Medical management of primary pulmonary hypertension

European Respiratory Journal

Volumn 9, Issue 11, 1996, Pages 2401-2409

  Kneussl, M P ^a   Lang, I M ^a   Brenot, F P ^b  

^a MEDICAL UNIVERSITY OF VIENNA   (Austria)

^b HÔPITAL ANTOINE BÉCLÈRE   (France)

Author keywords

Anticoagulation; Calcium channel blockers; Nitric oxide; Primary pulmonary hypertension; Prostacyclin; Standardized haemodynamic testing

Indexed keywords

ACETYLCHOLINE; ADENOSINE; NITRIC OXIDE; PROSTACYCLIN;

ANTICOAGULATION; HEART RIGHT VENTRICLE FAILURE; HEMODYNAMIC MONITORING; HUMAN; LUNG ARTERY PRESSURE; PRIORITY JOURNAL; PULMONARY HYPERTENSION; RESISTANCE BLOOD VESSEL; REVIEW; TREATMENT PLANNING; VASCULAR RESISTANCE; VASODILATATION;

ANTICOAGULANTS; ANTIHYPERTENSIVE AGENTS; EPOPROSTENOL; HEMODYNAMIC PROCESSES; HUMANS; HYPERTENSION, PULMONARY; VASODILATOR AGENTS;

EID: 0029910676     PISSN: 09031936     EISSN: None     Source Type: Journal    
DOI: 10.1183/09031936.96.09112401     Document Type: Review

References (104)

