Idiopathic pulmonary arterial hypertension

Seminars in Respiratory and Critical Care Medicine

Volumn 34, Issue 5, 2013, Pages 560-567

  Souza, Rogerio ^a   Jardim, Carlos ^a   Humbert, Marc ^b  

^a UNIVERSITY OF SÃO PAULO   (Brazil)

^b University of South Paris Kremlin Bicetre   (France)

Author keywords

epidemiology; heritable PAH; idiopathic pulmonary arterial hypertension; pulmonary arterial hypertension

Indexed keywords

AMBRISENTAN; AMINOREX; BOSENTAN; ILOPROST; MACITENTAN; PROSTACYCLIN; SILDENAFIL; TREPROSTINIL;

ARTICLE; GENETIC COUNSELING; GENETIC PREDISPOSITION; HEART FAILURE WITH PRESERVED EJECTION FRACTION; HEMODYNAMICS; HUMAN; MORTALITY; PRIORITY JOURNAL; PULMONARY HYPERTENSION; RISK FACTOR; SMOOTH MUSCLE FIBER; SURVIVAL; VASCULAR RESISTANCE; VASOCONSTRICTION;

AMINOREX; ANTIHYPERTENSIVE AGENTS; APPETITE DEPRESSANTS; EPOPROSTENOL; FENFLURAMINE; GENETIC PREDISPOSITION TO DISEASE; HUMANS; HYPERTENSION, PULMONARY; ILOPROST; PHENYLPROPIONATES; PIPERAZINES; PROTEIN KINASE INHIBITORS; PURINES; PYRAZOLES; PYRIDAZINES; PYRIMIDINES; RISK FACTORS; SULFONAMIDES; SULFONES; THIAZOLES; VASODILATOR AGENTS;

EID: 84884226537     PISSN: 10693424     EISSN: 10989048     Source Type: Journal    
DOI: 10.1055/s-0033-1355439     Document Type: Article

References (73)

