Progress in Cardiovascular Diseases
Volume 45, Issue 2 , Pages 115-128 , September 2002

Primary pulmonary hypertension: Current therapy

References 

  1. Rich S, Dantzker DR, Ayres SM, et al.  Primary pulmonary hypertension: A national prospective study. Ann Intern Med. 1987;107:216–223
  2. D'Alonzo GE, Barst RJ, Ayres SM, et al.  Survival in patients with primary pulmonary hypertension. Results from a national prospective study. Ann Intern Med. 1991;115:343–349
  3. Rubin LJ. Primary pulmonary hypertension. N Engl J Med. 1997;336:111–117
  4. Rich S. ed: Primary pulmonary hypertension: Executive Summary from the World Symposium—Primary Pulmonary Hypertension. Available from the World Health Organization via the Internet http://www.who.int/ncd/cvd/pph.html1998;
  5. Gaine SP, Rubin LJ. Primary pulmonary hypertension. Lancet. 1998;352:719–725
  6. Naeije R, Vachiery JL. Medical therapy of pulmonary hypertension. Conventional therapies. Clin Chest Med. 2001;22:517–527
  7. Kleiger RE, Boxer M, Ingham RE, et al.  Pulmonary hypertension in patients using oral contraceptives. Chest. 1976;69:143–147
  8. Bjornsson J, Edwards WD. Primary pulmonary hypertension: A histopathological study of 80 cases. Mayo Clin Proc. 1985;60:16–25
  9. Palevsky HI, Schloo BL, Pietra GG, et al.  Primary pulmonary hypertension. Vascular structure, morphometry and responsiveness to vasodilators agents. Circulation. 1989;80:1207–1221
  10. Fuster V, Steele PM, Edwards WD, et al.  Primary pulmonary hypertension: Natural history and the importance of thrombosis. Circulation. 1984;70:580–587
  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
  12. Khamashta MA, Cuadrado MJ, Mujic F, et al.  The management of thrombosis in the antiphospholipid-antibody syndrome. N Engl J Med. 1995;332:993–997
  13. Brenot F. Primary pulmonary hypertension: Case series from France. Chest. 1994;105:33S–36S
  14. Dantzker DR, Bower JS. Mechanisms of gas exchange abnormality in patients with chronic obliterative pulmonary vascular disease. J Clin Invest. 1979;64:1050
  15. 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
  16. Fahey PJ, Utell MJ, Condemi JJ, et al.  Raynaud's phenomenon of the lung. Am J Med. 1984;76:263–269
  17. Sitbon O, Brenot F, Denjean A, et al.  Inhaled nitric oxide as a screening vasodilator agent in primary pulmonary hypertension. A dose-response study and comparison with prostacyclin. Am J Respir Crit Care Med. 1995;151:384–389
  18. Sitbon O, Humbert M, Jagot JL, 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;12:265–270
  19. Reeves JT, Groves BM, Turkevich D. The case for treatment of selected patients with primary pulmonary hypertension. Am Rev Respir Dis. 1986;134:342–346
  20. Barst RJ, Maislin G, Fishman AP. Vasodilator therapy for primary pulmonary hypertension in children. Circulation. 1999;99:1197–1208
  21. Raffy O, Azarian R, Brenot F, et al.  Clinical significance of the pulmonary vasodilator response during short-term infusion of prostacyclin in primary pulmonary hypertension. Circulation. 1996;93:484–488
  22. Ricciardi MJ, Knight BP, Martinez FJ, et al.  Inhaled nitric oxide in primary pulmonary hypertension: A safe and effective agent for predicting response to nifedipine. J Am Coll Cardiol. 1998;32:1068–1073
  23. Rubin LJ, Groves BM, Reeves JT, et al.  Prostacyclin-induced acute pulmonary vasodilation in primary pulmonary hypertension. Circulation. 1982;66:334–338
  24. Simonneau G, Hervé P, Petitpretz P, et al.  Detection of a reversible component in primary pulmonary hypertension: Value of prostacyclin acute infusion. Am Rev Respir Dis. 1986;133:A223
  25. Morgan JM, McCormack DG, Griffiths MJD, et al.  Adenosine as a vasodilator in primary pulmonary hypertension. Circulation. 1991;84:1145–1149
  26. Schrader BJ, Inbar S, Kaufmann L, et al.  Comparison of the effects of adenosine and nifedipine in pulmonary hypertension. J Am Coll Cardiol. 1992;19:1060–1064
  27. Nootens M, Schrader B, Kaufmann E, et al.  Comparative acute effects of adenosine and prostacyclin in primary pulmonary hypertension. Chest. 1995;107:54–57
  28. Pepke-Zaba J, Higenbottam TW, Dinh-Xuan AT, et al.  Inhaled nitric oxide as a cause of selective pulmonary vasodilatation in pulmonary hypertension. Lancet. 1991;338:1173–1174
  29. Jolliet P, Bulpa P, Thorens JB, et al.  Nitric oxide and prostacyclin as test agents of vasoreactivity in severe precapillary pulmonary hypertension: Predictive-ability and consequences on haemodynamics and gas exchange. Thorax. 1997;52:369–372
