Updated treatment algorithm of pulmonary arterial hypertension

Journal of the American College of Cardiology

Volumn 62, Issue 25 SUPPL., 2013, Pages

  Galiè, Nazzareno ^a   Corris, Paul A ^b   Frost, Adaani ^c   Girgis, Reda E ^d   Granton, John ^e   Jing, Zhi Cheng ^f   Klepetko, Walter ^g   McGoon, Michael D ^h   McLaughlin, Vallerie V ⁱ   Preston, Ioana R ^j   Rubin, Lewis J ^k   Sandoval, Julio ^l   Seeger, Werner ^m   Keogh, Anne ⁿ  

^a UNIVERSITY OF BOLOGNA   (Italy)

^b NEWCASTLE UNIVERSITY   (United Kingdom)

^c BAYLOR COLLEGE OF MEDICINE   (United States)

^d MICHIGAN STATE UNIVERSITY   (United States)

^e UNIVERSITY OF TORONTO   (Canada)

^f FUWAI HOSPITAL   (China)

^g MEDICAL UNIVERSITY OF VIENNA   (Austria)

^h MAYO CLINIC   (United States)

ⁱ UNIVERSITY OF MICHIGAN   (United States)

^j TUFTS UNIVERSITY SCHOOL OF MEDICINE   (United States)

^k UNIVERSITY OF CALIFORNIA   (United States)

^l INSTITUTO NACIONAL DE CARDIOLOGÍA IGNACIO CHÁVEZ   (Mexico)

^m JUSTUS LIEBIG UNIVERSITY   (Germany)

ⁿ ST VINCENT S HOSPITAL   (Australia)

more..

Author keywords

endothelin receptor antagonists; guanylate cyclase stimulators; hypertension, pulmonary; lung transplantation; phosphodiesterase type 5 inhibitors; prostanoids; pulmonary

Indexed keywords

AMBRISENTAN; ANTICOAGULANT AGENT; BERAPROST; BOSENTAN; CALCIUM CHANNEL BLOCKING AGENT; DIGITALIS; DIURETIC AGENT; ENDOTHELIN A RECEPTOR; ENDOTHELIN B RECEPTOR; ENDOTHELIN RECEPTOR ANTAGONIST; ILOPROST; IMATINIB; MACITENTAN; OXYGEN; PHOSPHODIESTERASE V INHIBITOR; PLATELET DERIVED GROWTH FACTOR; PROSTACYCLIN; PROTEIN TYROSINE KINASE INHIBITOR; RIOCIGUAT; SILDENAFIL; SITAXSENTAN; TADALAFIL; TREPROSTINIL; VARDENAFIL;

ALGORITHM; CONFERENCE PAPER; CONTINUOUS INFUSION; DIARRHEA; EPISTAXIS; EXERCISE; HEADACHE; HOT FLUSH; HUMAN; JAW PAIN; LUNG TRANSPLANTATION; PREGNANCY; PRIORITY JOURNAL; PSYCHOSOCIAL CARE; PULMONARY HYPERTENSION; SEPTOSTOMY BALLOON CATHETER; TREATMENT RESPONSE;

6-MIN WALK DISTANCE; 6MWD; APAH; ASSOCIATED PULMONARY ARTERIAL HYPERTENSION; BALLOON ATRIAL SEPTOSTOMY; BAS; CALCIUM CHANNEL BLOCKER; CCB; ENDOTHELIN RECEPTOR ANTAGONIST; ENDOTHELIN RECEPTOR ANTAGONISTS; ERA; GUANYLATE CYCLASE STIMULATORS; HYPERTENSION, PULMONARY; LUNG TRANSPLANTATION; PAH; PHOSPHODIESTERASE TYPE-5 INHIBITORS; PROSTANOIDS; PULMONARY; PULMONARY ARTERIAL HYPERTENSION; PULMONARY VASCULAR RESISTANCE; PVR; RANDOMIZED CONTROLLED TRIALS; RCT; WHO-FC; WORLD HEALTH ORGANIZATION FUNCTIONAL CLASS;

ALGORITHMS; ANIMALS; ANTIHYPERTENSIVE AGENTS; CALCIUM CHANNEL BLOCKERS; HUMANS; HYPERTENSION, PULMONARY; LUNG TRANSPLANTATION; PHOSPHODIESTERASE INHIBITORS; TREATMENT OUTCOME;