1. Souza R., Jardim C. Trends in pulmonary arterial hypertension. Eur Respir Rev: 2009; 18 111 7 12
2. Humbert M. The fifth world symposium on pulmonary hypertension will REVEAL the impact of registries. Eur Respir Rev: 2012; 21 123 4 5
3. Simonneau G., Robbins I. M., Beghetti M., et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol: 2009; 54 (61, Suppl): S43 S54
4. Galiè N., Hoeper M. M., Humbert M., et al. ESC Committee for Practice Guidelines (CPG) Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J: 2009; 30 20 2493 2537
5. Kovacs G., Berghold A., Scheidl S., Olschewski H. Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review. Eur Respir J: 2009; 34 4 888 894
6. Badesch D. B., Champion H. C., Sanchez M. A., et al. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol: 2009; 54 (1, Suppl): S55 S66
7. Cogan J. D., Pauciulo M. W., Batchman A. P., et al. High frequency of BMPR2 exonic deletions/duplications in familial pulmonary arterial hypertension. Am J Respir Crit Care Med: 2006; 174 5 590 598
8. Thomson J. R., Machado R. D., Pauciulo M. W., et al. Sporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR-II, a receptor member of the TGF-beta family. J Med Genet: 2000; 37 10 741 745
9. Machado R. D., Eickelberg O., Elliott C. G., et al. Genetics and genomics of pulmonary arterial hypertension. J Am Coll Cardiol: 2009; 54 (1, Suppl): S32 S42
10. Sztrymf B., Coulet F., Girerd B., et al. Clinical outcomes of pulmonary arterial hypertension in carriers of BMPR2 mutation. Am J Respir Crit Care Med: 2008; 177 12 1377 1383
11. Germain M., Eyries M., Montani D., et al. Genome-wide association analysis identifies a susceptibility locus for pulmonary arterial hypertension. Nat Genet: 2013; 45 5 518 521
12. Girerd B., Montani D., Coulet F., et al. Clinical outcomes of pulmonary arterial hypertension in patients carrying an ACVRL1 (ALK1) mutation. Am J Respir Crit Care Med: 2010; 181 8 851 861
13. Girerd B., Montani D., Eyries M., et al. Absence of influence of gender and BMPR2 mutation type on clinical phenotypes of pulmonary arterial hypertension. Respir Res: 2010; 11 73
14. Gurtner H. P. Aminorex and pulmonary hypertension. A review. Cor Vasa: 1985; 27 2-3 160 171
15. Souza R., Humbert M., Sztrymf B., et al. Pulmonary arterial hypertension associated with fenfluramine exposure: report of 109 cases. Eur Respir J: 2008; 31 2 343 348
16. Walker A. M., Langleben D., Korelitz J. J., et al. Temporal trends and drug exposures in pulmonary hypertension: an American experience. Am Heart J: 2006; 152 3 521 526
17. Boutet K., Frachon I., Jobic Y., et al. Fenfluramine-like cardiovascular side-effects of benfluorex. Eur Respir J: 2009; 33 3 684 688
18. Frachon I., Etienne Y., Jobic Y., Le Gal G., Humbert M., Leroyer C. Benfluorex and unexplained valvular heart disease: a case-control study. PLoS ONE: 2010; 5 4 e10128
19. Savale L., Chaumais M. C., Cottin V., et al. Pulmonary hypertension associated with benfluorex exposure. Eur Respir J: 2012; 40 5 1164 1172
20. Montani D., Bergot E., Günther S., et al. Pulmonary arterial hypertension in patients treated by dasatinib. Circulation: 2012; 125 17 2128 2137
21. Yuan J. X., Rubin L. J. Pathogenesis of pulmonary arterial hypertension: the need for multiple hits. Circulation: 2005; 111 5 534 538
22. Rabinovitch M. Molecular pathogenesis of pulmonary arterial hypertension. J Clin Invest: 2012; 122 12 4306 4313
23. Firth A. L., Mandel J., Yuan J. X. Idiopathic pulmonary arterial hypertension. Dis Model Mech: 2010; 3 5-6 268 273
24. 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 24 1732 1739
25. Montani D., Souza R., Binkert C., et al. Endothelin-1/endothelin-3 ratio: a potential prognostic factor of pulmonary arterial hypertension. Chest: 2007; 131 1 101 108
26. Stewart D. J., Levy R. D., Cernacek P., Langleben D. Increased plasma endothelin-1 in pulmonary hypertension: marker or mediator of disease? Ann Intern Med: 1991; 114 6 464 469
27. Michelakis E. D. The role of the NO axis and its therapeutic implications in pulmonary arterial hypertension. Heart Fail Rev: 2003; 8 1 5 21
