Guidelines on diagnosis and treatment of pulmonary arterial hypertension;Guías de Práctica Clínica sobre el diagnóstico y tratamiento de la hipertensión arterial pulmonar

Revista Espanola de Cardiologia

Volumn 58, Issue 5, 2005, Pages 523-566

  Galiè, Nazzareno ^a   Torbicki, Adam ^b   Barst, Robyn ^b   Dartevelle, Philippe ^b   Haworth, Sheila ^b   Higenbottam, Tim ^b   Olschewski, Horst ^b   Peacock, Andrew ^b   Pietra, Giuseppe ^b   Rubin, Lewis J ^b   Simonneau, Gerald ^b   Priori, Silvia G ^b   Alonso García, María Ángeles ^b   Blanc, Jean Jacques ^b   Budaj, Andrzej ^b   Cowie, Martin ^b   Dean, Veronica ^b   Deckers, Jaap ^b   Fernández Burgos, Enrique ^b   Lekakis, John ^b   Lindahl, Bertil ^b   Mazzotta, Gianfranco ^b   McGregor, Keith ^b   Morais, João ^b   Oto, Ali ^b   Smiseth, Otto A ^b   Albert Barbera, Joan ^b   Gibbs, Simon ^b   Hoeper, Marius ^b   Humbert, Marc ^b   Naeije, Robert ^b   Pepke Zaba, Joanna ^b   more..

^a UNIVERSITY OF BOLOGNA   (Italy)

^b NONE

Author keywords

[No Author keywords available]

Indexed keywords

AMBRISENTAN; ANTICOAGULANT AGENT; ANTIRETROVIRUS AGENT; BERAPROST; BOSENTAN; DIGOXIN; DIURETIC AGENT; DOBUTAMINE; ILOPROST; NIFEDIPINE; OXYGEN; PLACEBO; PROSTACYCLIN; PROSTANOID; SILDENAFIL; SITAXSENTAN; UNIPROST; WARFARIN;

CLINICAL TRIAL; COMPUTER ASSISTED TOMOGRAPHY; DIAGNOSTIC PROCEDURE; DISEASE ASSOCIATION; DISEASE CLASSIFICATION; DISEASE SEVERITY; DRUG POTENTIATION; EISENMENGER COMPLEX; ELECTROCARDIOGRAM; EXERCISE TOLERANCE; HEART HEMODYNAMICS; HEMANGIOMATOSIS; HEMOGLOBIN DETERMINATION; HORMONE SUBSTITUTION; HUMAN; HUMAN IMMUNODEFICIENCY VIRUS INFECTION; HYPOTENSION; LUNG BIOPSY; LUNG PERFUSION; LUNG TRANSPLANTATION; LUNG VEIN OBSTRUCTION; NAUSEA; NUCLEAR MAGNETIC RESONANCE IMAGING; POSTMENOPAUSE; PRACTICE GUIDELINE; PULMONARY HYPERTENSION; REVIEW; RISK FACTOR; SCLERODERMA; THORAX RADIOGRAPHY; TRANSTHORACIC ECHOCARDIOGRAPHY;

EID: 21244461110     PISSN: 03008932     EISSN: None     Source Type: Journal    
DOI: 10.1157/13074846     Document Type: Article

References (230)

1. Simonneau G, Galie N, Rubin L, et al. Clinical classification of pulmonary arterial hypertension. J Am Coll Cardiol. 2004;43: S5-12.
2. D'Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med. 1991;115:343-9.
3. Rubin LJ. Primary pulmonary hypertension. N Engl J Med. 1997;336:111-7.
4. Galie N, Manes A, Uguccioni L, et al. Primary pulmonary hypertension: insights into pathogenesis from epidemiology. Chest. 1998;114 3 Suppl:S184-94.
5. Pietra GG, Edwards WD, Kay JM, et al. Histopathology of primary pulmonary hypertension. A qualitative and quantitative study of pulmonary blood vessels from 58 patients in the National Heart, Lung, and Blood Institute, Primary Pulmonary Hypertension Registry [see comments]. Circulation. 1989;80:1198-206.
6. Pietra GG, Capron F, Stewart S, et al. Pathologic assessment of vasculopathies in pulmonary hypertension. J Am Coll Cardiol. 2004; Suppl S:S25-32.
7. Humbert M, Morrell N, Archer S, et al. Cellular and molecular pathobiology of pulmonary arterial hypertension. J Am Coll Cardiol. 2004;43:S13-24.
8. Lane KB, Machado RD, Pauciulo MW, et al. Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension. The International PPH Consortium. Nat Genet. 2000;26:81-4.
9. Deng Z, Morse JH, Slager SL, et al. Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genetr. 2000;67:737-44.
10. Budhiraja R, Tuder RM, Hassoun PM. Endothelial dysfunction in pulmonary hypertension. Circulation. 2004;109:159-65.
11. Barst R, McGoon M, Torbicki A, et al. Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2004;43:S40-7.
12. Hoeper M, Oudiz R, Peacock A, et al. End-points and clinical trial designs in pulmonary arterial hypertension: clinical and regulatory perspectives. J Am Coll Cardiol. 2004;43:S48-55.
13. Galie N, Manes A, Branzi A. Prostanoids for pulmonary arterial hypertension. Am J Respir Med. 2003;2:123-37.
14. Galie N, Manes A, Branzi A. The endothelin system in pulmonary arterial hypertension. Cardiovasc Res. 2004;61:227-37.
15. Sastry BKS, Narasimhan C, Reddy NK, et al. Clinical efficacy of sildenafil in primary pulmonary hypertension 1: a randomized, placebo-controlled, double-blind, crossover study. J Am Coll Cardiol. 2004;43:1149-53.