1. Anderson EG, Simon G, Reid L. Primary and thromboembolic pulmonary hypertension: a quantitative pathological study. J Pathol 1973; 110: 273-293.
2. Bjornsson J, Edwards WD. Primary pulmonary hypertension: a histopathologic study of 80 cases. Mayo Clin Proc 1985; 60: 16-25.
3. Rich S. Primary pulmonary hypertension. Prog Cardiovasc Dis 1988; 31: 205-238.
4. Rubin LJ. Primary pulmonary hypertension. Chest 1993; 104: 236-250.
5. Higenbottam T, Weitzenblum E. Pulmonary hypertension: mechanisms and treatment. Eur Respir J 1996; 8: 1991-1992.
6. Petitpretz P, Brenot F, Azarian R, et al. Pulmonary hypertension in patients with human immunodeficiency virus infection: comparison with primary pulmonary hypertension. Circulation 1994; 89: 2722-2727.
7. Weiss JR, Pietra GG, Scharf SM. Primary pulmonary hypertension and the human immunodeficiency virus: report of two cases and review of the literature. Arch Intern Med 1995; 155: 2350-2354.
8. Frank H, Gurtner HP, Kneussl M, Lang I, Mlczoch J. Aminorex-induzierte, plexogene pulmonale Arteriopathie: 25 Jahre danach. Z Kardiol 1993; 82: 568-572.
9. Brenot F, Herve P, Petitpretz P, Parent F, Duroux P, Simonneau G. Primary pulmonary hypertension and fenfluramine use. Br Heart J 1993; 70: 537-541.
10. Atanassoff PG, Weiss BM, Schmid ER, Tomic M. Pulmonary hypertension and dexfenfluramine. Lancet 1992; 339: 436. (letter).
11. Thomas SH, Butt AY, Corns PA, et al. Appetite suppressants and primary pulmonary hypertension in the United Kingdom. Br Heart J 1995; 74: 660-663.
12. Eltorky MA, Headley AS, Winer-Muram H, Garrett HEJ, Griffin JP. Pulmonary capillary hemangiomatosis: a clinicopathologic review. Ann Thorac Surg 1994; 57: 772-776.
13. Ruchelli ED, Nojadera G, Rutstein RM, Rudy B. Pulmonary veno-occlusive disease: another vascular disorder associated with human immunodeficiency virus infection? Arch Pathol Lab Med 1994; 118: 664-666.
14. Herve P, Drouet L, Dosquet C. Primary pulmonary hypertension in a patient with a familial platelet storage pool disease: role of serotonin. Am J Med 1990; 89: 117-120.
15. Lloyd JE, Butler MG, Foroud TM, Conneally PM, Phillips JA3, Newman JH. Genetic anticipation and abnormal gender ratio at birth in familial primary pulmonary hypertension. Am J Respir Crit Care Med 1995; 152: 93-97.
16. Lock JE. Hemodynamic evaluation of congenital heart disease. In: Lock JE, Keane JF, Fellows KE, eds. Diagnostic and Interventional Catheterization in Congenital Heart Disease. Boston, Martinus Nijhoff, 1987; pp. 33-62.
17. Bush A, Busst C, Knight WB. Modification of pulmonary hypertension secondary to congenital heart disease by prostacyclin therapy. Am Rev Respir Dis 1987; 136: 267-269.
18. Jolliet P, Thorens JB, Chevrolet JC. Pulmonary vascular reactivity in severe pulmonary hypertension associated with mixed connective tissue disease. Thorax 1995; 50: 96-97.
19. Kallenberg CG. Overlapping syndromes, undifferentiated connective tissue disease, and other fibrosing conditions. Curr Opin Rheumatol 1995; 7: 568-573.
20. Philips JB. Neonatal pulmonary hypertension. In: Philips JB, ed. Clinics in Perinatology. Philadelphia, W.B. Saunders, 1984; pp. 515-757.
21. Dinh-Xuan AT, Higenbottam TW, Clelland CA. Impairment of endothelium-dependent pulmonary-artery relaxation in chronic obstructive lung disease. N Engl J Med 1991; 324: 1539-1547.
22. Kessler R, Chaouat A, Weitzenblum E, et al. Pulmonary hypertension in the obstructive sleep apnea syndrome: prevalence, causes and therapeutic consequences. Eur Respir J 1996; 9: 787-794.
23. Rich S, Hart K. Familial pulmonary hypertension in association with an abnormal hemoglobin: insights into the pathogenesis of primary pulmonary hypertension. Chest 1991; 99: 1208-1210.
24. Robalino BD, Moodie DS. Association between primary pulmonary hypertension and portal hypertension: analysis of its pathophysiologic and clinical, laboratory and hemodynamic manifestations. J Am Coll Cardiol 1991; 17: 492-498.
25. Moser KM, Bloor CM. Pulmonary vascular lesions occurring in patients with chronic major vessel thromboembolic pulmonary hypertension. Chest 1993; 103: 685-492.
26. Weir EK. Acute vasodilator testing and pharmacological treatment of primary pulmonary hypertension. In: Fishman AP, ed. The Pulmonary Circulation: Normal and Abnormal. Mechanisms, Management and the National Registry. Philadelphia, University of Pennsylvania Press, 1990; pp. 485-499.
