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Conventional and targeted medical therapies

Published online by Cambridge University Press:  01 May 2009

Robyn J. Barst  and
Duncan Stewart
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: [email protected]
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Abstract

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Keywords

Pulmonary arterial hypertensioncongenital heart diseasepulmonary vasodilatorsprostacyclinendothelin receptor antagonistsphosphodiesterase inhibitors
Type
Original Article
Information
Cardiology in the Young , Volume 19 , Issue S1: Statements on the Management of Pulmonary Hypertension Associated With Congenital Heart Disease , May 2009 , pp. 28 - 34
Copyright
Copyright © Cambridge University Press 2009

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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): S1–S7.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: 343–349.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: 1917–1928.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: 459–475.CrossRefGoogle ScholarPubMed
5.Recommendations on the management of pulmonary hypertension in clinical practice. Heart 2001; 86 (Suppl 1): I1–I13.Google Scholar
6.Humbert, M, Sitbon, O, Simonneau, G. Treatment of pulmonary arterial hypertension. N Engl J Med 2004; 351: 1425–1436.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: 385–390.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: 1682–1687.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: 787–792.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: 580–587.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: 76–81.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: 223–232.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: 518–523.CrossRefGoogle ScholarPubMed
15.Rosenzweig, EB, Widlitz, AC, Barst, RJ. Pulmonary arterial hypertension in children. Pediatr Pulmonol 2004; 38: 2–22.CrossRefGoogle ScholarPubMed
16.Gaine, S. Pulmonary hypertension. Jama 2000; 284: 3160–3168.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: 3105–3111.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: 70–75.CrossRefGoogle ScholarPubMed
19.Adatia, I, Haworth, SG. Circulating endothelin in children with congenital heart disease. Br Heart J 1993; 69: 233–236.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: 1925–1932.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: 296–302.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: 343–349.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: 273–277.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: 780–788.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: 425–434.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: 730–733.CrossRefGoogle ScholarPubMed
27.Rosenzweig, EB, Kerstein, D, Barst, RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999; 99: 1858–1865.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: 293–299.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: 1–5.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: 175–179.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: 151–155.CrossRefGoogle ScholarPubMed
32.Olschewski, H, Simonneau, G, Galie, N, et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 2002; 347: 322–329.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: 1866–1870.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: 1496–1502.CrossRefGoogle ScholarPubMed
35.Barst, RJ, McGoon, M, McLaughlin, V, et al. Beraprost therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2003; 41: 2119–2125.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: 605–613.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: 464–469.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: 1732–1739.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: 1838–1844.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: 99–103.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: 519–522.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: 423–428.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: 1562–1569.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: 1119–1123.CrossRefGoogle ScholarPubMed
46.Rubin, LJ, Badesch, DB, Barst, RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002; 346: 896–903.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: 244–249.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: 48–54.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: 441–447.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: 2056–2059.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: 524–526.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: 214–221.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: 135–145.CrossRefGoogle ScholarPubMed
56.Galie, N, Ghofrani, HA, Torbicki, A, et al. Sildenafil Citrate Therapy for Pulmonary Arterial Hypertension. N Engl J Med 2005; 353: 2148–2157.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: 1218–1222.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: 515–522.CrossRefGoogle ScholarPubMed
59.Kothari, SS, Duggal, B. Chronic oral sildenafil therapy in severe pulmonary artery hypertension. Indian Heart J 2002; 54: 404–409.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: 521–530.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: 166–172.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: 2028–2035.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: 297–304.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): 100–105.Google ScholarPubMed