16. Committee for Practice Guidelines (CPG) of the European Society of Cardiology. Recommendations for Task Force Creation and Report Production A document for Task Force members and expert panels responsible for the creation and production of Guidelines and Expert Consensus Documents Committee for Practice Guidelines (CPG) of the European Society of Cardiology; 2003. Disponible en: http://www.escardio.org
17. Hatano S, Strasser T. World Health Organization 1975 primary pulmonary hypertension. Geneva: WHO; 1975.
18. Fishman AP. Primary pulmonary arterial hypertension: a look back. J Am Coll Cardiol. 2004;43:S2-4.
19. Humbert M, Nunes H, Sitbon O, et al. Risk factors for pulmonary arterial hypertension. Clin Chest Med. 2001;22:459-75.
20. Hoeper MM, Niedermeyer J, Hoffmeyer F, et al. Pulmonary hypertension after splenectomy? Ann Intern Med. 1999;130:506-9.
21. Castro O, Hoque M, Brown BD. Pulmonary hypertension in sickle cell disease: cardiac catheterization results and survival. Blood. 2003;101:1257-61.
22. Atichartakarn V, Likittanasombat K, Chuncharunee S, et al. Pulmonary arterial hypertension in previously splenectomized patients with beta-thalassemic disorders. Int J Hematol. 2003;78: 139-45.
23. Dingli D, Utz JP, Krowka MJ, et al. Unexplained pulmonary hypertension in chronic myeloproliferative disorders. Chest. 2001; 120:801-8.
24. Humbert M, Labrune P, Simonneau G, et al. Pulmonary arterial hypertension and type I glycogen storage disease: the serotonin hypothesis. Eur Respir J. 2002;20:59-65.
25. Dawson A, Elias DJ, Rubenson D, et al. Pulmonary hypertension developing after alglucerase therapy in two patients with type 1 Gaucher disease complicated by the hepatopulmonary syndrome. Ann Intern Med. 1996;125:901-4.
26. Trembath RC, Thomson JR, Machado RD, et al. Clinical and molecular genetic features of pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia. N Engl J Med. 2001;345:325-34.
27. Daliento L, Somerville J, Presbitero P, et al. Eisenmenger syndrome. Factors relating to deterioration and death. Eur Heart J. 1998;19:1845-55.
28. Besterman E. Atrial septal defects with pulmonary hypertension. Br Heart J. 1961;23:587-98.
29. Hoffman JE, Rudolph AM. The natural history of ventricular septal defects in infancy. Am J Cardiol. 1985;16:634-53.
30. Steele P, Fuster V, Cohen M, et al. Isolated atrial septal defect with pulmonary vascular obstructive disease: long-term follow-up and prediction of outcome after surgical correction. Circulation. 1987;76:1037-42.
31. Vogel M, Berger F, Kramer A, et al. Incidence of secondary pulmonary hypertension in adults with atrial septal or sinus venosus defects. Heart. 1999;82:30-3.
32. Kidd L, Driscoll D, Gersony W et al. Second natural history study of congenital heart defects. Results of treatment of patients with ventricular septal defects. Circulation. 1993;87:138-51.
33. Neumayer U, Stone S, Somerville J. Small ventricular septal defects in adults. Eur Heart J. 1988;19:1573-82.
34. Wood P. Primary pulmonary hypertension, with special reference to the vasoconstrictive factor. Br Heart J. 1958;20:557-65.
35. Yuan JX, Aldinger AM, Juhaszova M, et al. Dysfunctional voltagegated K+ channels in pulmonary artery smooth muscle cells of patients with primary pulmonary hypertension. Circulation. 1998;98:1400-6.
36. Petkov V, Mosgoeller W, Ziesche R, et al. Vasoactive intestinal peptide as a new drug for treatment of primary pulmonary hypertension. J Clin Invest. 2003;111:1339-46.
37. Cowan KN, Heilbut A, Humpl T, et al. Complete reversal of fatal pulmonary hypertension in rats by a serine elastase inhibitor. Nat Med. 2000;6:698-702.
38. Du L, Sullivan CC, Chu D, et al. Signaling molecules in nonfamilial pulmonary hypertension. N Engl J Med. 2003;348:500-9.
39. Dorfmuller P, Perros F, Balabanian K, et al. Inflammation in pulmonary arterial hypertension. Eur Respir J. 2003;22:358-63.
40. Eddahibi S, Humbert M, Fadel E, et al. Serotonin transporter overexpression is responsible for pulmonary artery smooth muscle hyperplasia in primary pulmonary hypertension. J Clin Invest. 2001;108:1141-50.
41. Friedman R, Mears JG, Barst RJ. Continuous infusion of prostacyclin normalizes plasma markers of endothelial cell injury and platelet aggregation in primary pulmonary hypertension. Circulation. 1997;96:2782-4.
42. Eisenberg PR, Lucore C, Kaufman L, et al. Fibrinopeptide A levels indicative of pulmonary vascular thrombosis in patients with primary pulmonary hypertension. Circulation. 1990;82: 841-7.
43. Christman BW, McPherson CD, Newman JH, et al. An imbalance between the excretion of thromboxane and prostacyclin metabolites in pulmonary hypertension [see comments]. N Engl J Med. 1992;327:70-5.
44. Thomson JR, Trembath RC. Primary pulmonary hypertension: the pressure rises for a gene. J Clin Pathol. 2000;53:899-903.
45. Tsukada T, Yokoyama K, Arai T, et al. Evidence of association of the ecNOS gene polymorphism with plasma NO metabolite levels in humans. Biochem Biophys Res Common. 1998;245: 190-3.