EID: 84890574392     PISSN: 07351097     EISSN: 15583597     Source Type: Journal    
DOI: 10.1016/j.jacc.2013.10.031     Document Type: Conference Paper

References (86)

1. V.V. McLaughlin, and S. Rich Pulmonary hypertension - advances in medical and surgical interventions J Heart Lung Transplant 17 1998 739 743
2. N. Galie, W. Seeger, R. Naeije, G. Simonneau, and L. Rubin Comparative analysis of clinical trials and evidence-based treatment algorithm in pulmonary arterial hypertension J Am Coll Cardiol 43 2004 S81 S88
3. R. Barst, J. Gibbs, and A. Ghofrani Updated evidence-based treatment algorithm in pulmonary arterial hypertension J Am Coll Cardiol 54 2009 S78 S84
4. N. Galiè, M. Hoeper, and M. Humbert Guidelines on diagnosis and treatment of pulmonary hypertension: the Task Force on Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology and of the European Respiratory Society Eur Heart J 30 2009 2493 2537
5. N. Galie, A. Manes, L. Negro, M. Palazzini, M.L. Bacchi Reggiani, and A. Branzi A meta-analysis of randomized controlled trials in pulmonary arterial hypertension Eur Heart J 30 2009 394 403
6. Y. Bai, L. Sun, S. Hu, and Y. Wei Combination therapy in pulmonary arterial hypertension: a meta-analysis Cardiology 120 2011 157 165
7. X. Jais, K.M. Olsson, and J.A. Barbera Pregnancy outcomes in pulmonary arterial hypertension in the modern management era Eur Respir J 40 2012 881 885
8. D. Mereles, N. Ehlken, and S. Kreuscher Exercise and respiratory training improve exercise capacity and quality of life in patients with severe chronic pulmonary hypertension Circulation 114 2006 1482 1489
9. F.S. de Man, M.L. Handoko, and H. Groepenhoff Effects of exercise training in patients with idiopathic pulmonary arterial hypertension Eur Respir J 34 2009 669 675
10. E. Grunig, N. Ehlken, and A. Ghofrani Effect of exercise and respiratory training on clinical progression and survival in patients with severe chronic pulmonary hypertension Respiration 81 2011 394 401
11. E. Grunig, F. Maier, and N. Ehlken Exercise training in pulmonary arterial hypertension associated with connective tissue diseases Arthritis Res Ther 14 2012 R148
12. E. Grunig, M. Lichtblau, and N. Ehlken Safety and efficacy of exercise training in various forms of pulmonary hypertension Eur Respir J 40 2012 84 92
13. T. Becker-Grunig, H. Klose, and N. Ehlken Efficacy of exercise training in pulmonary arterial hypertension associated with congenital heart disease Int J Cardiol 168 2013 375 381
14. A.A. Weinstein, L.M.K. Chin, and R.E. Keyser Effect of aerobic exercise training on fatigue and physical activity in patients with pulmonary arterial hypertension Respir Med 107 2013 778 784
15. L. Chan, L.M. Chin, and M. Kennedy Benefits of intensive treadmill exercise training on cardiorespiratory function and quality of life in patients with pulmonary hypertension Chest 143 2013 333 343
16. D.R. Thiemann, J. Coresh, W.J. Oetgen, and N.R. Powe The association between hospital volume and survival after acute myocardial infarction in elderly patients N Engl J Med 340 1999 1640 1648
17. M. Delcroix, and R. Naeije Optimising the management of pulmonary arterial hypertension patients: emergency treatments Eur Respir Rev 19 2010 204 211
18. M. Delcroix, Y. Adir, and A.K. Andreassen Care organization for pulmonary arterial hypertension in developed countries: a survey J Heart Lung Transplant 31 4 Suppl 2012 s81 s82
19. Z.C. Jing, X. Jiang, and Z.Y. Han Iloprost for pulmonary vasodilator testing in idiopathic pulmonary arterial hypertension Eur Respir J 33 2009 1354 1360
20. A. Giaid, M. Yanagisawa, and D. Langleben Expression of endothelin-1 in the lungs of patients with pulmonary hypertension N Engl J Med 328 1993 1732 1739
21. D.J. Stewart, R.D. Levy, P. Cernacek, and D. Langleben Increased plasma endothelin-1 in pulmonary hypertension: marker or mediator of disease? Ann Intern Med 114 1991 464 469
22. N. Galie, A. Manes, and A. Branzi The endothelin system in pulmonary arterial hypertension Cardiovasc Res 61 2004 227 237