28. Yuan J. X., Aldinger A. M., Juhaszova M., et al. Dysfunctional voltage-gated K+ channels in pulmonary artery smooth muscle cells of patients with primary pulmonary hypertension. Circulation: 1998; 98 14 1400 1406
29. Price L. C., Wort S. J., Perros F., et al. Inflammation in pulmonary arterial hypertension. Chest: 2012; 141 1 210 221
30. Humbert M., Souza R., Galiè N., McLaughlin V., Simonneau G., Rubin L. Pulmonary arterial hypertension: bridging the present to the future. Eur Respir Rev: 2012; 21 126 267 270
31. Rich S., Dantzker D. R., Ayres S. M., et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med: 1987; 107 2 216 223
32. D'Alonzo G. E., Barst R. J., Ayres S. M., et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med: 1991; 115 5 343 349
33. Humbert M., Sitbon O., Chaouat A., et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med: 2006; 173 9 1023 1030
34. Humbert M., Sitbon O., Chaouat A., et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation: 2010; 122 2 156 163
35. Humbert M., Sitbon O., Yaïci A., et al. French Pulmonary Arterial Hypertension Network Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension. Eur Respir J: 2010; 36 3 549 555
36. Badesch D. B., Raskob G. E., Elliott C. G., et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest: 2010; 137 2 376 387
37. Benza R. L., Miller D. P., Gomberg-Maitland M., et al. Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). Circulation: 2010; 122 2 164 172
38. Ling Y., Johnson M. K., Kiely D. G., et al. Changing demographics, epidemiology, and survival of incident pulmonary arterial hypertension: results from the pulmonary hypertension registry of the United Kingdom and Ireland. Am J Respir Crit Care Med: 2012; 186 8 790 796
39. Lee W. T., Ling Y., Sheares K. K., Pepke-Zaba J., Peacock A. J., Johnson M. K. Predicting survival in pulmonary arterial hypertension in the UK. Eur Respir J: 2012; 40 3 604 611
40. Naeije R., Vachiery J. L., Yerly P., Vanderpool R. The transpulmonary pressure gradient for the diagnosis of pulmonary vascular disease. Eur Respir J: 2013; 41 1 217 223
41. Vachiéry J. L., Gaine S. Challenges in the diagnosis and treatment of pulmonary arterial hypertension. Eur Respir Rev: 2012; 21 126 313 320
42. Costa E. L., Jardim C., Bogossian H. B., Amato M. B., Carvalho C. R., Souza R. Acute vasodilator test in pulmonary arterial hypertension: evaluation of two response criteria. Vascul Pharmacol: 2005; 43 3 143 147
43. Sitbon O., Humbert M., Jaïs X., et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation: 2005; 111 23 3105 3111
44. Barst R. J., Rubin L. J., Long W. A., et al. Primary Pulmonary Hypertension Study Group A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med: 1996; 334 5 296 301
45. Rubin L. J., 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 7 485 491
46. Barst R. J., Gibbs J. S., Ghofrani H. A., et al. Updated evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol: 2009; 54 (1, Suppl): S78 S84
47. Olschewski H., Simonneau G., Galiè N., et al. Aerosolized Iloprost Randomized Study Group Inhaled iloprost for severe pulmonary hypertension. N Engl J Med: 2002; 347 5 322 329
48. Higenbottam T., Butt A. Y., McMahon A., Westerbeck R., Sharples L. Long-term intravenous prostaglandin (epoprostenol or iloprost) for treatment of severe pulmonary hypertension. Heart: 1998; 80 2 151 155
49. Simonneau G., Barst R. J., Galie N., et al. Treprostinil Study Group 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 6 800 804
50. Tapson V. F., Gomberg-Maitland M., McLaughlin V. V., et al. Safety and efficacy of IV treprostinil for pulmonary arterial hypertension: a prospective, multicenter, open-label, 12-week trial. Chest: 2006; 129 3 683 688
51. Tapson V. F., Torres F., Kermeen F., et al. Oral treprostinil for the treatment of pulmonary arterial hypertension in patients on background endothelin receptor antagonist and/or phosphodiesterase type 5 inhibitor therapy (the FREEDOM-C study): a randomized controlled trial. Chest: 2012; 142 6 1383 1390
52. Jing Z. C., Parikh K., Pulido T., et al. Efficacy and safety of oral treprostinil monotherapy for the treatment of pulmonary arterial hypertension: a randomized, controlled trial. Circulation: 2013; 127 5 624 633