  30. Cockrill BA, Kacmarek RM, Fifer MA, et al.  Comparison of the effects of nitric oxide, nitroprusside, and nifedipine on hemodynamics and right ventricular contractility in patients with chronic pulmonary hypertension. Chest. 2001;119:128–136
  31. Hoeper MM, Olschewski H, Ghofrani HA, et al.  A comparison of the acute hemodynamic effects of inhaled nitric oxide and aerosolized iloprost in primary pulmonary hypertension. German PPH study group. J Am Coll Cardiol. 2000;35:176–182
  32. Palevsky HI, Fishman AP. Comparison of acute hemodynamic responses to prostacyclin with standard vasodilators in patients with primary pulmonary hypertension. Chest. 1988;93:179S
  33. Palevsky HI, Long W, Crow J, et al.  Prostacyclin and acetylcholine as screening agents for acute pulmonary vasodilator responsiveness in primary pulmonary hypertension. Circulation. 1990;82:2018–2026
  34. 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
  35. Davidson D, Barefield ES, Kattwinkel J, et al.  Inhaled nitric oxide for the early treatment of persistent pulmonary hypertension of the term newborn: A randomized, double-masked, placebo-controlled, dose-response, multicenter study. The I-NO/PPHN Study Group. Pediatrics. 1998;101:325–334
  36. Roberts JD, Lang P, Bigatello LM, et al.  Inhaled nitric oxide in congenital heart disease. Circulation. 1993;87:447–453
  37. Girard C, Lehot J-J, Pannetier J-C, et al.  Inhaled nitric oxide after mitral valve replacement in patients with chronic pulmonary artery hypertension. Anesthesiology. 1992;77:880–883
  38. Rossaint R, Falke KJ, Lopez F, et al.  Inhaled nitric oxide for the adult respiratory distress syndrome. N Engl J Med. 1993;328:399–405
  39. Adnot S, Kouyoumdjian C, Defouilloy C, et al.  Hemodynamic and gas exchange responses to infusion of acetylcholine and inhalation of nitric oxide in patients with chronic obstructive lung disease and pulmonary hypertension. Am Rev Respir Dis. 1993;148:310–316
  40. Rubin LJ, Peter RH. Oral hydralazine therapy for primary pulmonary hypertension. N Engl J Med. 1980;302:69–73
  41. Packer M. Vasodilator therapy for primary pulmonary hypertension. Ann Intern Med. 1985;103:258–270
  42. Hughes JD, Rubin LJ. Primary pulmonary hypertension. An analysis of 28 cases and a review of the literature. Medicine (Baltimore). 1986;65:56–72
  43. Packer M, Greenberg B, Massie B, et al.  Deleterious effects of hydralazine in patients with pulmonary hypertension. N Engl J Med. 1982;306:1326–1331
  44. McGoon MD, Vlietstra RE. Vasodilator therapy for primary pulmonary hypertension. Mayo Clin Proc. 1984;59:672–677
  45. Packer M. Therapeutic application of calcium-channel antagonists for pulmonary hypertension. Am J Cardiol. 1985;55:196B–201B
  46. Packer M, Medina N, Yushak M, et al.  Detrimental effects of verapamil in patients with primary pulmonary hypertension. Br Heart J. 1984;52:106–111
  47. Woodmansey PA, O'Toole L, Channer KS, et al.  Acute pulmonary vasodilatory properties of amlodipine in humans with pulmonary hypertension. Heart. 1996;75:171–173
  48. Gaine S. Pulmonary hypertension. JAMA. 2000;284:3160–3168
  49. Jones K. Prostacyclin. In:  Peacock AJ editors. Pulmonary Circulation. A Handbook for Clinicians. London: Chapman & Hall; 1996;p. 115–122
  50. a. 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;327:912–918
  51. 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
  52. Higenbottam T, Wheeldon D, Wells F, et al.  Long-term treatment of primary pulmonary hypertension with continuous intravenous epoprostenol (prostacyclin). Lancet. 1984;1:1046–1047
  53. Rubin LJ, Mendoza J, Hood M, et al.  Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trial. Ann Intern Med. 1990;112:485–491
  54. Barst RJ, Rubin LJ, McGoon MD, et al.  Survival in primary pulmonary hypertension with long-term continuous intravenous prostacyclin. Ann Intern Med. 1994;121:409–415
  55. 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
  56. 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
  57. Higenbottam T, Butt AY, McMahon A, et al.  Long-term intravenous prostaglandin (epoprostenol or iloprost) for treatment of severe pulmonary hypertension. Heart. 1998;80:151–155
  58. McLaughlin VV, Genthner DE, Panella MM, et al.  Reduction in pulmonary vascular resistance with long-term epoprostenol (prostacyclin) therapy in primary pulmonary hypertension. N Engl J Med. 1998;338:273–277
  59. McLaughlin V, Genthner D, Panella M, et al.  Reduction in pulmonary vascular resistance with long-term epoprostenol (prostacyclin) therapy in primary pulmonary hypertension. N Engl J Med. 1998;338:273–277
  60. 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