46. Pearson DL, Dawling S, Walsh WF, et al. Neonatal pulmonary hypertension: urea-cycle intermediates, nitric oxide production, and carbamoyl-phosphate synthetase function. N Engl J Med. 2001;344:1832-8.
47. Chaouat A, Coulet F, Favre C, et al. Endoglin germline mutation in a patient with hereditary hemorrhagic telangiectasia and dexfenfluramine-associated pulmonary arterial hypertension. Thorax. 2004;59:446-8.
48. Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med 1987; 107:216-23.
49. MacGregor AJ, Canavan R, Knight C, et al. Pulmonary hypertension in systemic sclerosis: risk factors for progression and consequences for survival. Rheumatology (Oxford). 2001;40: 453-9.
50. Ahearn GS, Tapson VF, Rebeiz A, et al. Electrocardiography to define clinical status in primary pulmonary hypertension and pulmonary arterial hypertension secondary to collagen vascular disease. Chest. 2002;122:524-7.
51. Ommen SR, Nishimura RA, Hurrell DG, et al. Assessment of right atrial pressure with 2-dimensional and Doppler echocardiography: a simultaneous catheterization and echocardiographic study. Mayo Clin Proc. 2000;75:24-9.
52. Borgeson DD, Seward JB, Miller FA Jr, et al. Frequency of Doppler measurable pulmonary artery pressures. J Am Soc Echocardiogr. 1996;9:832-7.
53. Denton CP, Cailes JB, Phillips GD, et al. Comparison of Doppler echocardiography and right heart catheterization to assess pulmonary hypertension in systemic sclerosis. Br J Rheumatol. 1997;36:239-43.
54. Vachiery JL, Brimioulle S, Crasset V, et al. False-positive diagnosis of pulmonary hypertension by Doppler echocardiography. Eur Respir. J 1998;12:1476-8.
55. McQuillan BM, Picard MH, Leavitt M, et al. Clinical correlates and reference intervals for pulmonary artery systolic pressure among echocardiographically normal subjects. Circulation. 2001;104:2797-802.
56. Mukerjee D, St George D, Knight C, et al. Echocardiography and pulmonary function as screening tests for pulmonary arterial hypertension in systemic sclerosis. Rheumatology. 2004;43: 461-6.
57. Hinderliter AL, Willis PW, Barst RJ, et al. Effects of long-term infusion of prostacyclin (epoprostenol) on echocardiographic measures of right ventricular structure and function in primary pulmonary hypertension. Primary Pulmonary Hypertension Study Group [see comments]. Circulation. 1997;95: 1479-86.
58. Galie N, Hinderliter AL, Torbicki A, et al. Effects of the oral endothelin-receptor antagonist bosentan on echocardiographic and doppler measures in patients with pulmonary arterial hypertension. J Am Coll Cardiol. 2003;41:1380-6.
59. Pauwels RA, Buist AS, Calverley PM, et al. Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease. NHLBI/WHO Global Initiative for Chronic Obstructive Lung Disease (GOLD) Workshop summary. Am J Respir Crit Care Med. 2001;163:1256-76.
60. Kazerooni EA, Martinez FJ, Flint A, et al. Thin-section CT obtained at 10-mm increments versus limited three-level thin-section CT for idiopathic pulmonary fibrosis: correlation with pathologic scoring. AJR Am J Roentgenol. 1997;169:977-83.
61. Fedullo PF, Auger WR, Kerr KM, et al. Chronic thromboembolic pulmonary hypertension. N Engl J Med. 2001;345:1465-72.
62. Resten A, Maitre S, Capron F, et al. Pulmonary hypertension: CT findings in pulmonary veno-occlusive disease. J Radiol. 2003;84:1739-45.
63. Ley S, Kreitner KF, Fink C, et al. Assessment of pulmonary hypertension by CT and MR imaging. Eur Radiol. 2004;14:359-68.
64. Dartevelle P, Fadel E, Mussot S, et al. Chronic thromboembolic pulmonary hypertension. Eur Respir J. 2004;23:637-48.
65. Rich S, Kieras K, Groves B, et al. Antinuclear antibodies in primary pulmonary hypertension. J Am Coll Cardiol. 1986;8:1307-11.
66. Albrecht T, Blomley MJ, Cosgrove DO, et al. Non-invasive diagnosis of hepatic cirrhosis by transit-time analysis of an ultrasound contrast agent. Lancet 1999;353:1579-83.
67. Naeije R. Hepatopulmonary syndrome and portopulmonary hypertension. Swiss Medical Weekly. 2003;133:163-9.
68. Miyamoto S, Nagaya N, Satoh T, et al. Clinical correlates and prognostic significance of six-minute walk test in patients with primary pulmonary hypertension. Comparison with cardiopulmonary exercise testing. Am J Respir Crit Care Med. 2000;161: 487-92.
69. Wensel R, Opitz CF, Anker SD, et al. Assessment of survival in patients with primary pulmonary hypertension: importance of cardiopulmonary exercise testing. Circulation. 2002;106:319-24.
70. Galie N, Manes A, Branzi A. The new clinical trials on pharmacological treatment in pulmonary arterial hypertension. Eur Respir J. 2002;20:1037-49.
71. Peacock A, Naeije R, Galie N, et al. End-points for clinical trials in pulmonary arterial hypertension. Eur Respir J. 2004;23:947-53.
72. Guyatt GH, Sullivan MJ, Thompson PJ, et al. The 6-minute walk: a new measure of exercise capacity in patients with chronic failure. Can Med Assoc J. 1985;132:919-23.