23. N. Galie, B.D. Badesch, and R. Oudiz Ambrisentan therapy for pulmonary arterial hypertension J Am Coll Cardiol 46 2005 529 535
24. N. Galie, H. Olschewski, and R.J. Oudiz 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 117 2008 3010 3019
25. M. McGoon, A. Frost, and R. Oudiz Ambrisentan therapy in patients with pulmonary arterial hypertension who discontinued bosentan or sitaxsentan due to liver function test abnormalities Chest 135 2009 122 129
26. R.N. Channick, G. Simonneau, and O. Sitbon Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study Lancet 358 2001 1119 1123
27. L.J. Rubin, D.B. Badesch, and R.J. Barst Bosentan therapy for pulmonary arterial hypertension N Engl J Med 346 2002 896 903
28. M. Humbert, R.J. Barst, and I.M. Robbins Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2 Eur Respir J 24 2004 353 359
29. N. Galiè, L.J. Rubin, and M. Hoeper Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial Lancet 371 2008 2093 2100
30. N. Galie, M. Beghetti, and M.A. Gatzoulis Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study Circulation 114 2006 48 54
31. T. Pulido, I. Adzerikho, and R.N. Channick Macitentan and morbidity and mortality in pulmonary arterial hypertension N Engl J Med 369 2013 809 818
32. A. Giaid, and D. Saleh Reduced expression of endothelial nitric oxide synthase in the lungs of patients with pulmonary hypertension N Engl J Med 333 1995 214 221
33. H.A. Ghofrani, N. Galie, and F. Grimminger Riociguat for the treatment of pulmonary arterial hypertension N Engl J Med 369 2013 330 340
34. N. Galie, D. Neuser, Muller, A.V. Scalise, and E. Grunig A placebo-controlled, double-blind phase II interaction study to evaluate blood pressure following addition of riociguat to patients with symptomatic pulmonary arterial hypertension (PAH) receiving sildenafil (PATENT PLUS) Am J Resp Crit Care Med 187 2013 A3530
35. J. Wharton, J.W. Strange, and G.M.O. Moller Antiproliferative effects of phosphodiesterase ype 5 inhibition in human pulmonary artery cells Am J Respir Crit Care Med 172 2005 105 113
36. B. Tantini, A. Manes, and E. Fiumana Antiproliferative effect of sildenafil on human pulmonary artery smooth muscle cells Basic Res Cardiol 100 2005 131 138
37. H.A. Ghofrani, R. Voswinckel, and F. Reichenberger Differences in hemodynamic and oxygenation responses to three different phosphodiesterase-5 inhibitors in patients with pulmonary arterial hypertension: a randomized prospective study J Am Coll Cardiol 44 2004 1488 1496
38. N. Galie, H.A. Ghofrani, and A. Torbicki Sildenafil citrate therapy for pulmonary arterial hypertension N Engl J Med 353 2005 2148 2157
39. B.K.S. Sastry, C. Narasimhan, N.K. Reddy, and B.S. Raju Clinical efficacy of sildenafil in primary pulmonary hypertension: a randomized, placebo-controlled, double-blind, crossover study J Am Coll Cardiol 43 2004 1149 1153
40. K. Iversen, A.S. Jensen, T.V. Jensen, N.G. Vejlstrup, and L. Søndergaard Combination therapy with bosentan and sildenafil in Eisenmenger syndrome: a randomized, placebo-controlled, double-blinded trial Eur Heart J 31 2010 1124 1131
41. T. Singh, M. Rohit, A. Grover, S. Malhotra, and R. Vijayvergiya A randomized, placebo-controlled, double-blind, crossover study to evaluate the efficacy of oral sildenafil therapy in severe pulmonary artery hypertension Am Heart J 151 2006 851.e1 851.e5
42. G. Simonneau, L. Rubin, and N. Galie Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension Ann Intern Med 149 2008 521 530
43. J.L. Vachiery, S. Huez, and H. Gillies Safety, tolerability and pharmacokinetics of an intravenous bolus of sildenafil in patients with pulmonary arterial hypertension Br J Clin Pharmacol 71 2011 289 292
44. N. Galie, B.H. Brundage, and H.A. Ghofrani Tadalafil therapy for pulmonary arterial hypertension Circulation 119 2009 2894 2903
45. Z.C. Jing, Z.X. Yu, and J.Y. Shen Vardenafil in pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled study Am J Respir Crit Care Med 183 2011 1723 1729