  61. Robbins IM, Christman BW, Newman JH, et al.  A survey of diagnostic practices and the use of epoprostenol in patients with primary pulmonary hypertension. Chest. 1998;114:1269–1275
  62. Conte JV, Gaine SP, Orens JB, et al.  The influence of continuous intravenous prostacyclin therapy for primary pulmonary hypertension on the timing and outcome of transplantation. J Heart Lung Transplant. 1998;17:679–685
  63. Rich S, McLaughlin VV. The effects of chronic prostacyclin therapy on cardiac output and symptoms in primary pulmonary hypertension. J Am Coll Cardiol. 1999;34:1184–1187
  64. Humbert M, Maitre S, Capron F, et al.  Pulmonary edema complicating continuous intravenous prostacyclin in pulmonary capillary hemangiomatosis. Am J Respir Crit Care Med. 1998;157:1681–1685
  65. Resten A, Maitre S, Humbert M, et al.  Pulmonary arterial hypertension: Thin-section CT predictors of epoprostenol therapy failure. Radiology. 2002;222:782–788
  66. 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
  67. Humbert M, Sanchez O, Fartoukh M, et al.  Short-term and long-term epoprostenol (prostacyclin) therapy in pulmonary hypertension secondary to connective tissue diseases: Results of a pilot study. Eur Respir J. 1999;13:1351–1356
  68. Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation. 1999;99:1858–1865
  69. McLaughlin VV, Genthner DE, Panella MM, et al.  Compassionate use of continuous prostacyclin in the management of secondary pulmonary hypertension: A case series. Ann Intern Med. 1999;130:740–743
  70. Kuo PC, Johnson LB, Plotkin JS, et al.  Continuous intravenous infusion of epoprostenol for the treatment of portopulmonary hypertension. Transplantation. 1997;63:604–606
  71. Aguilar RV, Farber HW. Epoprostenol (prostacyclin) therapy in HIV-associated pulmonary hypertension. Am J Respir Crit Care Med. 2000;162:1846–1850
  72. Nunes H, Humbert M, Sitbon O, et al.  Declining mortality from HIV-associated pulmonary arterial hypertension with combined use of highly active antiretroviral therapy and long-term epoprostenol infusion. Am J Respir Crit Care Med. 2002;165:A412
  73. Jones D, Benjamin C, Linseman D. Activation of thromboxane and prostacyclin receptors elicits opposing effects on vascular smooth muscle cell growth and mitogen-activated protein kinase signaling cascades. Mol Pharmacol. 1995;48:890–896
  74. Hoeper M, Voelkel N, Bates T, et al.  Prostaglandins induce vascular endothelial growth factor in a human monocytic cell line and in rat lung via cAMP. Am J Respir Cell Mol Biol. 1997;17:748–756
  75. Castelain V, Chemla D, Humbert M, et al.  Improvement of the pressure flow relationships of the pulmonary circulation in primary pulmonary hypertension after six weeks prostacyclin. Am J Respir Crit Care Med. 2002;165:338–340
  76. Austen WG, Morrow AG, Berry WB. Experimental studies of the surgical treatment of primary pulmonary hypertension. J Thorac Cardiovasc Surg. 1964;48:448–455
  77. Rozkovec A, Montanes P, Oakley CM. Factors that influence the outcome of primary pulmonary hypertension. Br Heart J. 1986;55:449–458
  78. Glanville AR, Burke CM, Theodore J, et al.  Primary pulmonary hypertension. Length of survival in patients referred for heart-lung transplantation. Chest. 1987;91:675–681
  79. Rich S, Lam W. Atrial septostomy as palliative therapy for refractory primary pulmonary hypertension. Am J Cardiol. 1983;51:1560–1561
  80. Kerstein D, Levy PS, Hsu DT, et al.  Blade balloon atrial septostomy in patients with severe primary pulmonary hypertension. Circulation. 1995;91:2028–2035
  81. Sandoval J, Rothman A, Pulido T. Atrial septostomy for pulmonary hypertension. Clin Chest Med. 2001;22:547–560
  82. Reitz BA, Wallwork JL, Hunt SA, et al.  Heart-lung transplantation: Successful therapy for patients with pulmonary vascular disease. N Engl J Med. 1982;306:557–564
  83. Levine SM, Gibbons WJ, Bryan CL, et al.  Single lung transplantation for primary pulmonary hypertension. Chest. 1990;98:1107–1115
  84. Hosenpud JD, Bennett LE, Keck BM, et al.  The Registry of the International Society for Heart and Lung Transplantation: 18th Official Report—2001. J Heart Lung Transplant. 2001;20:805–815
  85. Trulock EP. Lung transplantation for primary pulmonary hypertension. Clin Chest Med. 2001;22:583–593

☆ Address reprint requests to Olivier Sitbon, MD, Service de Pneumologie et Réanimation, Hôpital Antoine Béclère, 157 rue de la Porte de Trivaux, 92140 Clamart, France.

PII: S0033-0620(02)70002-9

doi: 10.1053/pcad.2002.128449

Progress in Cardiovascular Diseases
Volume 45, Issue 2 , Pages 115-128 , September 2002

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