73. Paciocco G, Martinez F, Bossone E, et al. Oxygen desaturation on the six-minute walk test and mortality in untreated primary pulmonary hypertension. Eur Respir J. 2001;17:647-52.
74. Barst RJ, McGoon M, McLaughlin VV, et al. Beraprost therapy for pulmonary arterial hypertension. J Am Coll Cardiol. 2003; 41:2115-25.
75. Barst RJ, Langleben D, Frost A, et al. Sitaxsentan therapy for pulmonary arterial hypertension. Am J Respir Crit Care Med. 2004;169:441-7.
76. 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.
77. McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation. 2002;106:1477-82.
78. McLaughlin V, Sitbon O, Rubin L, et al. The effect of first-line Bosentan on survival of patients with primary pulmonary hypertension. Am J Respir Crit Care Med. 2003;167:A442.
79. Rich S, Kaufmann E, Levy PS. The effect of high doses of calciumchannel blockers on survival in primary pulmonary hypertension [see comments]. N Engl J Med. 1992;327:76-81.
80. Sitbon O, Humbert M, Jagot JL, et al. Inhaled nitric oxide as a screening agent for safely identifying responders to oral calciumchannel blockers in primary pulmonary hypertension. Eur Respir J. 1998;12:265-70.
81. Sitbon O, Humbert M, Ioos V, et al. Who benefits from long-term calcium-channel blocker therapy in primary pulmonary hypertension? Am J Resp Crit Care Med. 2003;167:A440.
82. Weir EK, Rubin LJ, Ayres SM, et al. The acute administration of vasodilators in primary pulmonary hypertension. Experience from the National Institutes of Health Registry on Primary Pulmonary Hypertension. Am Rev Respir Dis. 1989;140:1623-30.
83. Galie N, Ussia G, Passarelli P, et al. Role of pharmacologic tests in the treatment of primary pulmonary hypertension. Am J Cardiol. 1995;75:A55-62.
84. McLaughlin VV, Genthner DE, Panella MM, et al. Reduction in pulmonary vascular resistance with long-term epoprostenol (prostacyclin) therapy in primary pulmonary hypertension [see comments]. N Engl J Med. 1998;338:273-7.
85. Galie N, Seeger W, Naeije R, et al. Comparative analysis of clinical trials and evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol. 2004;43:S81-8.
86. Nootens M, Kaufman E, Rich S. Short-term effectiveness of nifedipine in secondary pulmonary hypertension. Am J Cardiol. 1993;71:1475-6.
87. 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: 780-8.
88. Eysmann SB, Palevsky HI, Reichek N, et al. Two-dimensional and Doppler-echocardiographic and cardiac catheterization correlates of survival in primary pulmonary hypertension. Circulation. 1989;80:353-60.
89. Raymond RJ, Hinderliter AL, Willis PW, et al. Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. J Am Coll Cardiol 2002;39:1214-9.
90. Tei C, Dujardin KS, Hodge DO, et al. Doppler echocardiographic index for assessment of global right ventricular function [see comments]. J Am Soc Echocardiogr. 1996;9:838-47.
91. Yeo TC, Dujardin KS, Tei C, et al. Value of a Doppler-derived index combining systolic and diastolic time intervals in predicting outcome in primary pulmonary hypertension. Am J Cardiol. 1998;81:1157-61.
92. 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;93:484-8.
93. Voelkel MA, Wynne KM, Badesch DB, et al. Hyperuricemia in severe pulmonary hypertension. Chest. 2000;117:19-24.
94. Nagaya N, Nishikimi T, Uematsu M, et al. Plasma brain natriuretic peptide as a prognostic indicator in patients with primary pulmonary hypertension. Circulation. 2000;102:865-70.
95. Nootens M, Kaufmann E, Rector T, et al. Neurohormonal activation in patients with right ventricular failure from pulmonary hypertension: relation to hemodynamic variables and endothelin levels. J Am Coll Cardiol. 1995;26:1581-5.
96. Rubens C, Ewert R, Halank M, et al. Big endothelin-1 and endothelin1 plasma levels are correlated with the severity of primary pulmonary hypertension. Chest. 2001;120:1562-9.
97. Torbicki A, Kurzyna M, Kuca P, et al. Detectable serum cardiac troponin T as a marker of poor prognosis among patients with chronic precapillary pulmonary hypertension. Circulation. 2003;108:844-8.
98. Galie N. Do we need controlled clinical trials in pulmonary arterial hypertension? Eur Respir J. 2001;17:1-3.
99. Badesch BD, Abman SH, Ahearn GS, et al. Medical therapy for pulmonary arterial hypertension. ACCP Evidence-Based Guidelines for Clinical Practice. Chest. 2004;126:355-625.
100. Manes A, Galie N, Branzi A. Women and pulmonary arterial hypertension. Eur Respir J (Monograph). 2003;8:219-27.
101. Nelson DM, Main E, Crafford W, et al. Peripartum heart failure due to primary pulmonary hypertension. Obstet Gynecol. 1983; 62:S58-63.
102. Weiss BM, Zemp L, Seifert B, et al. Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996. J Am Coll Cardiol. 1998;31:1650-7.
103. Nootens M, Rich S. Successful management of labor and delivery in primary pulmonary hypertension. Am J Cardiol. 1993; 71:1124-5.
104. Expert consensus document on management of cardiovascular diseases during pregnancy. Eur Heart J. 2003;24:761-81.
105. Abenhaim L, Moride Y, Brenot F, et al. Appetite-suppressant drugs and the risk of primary pulmonary hypertension. International Primary Pulmonary Hypertension Study Group [see comments]. N Engl J Med. 1996;335:609-16.
106. Deanfield J, Thaulow E, Warnes C, et al. Management of grown up congenital heart disease. Eur Heart J. 2003;24:1035-84.