27. Weir EK, Rubin LJ, Ayres SM, et al. The acute administration of vasodilators in primary pulmonary hypertension (experience from the National Institute of Health registry on primary pulmonary hypertension). Am Rev Respir Dis 1989; 140: 1623-1630.
28. 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.
29. Simonneau G, Herve P, Petitpretz P, et al. Detection of a reversible component in primary pulmonary hypertension: value of prostacyclin acute infusion (Abstract). Am Rev Respir Dis 1986; 1933: A223.
30. Barst R, Rubin LJ, Long W, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med 1996; 334: 296-301.
31. Palevsky HI, Long W, Crow J, Fishman AP. Prostacyclin and acetylcholine as screening agents for acute pulmonary vasodilator responsiveness in primary pulmonary hypertension. Circulation 1990; 82: 2018-2026.
32. Schrader BJ, Inbar S, Kaufmann L, Vestal RE, Rich S. Comparison of the effects of adenosine and nifedipine in pulmonary hypertension. J Am Coll Cardiol 1992; 19: 1060-1064.
33. Samet P, Bernstein WH, Widrich J. Intracardiac infusion of acetylcholine in primary pulmonary hypertension. Am Heart J 1960; 60: 433-439.
34. 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.
35. Palmer RMJ, Ferrige AC, Moncada S. Nitric oxide release accounts for the biological activity of endothelium-derived relaxing factor. Nature 1987; 327: 524-526.
36. Frostell C, Fratacci MD, Wain JC, Jones R, Zapol WM. Inhaled nitric oxide: a selective pulmonary vasodilator reversing hypoxic pulmonary vasoconstriction. Circulation 1991; 83: 2038-2047.
37. Frostell CG, Blomqvist H, Hedenstierna G, Lundberg J, Zapol WM. Inhaled nitric oxide selectively reverses human hypoxic pulmonary vasoconstriction without causing systemic vasodilation. Anesthesiology 1993; 78: 427-435.
38. Roberts JD, Polaner DM, Lang P, Zapol WM. Inhaled nitric oxide in persistent pulmonary hypertension of the newborn. Lancet 1992; 340: 818-819.
39. Kinsella JP, Neish SR, Schaffer E, Abman SH. Low-dose inhalational nitric oxide in persistent pulmonary hypertension of the newborn. Lancet 1992; 340: 819-820.
40. Roberts JD, Lang P, Bigatello LM, Vlahakes GJ, Zapol WP. Inhaled nitric oxide in congenital heart disease. Circulation 1993; 87: 447-453.
41. Schranz D, Zepp F, Iversen S, et al. Effects of tolazoline and prostacyclin on pulmonary hypertension in infants after cardiac surgery. Crit Care Med 1992; 20: 1243-1249.
42. Girard C, Lehot JJ, Pannetier MC, Filley S, French P, Extanove S. Inhaled nitric oxide after mitral valve replacement in patients with chronic pulmonary artery hypertension. Anesthesiology 1992; 77: 880-883.
43. Goldman AP, Delius RE, Deanfield JE, Macrae DJ. Nitric oxide is superior to prostacyclin for pulmonary hypertension after cardic operations. Ann Thorac Surg 1995; 60: 300-306.
44. Rossaint R, Falke KJ, Lopez F, Slama K, Pison U, Zapol WM. Inhaled nitric oxide for the adult respiratory distress syndrome. N Engl J Med 1993; 328: 399-405.
45. Pepke-Zaba J, Higenbottam TW, Dinh-Xuan AT, Stone D, Wallwork J. Inhaled nitric oxide as a cause of selective pulmonary vasodilatation in pulmonary hypertension. Lancet 1991; 338: 1173-1174.
46. Nootens M, Schrader B, Kaufmann E, Vestal R, Long W, Rich S. Comparative acute effects of adenosine and prostacyclin in primary pulmonary hypertension. Chest 1995; 107: 54-57.
47. Rich S, Brundage BH. High-dose calcium channel-blocking therapy for primary pulmonary hypertension: evidence for long-term reduction in pulmonary arterial pressure and regression of right ventricular hypertrophy. Circulation 1987; 76: 135-141.
48. Rich S, Kaufmann K, 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.
49. Bush A, Busst CM, Knight WB, Shinebourne EA. Comparison of the haemodynamic effects of epoprostenol (prostacyclin) and tolazoline. Br Heart J 1988; 60: 141-148.
50. Grover RF, Reeves JT, Blount SG. Tolazoline hydrochloride (Priscoline): an effective pulmonary vasodilator. Am Heart J 1961; 61: 5-15.
51. Levine TB, Rose T, Kane M. Treatment of primary pulmonary hypertension by alpha-adrenergic blockade. Circulation 1980; 62 (Suppl. III): 111-126.
