Med 2005; 353: 21482157.CrossRefGoogle ScholarPubMed
57.Wilkens, H, Guth, A, Konig, J, et al. Effect of inhaled iloprost plus oral sildenafil in patients with primary pulmonary hypertension. Circulation 2001; 104: 12181222.CrossRefGoogle ScholarPubMed
58.Ghofrani, HA, Wiedemann, R, Rose, F, et al. Combination therapy with oral sildenafil and inhaled iloprost for severe pulmonary hypertension. Ann Intern Med 2002; 136: 515522.CrossRefGoogle ScholarPubMed
59.Kothari, SS, Duggal, B. Chronic oral sildenafil therapy in severe pulmonary artery hypertension. Indian Heart J 2002; 54: 404409.Google ScholarPubMed
60.Simonneau, G, Rubin, LJ, Galie, N, et al. PACES Study Group. Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial. Ann Intern Med 2008; 149: 521530.CrossRefGoogle Scholar
61.Nihill, MR, O’Laughlin, MP, Mullins, CE. Effects of atrial septostomy in patients with terminal cor pulmonale due to pulmonary vascular disease. Cathet Cardiovasc Diagn 1991; 24: 166172.CrossRefGoogle ScholarPubMed
62.Kerstein, D, Levy, PS, Hsu, DT, Hordof, AJ, Gersony, WM, Barst, RJ. Blade balloon atrial septostomy in patients with severe primary pulmonary hypertension. Circulation 1995; 91: 20282035.CrossRefGoogle ScholarPubMed
63.Sandoval, J, Gaspar, J, Pulido, T, et al. Graded balloon dilation atrial septostomy in severe primary pulmonary hypertension. A therapeutic alternative for patients nonresponsive to vasodilator treatment. J Am Coll Cardiol 1998; 32: 297304.CrossRefGoogle ScholarPubMed
64.Blanc, J, Vouhé, P, Bonnet, D. Potts shunt in patients with pulmonary hypertension. N Engl J Med 2004; 350: 623.CrossRefGoogle ScholarPubMed
65.Hopkins, WE, Ochoa, LL, Richardson, GW, Trulock, EP. Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome. J Heart Lung Transplant 1996; 15 (1 pt 1): 100105.Google ScholarPubMed