107. Sanguinetti M, Galie N, Magelli C, et al. Precapillary pulmonary hypertension: effect of Captopril. G Ital Cardiol. 1982;12: 436-41.
108. Girgis A, Sanson-Fisher RW. Breaking bad news: consensus guidelines for medical practitioners. J Clin Oncol. 1995;13: 2449-56.
109. Stewart M, Davidson K, Meade D, et al. Group support for couples coping with a cardiac condition. J Adv Nurs. 2001;33: 190-9.
110. Moser KM, Fedullo PF, Finkbeiner WE, et al. Do patients with primary pulmonary hypertension develop extensive central thrombi? Circulation. 1995;91:741-5.
111. Fuster V, Steele PM, Edwards WD, et al. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation. 1984;70:580-7.
112. Frank H, Mlczoch J, Huber K, et al. The effect of anticoagulant therapy in primary and anorectic drug-induced pulmonary hypertension. Chest. 1997;112:714-21.
113. 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 [see comments]. Ann Intern Med. 2000;132:425-34.
114. Sandoval J, Aguirre JS, Pulido T, et al. Nocturnal oxygen therapy in patients with the eisenmenger syndrome. Am J Respir Crit Care Med. 2001;164:1682-7.
115. 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;114:787-92.
116. Naeije R, Vachiery F. Medical treatment of pulmonary hypertension. Clin Chest Med. 2004;22:517-27.
117. 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;76:135-41.
118. Barst RJ, Maislin G, Fishman AP. Vasodilator therapy for primary pulmonary hypertension in children. Circulation. 1999;99: 1197-208.
119. Rich S, Kaufmann E. High dose titration of calcium channel blocking agents for primary pulmonary hypertension: guidelines for shortterm drug testing. J Am Coll Cardiol. 1991;18:1323-7.
120. Jones DA, Benjamin CW, Linseman DA. 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: 890-6.
121. 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 [see comments]. N Engl J Med. 1996;334:296-302.
122. 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;112:485-91.
123. Robbins IM, Gaine SP, Schilz R, et al. Epoprostenol for treatment of pulmonary hypertension in patients with systemic lupus erythematosus [see comments]. Chest. 2000;117:14-8.
124. McLaughlin VV, Genthner DE, Panella MM, et al. Compassionate use of continuous prostacyclin in the management of secondary pulmonary hypertension: a case series [see comments]. Ann Intern Med. 1999;130:740-3.
125. Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation. 1999;99:1858-65.
126. Kuo PC, Johnson LB, Plotkin JS, et al. Continuous intravenous infusion of epoprostenol for the treatment of portopulmonary hypertension. Transplantation. 1997;63:604-6.
127. Bakst AE, Gaine SP, Rubin LJ. Continuous intravenous epoprostenol therapy for pulmonary hypertension in Gaucher's disease. Chest. 1999;116:1127-9.
128. Aguilar RV, Farber HW. Epoprostenol (prostacyclin) therapy in HIVassociated pulmonary hypertension. Am J Respir Crit Care Med. 2000;162:1846-50.
129. Higenbottam T, Butt AY, McMahon A, et al. Long-term intravenous prostaglandin (epoprostenol or iloprost) for treatment of severe pulmonary hypertension. Heart. 1998;80:151-5.
130. Simonneau G, Barst RJ, Galie N, et al. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension. A double-blind, randomized, placebo-controlled trial. Am J Respir Crit Care Med. 2002; 165:800-4.
131. McLaughlin VV, Gaine SP, Barst RJ, et al. Efficacy and safety of treprostinil: an epoprostenol analog for primary pulmonary hypertension. J Cardiovasc Pharmacol. 2003;41:293-9.
132. Vachiery JL, Hill N, Zwicke D, et al. Transitioning from i.v. epoprostenol to subcutaneous treprostinil in pulmonary arterial hypertension. Chest. 2002;121:1561-5.
133. Galie N, Humbert M, Vachiery JL, et al. Effects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: a randomised, double-blind placebo-controlled trial. J Am Coll Cardiol. 2002;39:1496-502.
134. 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;35:176-82.
135. Olschewski H, Simonneau G, Galie N, et al. Inhaled Iloprost in severe pulmonary hypertension. N Engl J Med. 2002;347:322-9.
136. 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;342:1866-70.
137. Olschewski H, Ghofrani HA, Walmrath D, et al. Inhaled prostacyclin and iloprost in severe pulmonary hypertension secondary to lung fibrosis. Am J Respir Crit Care Med. 1999;160: 600-7.
138. Higenbottam TW, Butt AY, Dinh-Xaun AT, et al. Treatment of pulmonary hypertension with the continuous infusion of a prostacyclin analogue, iloprost. Heart. 1998;79:175-9.
139. Ewert R, Opitz C, Wensel R, et al. Iloprost as inhalational and intravenous long-term treatment of patients with primary pulmonary hypertension. Register of the Berlin Study Group for Pulmonary Hypertension. Z Kardiol. 2000;89:987-99.
140. Stewart DJ, Levy RD, Cernacek P et al. Increased plasma endothelin-1 in pulmonary hypertension: marker or mediator of disease? Ann Intern Med. 1991;114:464-9.
141. Giaid A, Yanagisawa M, Langleben D, et al. Expression of endothelin1 in the lungs of patients with pulmonary hypertension. N Engl J Med. 1993;328:1732-9.
142. Clozel M, Breu V, Gray GA, et al. In vivo pharmacology of Ro 462005, the first synthetic nonpeptide endothelin receptor antagonist: implications for endothelin physiology. J Cardiovasc Pharmacol. 1993;22:S377-9.