52. Ruskin JN, Hutter AM. Primary pulmonary hypertension treated with oral phentolamine. Ann Intern Med 1979; 90: 772-774.
53. Adnot S, Defouilloy C, Brun-Buisson C, Abrouk F, Piquet J, Lemaire F. Hemodynamic effects of urapidil in patients with pulmonary hypertension. Am Rev Respir Dis 1987; 135: 288-293.
54. Rubin LJ, Peter RH. Oral hydralazine therapy for primary pulmonary hypertension. N Engl J Med 1980; 302: 69-74.
55. Rich S, Ganz R, Levy PS. Comparative effects of hydralazine, nifedipine and amrinone in primary pulmonary hypertension. Am J Cardiol 1983; 52: 1104-1107.
56. Packer M, Greenberg B, Massie B, Dash H. Deleterious effects of hydralazine in patients with primary pulmonary hypertension. N Engl J Med 1982; 306: 1326-1331.
57. Lupi-Herrera E, Sandoval J, Seaane M, Bialostozky D. The role of hydralazine therapy for pulmonary artery hypertension of unknown cause. Circulation 1982; 65: 645-650.
58. Groves BM, Rubin LJ, Reeves JT, Frosolono MF. Comparable hemodynamic effects of prostacyclin and hydralazine in primary pulmonary hypertension. Am Heart J 1985; 110: 1200-1204.
59. Brent BN, Berger HJ, Matthay RA, Mahler D, Pytlik L, Zaret BL. Contrasting acute effects of vasodilators (nitroglycerin, nitroprusside and hydralazine) on right ventricular performance in patients with chronic obstructive pulmonary disease and pulmonary hypertension: a combined radionuclide-hemodynamic study. Am J Cardiol 1983; 51: 1682-1689.
60. Buch J, Wennevold A. Hazards of diazoxide in pulmonary hypertension. Br Heart J 1981; 46: 401-403.
61. Wang SWS, Pohl JEF, Rowland DJ, Wade EG. Diazoxide in treatment of primary pulmonary hypertension. Br Heart J 1978; 40: 572-574.
62. Honey M, Cotter L, Davies N, Denison D. Clinical and haemodynamic effects of diazoxide in primary pulmonary hypertension. Thorax 1980; 35: 269-276.
63. Hall DR. Remission of primary pulmonary hypertension during treatment with diazoxide. Br Med J Clin Res Ed 1981; 282: 1118.
64. Chan NS, McLay J, Kenmore ACF. Reversibility of primary pulmonary hypertension during six years of treatment with oral diazoxide. Br Heart J 1987; 57: 207-209.
65. Fuleihan DS, Mookherjee S, Potts JL, Obeid AI, Warner RA, Eich RH. Sodium nitroprusside: a new role as a pulmonary vasodilator. Am J Cardiol 1979; 43: 405.
66. Ikram H, Maslowski AH, Nicholls MG, Espiner EA, Hull FT. Haemodynamic and hormonal effects of captopril in primary pulmonary hypertension. Br Heart J 1982; 48: 541-545.
67. Rich S, Martinez J, Lam W, Rosen KM. Captopril as treatment for patients with pulmonary hypertension: problem of variability in assessing chronic drug treatment. Br Heart J 1982; 48: 272-277.
68. Leier CV, Bambach D, Selson S, et al. Captopril in primary pulmonary hypertension. Circulation 1983; 67: 155-161.
69. Wood BA, Tortoledo F, Luck JC, Fennell WH. Rapid attenuation of responses to nifedipine in primary pulmonary hypertension. Chest 1982; 82: 793-794.
70. Saito D, Haraoka S, Yoshida H, et al. Primary pulmonary hypertension improved by long-term oral administration of nifedipine. Am Heart J 1983; 105: 1041-1042.
71. Rubin LJ, Nicod P, Hillis LD, Firth BG. Treatment of primary pulmonary hypertension with nifedipine: a hemodynamic and scintigraphic evaluation. Ann Intern Med 1983; 99: 433-438.
72. Rozkovec A, Stradling J, Shepherd G, MacDermot J, Oakley C, Dollery CT. Prediction of favourable responses to long-term vasodilation treatment of pulmonary hypertension by short-term administration of epoprostenol (prostacyclin) or nifedipine. Br Heart J 1988; 59: 696-705.
73. Olivari MT, Levine TB, Weir EK, Cohn JN. Hemodynamic effects of nifedipine at rest and during exercise in primary pulmonary hypertension. Chest 1984; 86: 14-19.
74. Lunde P, Rasmussen K. Long-term beneficial effect of nifedipine in primary pulmonary hypertension. Am Heart J 1984; 108: 415-416.
75. Kennedy T, Summer W. Inhibition of hypoxic pulmonary vasoconstriction by nifedipine. Am J Cardiol 1982; 50: 864-868.
76. Fisher J, Borer JS, Moses JW, et al. Hemodynamic effects of nifedipine versus hydralazine in primary pulmonary hypertension. Am J Cardiol 1984; 54: 646-650.
77. Farber HW, Karlinsky JB, Faling LJ. Fatal outcome following nifedipine for primary pulmonary hypertension. Chest 1983; 23: 708-709.