53. Rubin L. J., Badesch D. B., Barst R. J., et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med: 2002; 346 12 896 903
54. Humbert M., Segal E. S., Kiely D. G., Carlsen J., Schwierin B., Hoeper M. M. Results of European post-marketing surveillance of bosentan in pulmonary hypertension. Eur Respir J: 2007; 30 2 338 344
55. Galiè N., Olschewski H., Oudiz R. J., et al. Ambrisentan in Pulmonary Arterial Hypertension, Randomized, Double-Blind, Placebo-Controlled, Multicenter, Efficacy Studies (ARIES) Group Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation: 2008; 117 23 3010 3019
56. McGoon M. D., Frost A. E., Oudiz R. J., et al. Ambrisentan therapy in patients with pulmonary arterial hypertension who discontinued bosentan or sitaxsentan due to liver function test abnormalities. Chest: 2009; 135 1 122 129
57. Rubin L., Pulido T., Channick R., et al. Effect of macitentan on morbidity and mortality in pulmonary arterial hypertension (PAH): Results from the SERAPHIN trial. [Abstract]. Chest: 2012; 142 4 1026A
58. Galiè N., Ghofrani H. A., Torbicki A., et al. Sildenafil Use in Pulmonary Arterial Hypertension (SUPER) Study Group Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med: 2005; 353 20 2148 2157
59. Galiè N., Brundage B. H., Ghofrani H. A., et al. Pulmonary Arterial Hypertension and Response to Tadalafil (PHIRST) Study Group Tadalafil therapy for pulmonary arterial hypertension. Circulation: 2009; 119 22 2894 2903
60. Jing Z. C., Yu Z. X., Shen J. Y., et al. Efficacy and Safety of Vardenafil in the Treatment of Pulmonary Arterial Hypertension (EVALUATION) Study Group Vardenafil in pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled study. Am J Respir Crit Care Med: 2011; 183 12 1723 1729
61. Ghofrani H. A., Voswinckel R., Gall H., et al. Riociguat for pulmonary hypertension. Future Cardiol: 2010; 6 2 155 166
62. Ghofrani H. A., Hoeper M. M., Halank M., et al. Riociguat for chronic thromboembolic pulmonary hypertension and pulmonary arterial hypertension: a phase II study. Eur Respir J: 2010; 36 4 792 799
63. Ghofrani H., Galie N., Grimminger F., et al. Riociguat for the treatment of pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled study (PATENT-1). [Abstract]. Chest: 2012; 142 4 1027A 10.1378/chest.1462799
64. Ghofrani H., Grimminger F., Hoeper M., et al. Riociguat for the treatment of inoperable chronic thromboembolic pulmonary hypertension: a randomized, double-blind, placebo-controlled study (CHEST-1). [Abstract]. Chest: 2012; 142 4 1023A
65. McLaughlin V. V., Benza R. L., Rubin L. J., et al. Addition of inhaled treprostinil to oral therapy for pulmonary arterial hypertension: a randomized controlled clinical trial. J Am Coll Cardiol: 2010; 55 18 1915 1922
66. Simonneau G., Rubin L. J., Galiè 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 8 521 530
67. Humbert M., Barst R. J., Robbins I. M., et al. Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2. Eur Respir J: 2004; 24 3 353 359
68. Kemp K., Savale L., O'Callaghan D. S., et al. Usefulness of first-line combination therapy with epoprostenol and bosentan in pulmonary arterial hypertension: an observational study. J Heart Lung Transplant: 2012; 31 2 150 158
69. Souza R., Jardim C., Martins B., et al. Effect of bosentan treatment on surrogate markers in pulmonary arterial hypertension. Curr Med Res Opin: 2005; 21 6 907 911
70. Galiè N., Manes A., Negro L., Palazzini M., Bacchi-Reggiani M. L., Branzi A. A meta-analysis of randomized controlled trials in pulmonary arterial hypertension. Eur Heart J: 2009; 30 4 394 403
71. dos Santos Fernandes C. J., Jardim C. V., Hovnanian A., et al. Survival in schistosomiasis-associated pulmonary arterial hypertension. J Am Coll Cardiol: 2010; 56 9 715 720
72. Sitbon O., McLaughlin V. V., Badesch D. B., et al. Survival in patients with class III idiopathic pulmonary arterial hypertension treated with first line oral bosentan compared with an historical cohort of patients started on intravenous epoprostenol. Thorax: 2005; 60 12 1025 1030
73. O'Callaghan D. S., Humbert M. A critical analysis of survival in pulmonary arterial hypertension. Eur Respir Rev: 2012; 21 125 218 222