143. Channick R, Badesch DB, Tapson VF, et al. Effects of the dual endothelin receptor antagonist bosentan in patients with pulmonary hypertension: a placebo-controlled study. J Heart Lung Transplant. 2001;20:262-3.
144. Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med. 2002;346:896-903.
145. Fattinger K, Funk C, Pantze M, et al. The endothelin antagonist bosentan inhibits the canalicular bile salt export pump: a potential mechanism for hepatic adverse reactions. Clin Pharmacol Therapeutics. 2001;69:223-31.
146. Sitbon O, Badesch DB, Channick RN, et al. Effects of the dual endothelin receptor antagonist bosentan in patients with pulmonary arterial hypertension: a 1-year follow-up study. Chest. 2003;124:247-54.
147. Kim NS, Channick R, Rubin LJ. Successful withdrawal of chronic epoprostenol therapy for pulmonary arterial hypertension. Chest. 2003;124:1612-5.
148. Barst RJ, Ivy D, Dingemanse J, et al. Pharmacokinetics, safety, and efficacy of bosentan in pediatric patients with pulmonary arterial hypertension. Clin Pharmacol Ther. 2003;73:372-82.
149. Barst RJ, Rich S, Widlitz A, et al. Clinical efficacy of sitaxsentan, an endothelin-A receptor antagonist, in patients with pulmonary arterial hypertension: open-label pilot study. Chest. 2002; 121:1860-8.
150. Rubin LJ, Galie N, Badesch BD, et al. Ambrisentan improves exercise capacity and clinical measures in pulmonary arterial hypertension (PAH). Am J Crit Care Med. 2004;169:A210.
151. Corbin JD, Francis SH. Cyclic GMP phosphodiesterase-5: target of sildenafil. J Biol Chem. 1999;274:13729-32.
152. Manes A, Tantini B, Galie N, et al. Effects of sildenafil on growth and viability of cultured human pulmonary artery smooth muscle cells. J Am Coll Cardiol. 2003;41:223A.
153. Polson JB, Strada SJ. Cyclic nucleotide phosphodiesterases and vascular smooth muscle. Annu Rev Pharmacol Toxicol. 1996; 36:403-27.
154. Cohen AH, Hanson K, Morris K, et al. Inhibition of cyclic 30-50 guanosine monophosphate-specific phosphodiesterase selectively vasodilates the pulmonary circulation in chronically hypoxic rats. J Clin Invest. 1996;97:172-9.
155. Hanson KA, Burns F, Rybalkin SD, et al. Developmental changes in lung cGMP phosphodiesterase-5 activity, protein, and message. Am J Respir Crit Care Med. 1998;158:279-88.
156. Hanson KA, Ziegler JW, Rybalkin SD, et al. Chronic pulmonary hypertension increases fetal lung cGMP phosphodiesterase activity. Am J Physiol. 1998;275:L931-41.
157. Prasad S, Wilkinson J, Gatzoulis MA. Sildenafil in primary pulmonary hypertension. N Engl J Med. 2000;343:1342.
158. Bhatia S, Frantz RP, Severson CJ, et al. Immediate and long-term hemodynamic and clinical effects of sildenafil in patients with pulmonary arterial hypertension receiving vasodilator therapy. Mayo Clin Proc. 2003;78:1207-13.
159. Michelakis ED, Tymchak W, Noga M, et al. Long-term treatment with oral sildenafil is safe and improves functional capacity and hemodynamics in patients with pulmonary arterial hypertension. Circulation. 2003;108:2066-9.
160. Ghofrani HA, Schermuly RT, Rose F, et al. Sildenafil for long-term treatment of nonoperable chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med. 2003;167:1139-41.
161. Ghofrani HA, Wiedemann R, Rose F, et al. Sildenafil for treatment of lung fibrosis and pulmonary hypertension: a randomised controlled trial. Lancet. 2002;360:895-900.
162. Ghofrani A, for the Sildenafil 1140 Study Group. Efficacy and safety of sildenafil citrate in pulmonary arterial hypertension: results of a multinational, randomized, double-blind, placebo-controlled trial. Am Coll Chest Phys, 2004.
163. Humbert M, Barst R, Robbins I, et al. Combination of Epoprosterol and Bosentan in pulmonary arterial hypertension: BREATHE-2. Eur Respir J. 2004;24:353-9.
164. Hoeper M, Taha N, Bekjarova A, et al. Bosentan treatment in patients with primary pulmonary hypertension receiving nonparenteral prostanoids. Eur Respir J. 2003;330-4.
165. Ghofrani HA, Rose F, Schermuly RT, et al. Oral sildenafil as longterm adjunct therapy to inhaled iloprost in severe pulmonary arterial hypertension. J Am Coll Cardiol. 2003;42:158-64.
166. Austen WG, Morrow AG, Berry WB. Experimental studies of the surgical treatment of primary pulmonary hypertension. J Thorac Cardiovasc Surg. 1964;48:448-55.
167. Rozkovec A, Montanes P, Oakley CM. Factors that influence the outcome of primary pulmonary hypertension. Br Heart J. 1986;55:449-58.
168. Sandoval J, Rothman A, Pulido T. Atrial septostomy for pulmonary hypertension. Clin Chest Med. 2001;22:547-60.
169. Klepetko W, Mayer E, Sandoval J, et al. Interventional and surgical modalities of treatment for pulmonary arterial hypertension. J Am Coll Cardiol. 2004;43:S73-80.
170. Hertz MI, Taylor DO, Trulock EP, et al. The registry of the International Society for Heart and Lung Transplantation: Nineteenth Official Report 2002. J Heart Lung Transplant. 2002;21: 950-70.