78. DeFeyter PJ, Kerkkamp HJJ, de Jong JP. Sustained beneficial effect of nifedipine in primary pulmonary hypertension. Am Heart J 1983; 105: 333-334.
79. Young TE, Lundquist LJ, Chesler E, Weir EK. Comparative effects of nifedipine, verapamil and diltiazem on experimental pulmonary hypertension. Am J Cardiol 1983; 51: 195-200.
80. Kambara H, Fujimoto K, Wakabayashi A, Kawai C. Primary pulmonary hypertension: beneficial therapy with diltiazem. Am Heart J 1981; 101: 230-232.
81. Packer M, Medina N, Yushak M, Wiener I. Detrimental effects of verapamil in patients with primary pulmonary hypertension. Br Heart J 1984; 52: 106-111.
82. Landmark K, Refsum AM, Simonsen S, Storstein O. Verapamil and pulmonary hypertension. Acta Med Scand 1978; 204: 299-302.
83. 2. J Appl Physiol 1996; 80: 191-197.
84. Barst RJ, Rubin LJ, McGoon MD, Caldwell EJ, Long WA, Levy S. Survival in primary pulmonary hypertension with long-term continuous intravenous prostacyclin. Ann Intern Med 1994; 121: 409-415.
85. Boyer-Neumann C, Brenot F, Wolf M, et al. Continuous infusion of prostacyclin decreases plasma levels of t-PA and PAI-1 in primary pulmonary hypertension. Thromb Haemost 1995; 73: 735-736.
86. Brenot F, Sitbon O, Parent F, et al. Influence of long-term continuous infusion of prostacyclin on survival in patients with primary pulmonary hypertension: the French experience. Am J Respir Crit Care Med 1995; 151: A723.
87. Higenbottam TW, Wheeldon D, Wells FC, Wallwork J. Long-term treatment of primary pulmonary hypertension with continuous intravenous epoprostenol (prostacyclin). Lancet 1984; i: 1046-1047.
88. Higenbottam TW, Spiegelhalter D, Scott JP, et al. Prostacyclin (epoprostenol) and heart-lung transplantation as treatments for severe pulmonary hypertension. Br Heart J 1993; 70: 366-370.
89. Jones DK, Higenbottam TW, Wallwork J. Treatment of primary pulmonary hypertension with intravenous epoprostenol (prostacyclin). Br Heart J 1987; 57: 270-278.
90. 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.
91. 1) on pulmonary hypertension and lung vascular remodeling in a rat monocrotaline model of human pulmonary hypertension. Nippon Kyobu Shikkan Gakkai Zasshi 1995; 33: 862-867.
92. Wasler A, Iberer F, Tscheliessnigg KH, Auer T, Petutschnigg B. Prostaglandin E, in the pretransplantation period in patients with pulmonary hypertension. J Heart Lung Transplant 1993; 12: 884. (letter).
93. Olschewski H, Walmrath D, Ghofrani HA, Schermuly R, Grimminger F, Seeger W. Inhaled prostacyclin and iloprost for treatment of severe primary pulmonary hypertension. Am J Respir Crit Care Med 1996; 153: A86.
94. Fuster V, Steele PM, Edwarts ED, Gersh BJ, McGoon MD, Frye RL. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation 1984; 70: 580-587.
95. Moser KM, Fedullo PF, Finkbeiner WE, Golden J. Do patients with primary pulmonary hypertension develop extensive central thrombi? Circulation 1995; 91: 741-745.
96. Kiss H, Egarter C, Asseryanis E, Putz D, Kneussl M. Primary pulmonary hypertension in pregnancy: a case report. Am J Obstet Gynecol 1995; 172: 1052-1054.
97. Rubin LJ, Groves BM, Reeves JT, Frosolono M, Handel F, Cato AE. Prostacyclin-induced acute pulmonary vasodilation in primary pulmonary hypertension. Circulation 1982; 66: 334-338.
98. Raffy O, Azarian F, Brenot F, et al. Clinical significance of the pulmonary vasodilator response during short-term infusion of prostacyclin in primary pulmonary hypertension. Circulation 1996; 93: 484-188.
99. 2) on survival in moderate and severe pulmonary hypertension. Am J Respir Crit Care Med 1994; 149: A748.
100. Christman BW, McPherson CD, Newmann JH, et al. An imbalance between the excretion of thromboxane and prostacyclin metabolites in pulmonary hypertension. N Engl J Med 1992; 327: 70-75.
101. Wagenvoort CA, Wagenvoort N. Primary pulmonary hypertension: a pathologic study of the lung vessels in 156 clinically diagnosed cases. Circulation 1970; 42: 1163-1184.
102. Goldman AP, Rees PC, Macrae DJ. Is it time to consider domiciliary nitric oxide? Lancet 1995; 345: 199-200.
103. Channick RN, Williams PJ, Newhart JW, Johnson FW, McIntyre D. Chronic inhaled nitric oxide in the out-patient treatment of primary pulmonary hypertension. Am J Respir Crit Care Med 1996; 153: A86.
104. Adnot S, Raffestin B. Pulmonary hypertension: NO therapy? Thorax 1996; 51: 762-764.