171. Long W. Persistent pulmonary hypertension of the newborn syndrome. In: Long WA, editor. Fetal and neonatal cardiology. Philadelphia: WB Saunders; 1989. p. 627-55.
172. Weigel TJ, Hageman JR. National survey of diagnosis and management of persistent pulmonary hypertension of the newborn. J Perinatol. 1990;10:369-75.
173. Clark RH, Kueser TJ, Walker MW, et al. Low-dose nitric oxide therapy for persistent pulmonary hypertension of the newborn. Clinical Inhaled Nitric Oxide Research Group. N Engl J Med. 2000;342:469-74.
174. Walsh MC, Stork EK. Persistent pulmonary hypertension of the newborn. Rational therapy based on pathophysiology. Clin Perinatol. 2001;28:609-27.
175. Sandoval J, Bauerle O, Gómez A, et al. Primary pulmonary hypertension in children: clinical characterization and survival. J Am Coll Cardiol. 1995;25:466-74.
176. Gaine SP, Rubin LJ. Primary pulmonary hypertension. Lancet. 1998;352:719-25 [fe de effatas en Lancet. 1999;353:74].
177. Barst R, Ivy D, Widlitz AC, et al. Pharmacokinetics, safety, and efficacy of bosentan in pediatric patients with pulmonary arterial hypertension. Clin Pharmacol Ther. 2003;73:372-82.
178. Abrams D, Schulze-Neick I, Magee AG. Sildenafil as a selective pulmonary vasodilator in childhood primary pulmonary hypertension. Heart. 2000;84:E4.
179. Vongpatanasin W, Brickner ME, Hillis LD, et al. The Eisenmenger syndrome in adults. Ann Intern Med. 1998;128:745-55.
180. Hopkins WE, Ochoa LL, Richardson GW, et al. Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome. J Heart Lung Transplant. 1996;15:100-5.
181. Berman EB, Barst RJ. Eisenmenger's syndrome: current management. Prog Cardiovasc Dis. 2002;45:129-38.
182. Sandoval J, Aguirre JS, Pulido T, et al. Nocturnal oxygen therapy in patients with the Eisenmenger syndrome. Am J Respir Crit Care Med. 2001;164:1682-7.
183. Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults. Second of two parts. N Engl J Med. 2000;342:334-42 [fe de erratas en N Engl J Med. 2000;342:988].
184. Hadengue A, Benhayoun MK, Lebrec D, et al. Pulmonary hypertension complicating portal hypertension: prevalence and relation to splanchnic hemodynamics. Gastroenterology. 1991; 100:520-8.
185. Herve P, Lebrec D, Brenot F, et al. Pulmonary vascular disorders in portal hypertension. Eur Respir J. 1998;11:1153-66.
186. Hoeper MM, Krowka MJ, Strassburg CP. Portopulmonary hypertension and hepatopulmonary syndrome. Lancet. 2004;363: 1461-8.
187. McDonnell PJ, Toye PA, Hutchins GM. Primary pulmonary hypertension and cirrhosis: are they related. Am Rev Respir Dis. 1983;127:437-41.
188. The International Primary Pulmonary Hypertension Study (IPPHS). Chest. 1994;105:S37-41.
189. Lebrec D, Capron JP, Dhumeaux D, et al. Pulmonary hypertension complicating portal hypertension. Am Rev Respir Dis. 1979;120:849-56.
190. Ruttner JR, Bartschi JP, Niedermann R, et al. Plexogenic pulmonary arteriopathy and liver cirrhosis. Thorax. 1980;35:133-6.
191. Kuo PC, Plotkin JS, Johnson LB, et al. Distinctive clinical features of portopulmonary hypertension [see comments]. Chest. 1997;112:980-6.
192. Robalino BD, Moodie DS. Association between primary pulmonary hypertension and portal hypertension: analysis of its pathophysiology and clinical, laboratory and hemodynamic manifestations. J Am Coll Cardiol. 1991;17:492-8.
193. Plotkin JS, Kuo PC, Rubin LJ, et al. Successful use of chronic epoprostenol as a bridge to liver transplantation in severe portopulmonary hypertension. Transplantation. 1998;65:457-9.
194. Krowka MJ, Frantz RP, McGoon MD, et al. Improvement in pulmonary hemodynamics during intravenous epoprostenol (prostacyclin): a study of 15 patients with moderate to severe portopulmonary hypertension. Hepatology. 1999;30: 641-8.
195. Findlay JY, Plevak DJ, Krowka MJ, et al. Progressive splenomegaly after epoprostenol therapy in portopulmonary hypertension. Liver Transpl Surg. 1999;5:362-5.
196. Krowka MJ, Plevak DJ, Findlay JY, et al. Pulmonary hemodynamics and perioperative cardiopulmonary-related mortality in patients with portopulmonary hypertension undergoing liver transplantation. Liver Transpl. 2000;6:443-50.
197. Tan HP, Markowitz JS, Montgomery RA, et al. Liver transplantation in patients with severe portopulmonary hypertension treated with preoperative chronic intravenous epoprostenol. Liver Transpl. 2001;7:745-9.
198. Pirenne J, Verleden G, Nevens F, et al. Combined liver and (heart) lung transplantation in liver transplant candidates with refractory portopulmonary hypertension. Transplantation. 2002; 73:140-2.
199. Schott R, Chaouat A, Launoy A, et al. Improvement of pulmonary hypertension after liver transplantation. Chest. 1999;115: 1748-9.
200. Mitchell DM, Miller RF. AIDS and the lung: update 1995. 2. New developments in the pulmonary diseases affecting HIV infected individuals. Thorax. 1995;50:294-302.
201. Mehta NJ, Khan IA, Mehta RN, et al. HIV-related pulmonary hypertension: analytic review of 131 cases. Chest. 2000;118: 1133-41.
202. Mehta NJ, Khan IA, Mehta RN, et al. Acute coronary syndrome in patients with human immunodeficiency virus disease. Angiology. 2002;53:545-9.
203. Opravil M, Pechere M, Speich R, et al. HIV-associated primary pulmonary hypertension. A case control study. Swiss HIV Cohort Study. Am J Respir Crit Care Med. 1997;155:990-5.
204. Humbert M, Monti G, Fartoukh M, et al. Platelet-derived growth factor expression in primary pulmonary hypertension: comparison of HIV seropositive and HIV seronegative patients. Eur Respir J. 1998;11:554-9.
205. Ehrenreich H, Rieckmann P, Sinowatz F, et al. Potent stimulation of monocytic endothelin-1 production by HIV-1 glycoprotein 120. J Immunol. 1993;150:4601-9.
206. Mette SA, Palevsky HI, Pietra GG, et al. Primary pulmonary hypertension in association with human immunodeficiency virus infection. A possible viral etiology for some forms of hypertensive pulmonary arteriopathy. Am Rev Respir Dis. 1992;145: 1196-200.
207. Tuder RM, Groves B, Badesch DB, et al. Exuberant endothelial cell growth and elements of inflammation are present in plexiform lesions of pulmonary hypertension. Am J Pathol. 1994; 144:275-85.
208. Voelkel NF, Cool C, Lee SD, et al. Primary pulmonary hypertension between inflammation and cancer. Chest. 1998;114: S225-30.
209. Nunes H, Humbert M, Sitbon O, et al. Prognostic factors for survival in human immunodeficiency virus-associated pulmonary arterial hypertension. Am J Respir Crit Care Med. 2003; 167:1433-9.
210. Petitpretz P, Brenot F, Azarian R, et al. Pulmonary hypertension in patients with human immunodeficiency virus infection. Comparison with primary pulmonary hypertension. Circulation. 1994;89:2722-7.
211. Speich R, Jenni R, Opravil M, et al. Regression of HIV-associated pulmonary arterial hypertension and long-term survival during antiretroviral therapy. Swiss Med Wkly. 2001;131:663-5.
212. Sitbon O, Gressin V, Speich R, et al. Bosentan in pulmonary arterial hypertension associated with HIV infection. Eur Respir J. 2003;22:S563.
213. Hoeper M, Galie N, Simonneau G, et al. New treatments for pulmonary arterial hypertension. Am J Respir Crit Care Med. 2002;165:1209-16.
214. Mukerjee D, St George D, Coleiro B, et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis. 2003;62:1088-93.
215. Pan TL, Thumboo J, Boey ML. Primary and secondary pulmonary hypertension in systemic lupus erythematosus. Lupus. 2000;9:338-42.
216. Sanchez O, Humbert M, Sitbon O, et al. Treatment of pulmonary hypertension secondary to connective tissue diseases. Thorax. 1999;54:273-7.
217. Fagan KA, Badesch DB. Pulmonary hypertension associated with connective tissue disease. Prog Cardiovasc Dis. 2002;45: 225-34.
218. Yoshida S, Katayama M. Pulmonary hypertension in patients with connective tissue diseases. Nippon Rinsho. 2001;59:1164-7.
219. 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;13:1351-6.
220. Kuhn KP, Byrne DW, Arbogast PG, et al. Outcome in 91 consecutive patients with pulmonary arterial hypertension receiving epoprostenol. Am J Respir Crit Care Med. 2003;167:580-6.
221. Oudiz R, Schilz R, Barst R, et al. Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease. Chest. 2004;126:420-7.
222. Holcomb Jr BW, Loyd JE, Ely EW, et al. Pulmonary veno-occlusive disease: a case series and new observations. Chest. 2000;118:1671-9.
223. Langleben D, Heneghan JM, Batten AP, et al. Familial pulmonary capillary hemangiomatosis resulting in primary pulmonary hypertension. Ann Intern Med. 1988;109:106-9.
224. Runo JR, Vnencak-Jones CL, Prince M, et al. Pulmonary veno-occlusive disease caused by an inherited mutation in bone morphogenetic protein receptor II. Am J Respir Crit Care Med. 2003; 167:889-94.
225. Dufour B, Maitre S, Humbert M, et al. High-resolution CT of the chest in four patients with pulmonary capillary hemangiomatosis or pulmonary venoocclusive disease. Am J Roentgenol. 1998;171:1321-4.
226. Resten A, Maitre S, Humbert M, et al. Pulmonary hypertension: CT of the chest in pulmonary venoocclusive disease. Am J Roentgenol. 2004;183:65-70.
227. Swensen SJ, Tashjian JH, Myers JL, et al. Pulmonary venoocclusive disease: CT findings in eight patients. Am J Roentgenol. 1996;167:937-40.
228. Rabiller A, Humbert M, Sitbon M, et al. Bronchoalveolar lavage as a diagnostic tool in pulmonary hypertension: occult alveolar hemorrhage is a common feature of pulmonary venoocclusive disease. Am J Resp Crit Care Med. 2004;167:A276.
229. Palmer SM, Robinson LJ, Wang A, et al. Massive pulmonary edema and death after prostacyclin infusion in a patient with pulmonary veno-occlusive disease. Chest. 1998;113:237-40.
230. Humbert M, Maître S, Capron F, et al. Pulmonary edema complicating continuous intravenous prostacyclin in pulmonary capillary hemangiomatosis. Am J Respir Crit Care Med. 1998; 157:1681-5.