46. H.A. Ghofrani, N.W. Morrell, and M.M. Hoeper Imatinib in pulmonary arterial hypertension patients with inadequate response to established therapy Am J Respir Crit Care Med 182 2010 1171 1177
47. M.M. Hoeper, R.J. Barst, and R.C. Bourge Imatinib mesylate as add-on therapy for pulmonary arterial hypertension: results of the randomized IMPRES study Circulation 127 2013 1128 1138
48. D.A. Jones, C.W. Benjamin, and D.A. Linseman Activation of thromboxane and prostacyclin receptors elicits opposing effects on vascular smooth muscle cell growth and mitogen-activated protein kinase signaling cascades Mol Pharmacol 48 1995 890 896
49. B.W. Christman, C.D. McPherson, and J.H. Newman An imbalance between the excretion of thromboxane and prostacyclin metabolites in pulmonary hypertension N Engl J Med 327 1992 70 75
50. N. Galie, A. Manes, and A. Branzi Prostanoids for pulmonary arterial hypertension Am J Respir Med 2 2003 123 137
51. N. Galie, M. Humbert, and J.L. Vachiery 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 39 2002 1496 1502
52. R.J. Barst, M. McGoon, and V.V. Mc Laughlin Beraprost therapy for pulmonary arterial hypertension J Am Coll Cardiol 41 2003 2125
53. L.J. Rubin, J. Mendoza, and M. Hood Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trial Ann Intern Med 112 1990 485 491
54. R.J. Barst, L.J. Rubin, W.A. Long Primary Pulmonary Hypertension Study Group A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension N Engl J Med 334 1996 296 302
55. D.B. Badesch, V.F. Tapson, and M.D. McGoon Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial Ann Intern Med 132 2000 425 434
56. H. Olschewski, G. Simonneau, and N. Galie Inhaled iloprost in severe pulmonary hypertension N Engl J Med 347 2002 322 329
57. V.V. McLaughlin, R.J. Oudiz, and A. Frost Randomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertension Am J Respir Crit Care Med 174 2006 1257 1263
58. M. Hoeper, H. Leuchte, and M. Halank Combining inhaled iloprost with bosentan in patients with idiopathic pulmonary arterial hypertension Eur Respir J 4 2006 691 694
59. T. Higenbottam, A.Y. Butt, A. McMahon, R. Westerbeck, and L. Sharples Long-term intravenous prostaglandin (epoprostenol or iloprost) for treatment of severe pulmonary hypertension Heart 80 1998 151 155
60. G. Simonneau, R.J. Barst, and N. Galie 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 165 2002 800 804
61. J. Hiremath, S. Thanikachalam, and K. Parikh Exercise improvement and plasma biomarker changes with intravenous treprostinil therapy for pulmonary arterial hypertension: a placebo-controlled trial J Heart Lung Transplant 29 2010 137 149
62. V. McLaughlin, L. Rubin, and R.L. Benza Addition of inhaled treprostinil to oral therapy for pulmonary arterial hypertension: a randomized controlled clinical trial J Am Coll Cardiol 55 2010 1915 1922
63. V.F. Tapson, F. Torres, and F. Kermeen 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 142 2012 1383 1390
64. V.F. Tapson, Z.C. Jing, and K.F. Xu 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-C2 Study): a randomized controlled trial Chest 142 2013 1363 1364
65. Z.C. Jing, K. Parikh, and T. Pulido Efficacy and safety of oral treprostinil monotherapy for the treatment of pulmonary arterial hypertension: a randomized, controlled trial Circulation 127 2013 624 633
66. G. Simonneau, A. Torbicki, and M.M. Hoeper Selexipag, an oral, selective IP receptor agonist for the treatment of pulmonary arterial hypertension Eur Respir J 40 2012 874 880
67. N. Galie, M. Palazzini, and A. Manes Pulmonary arterial hypertension: from the kingdom of the near-dead to multiple clinical trial meta-analyses Eur Heart J 31 2010 2080 2086
68. N. Nickel, H. Golpon, and M. Greer The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension Eur Respir J 39 2012 589 596
69. K. Kemp, L. Savale, and D.S. O'Callaghan Usefulness of first-line combination therapy with epoprostenol and bosentan in pulmonary arterial hypertension: An observational study J Heart Lung Transplant 31 2012 150 158
70. A. Keogh, R.L. Benza, and P. Corris Interventional and surgical modalities of treatment in pulmonary arterial hypertension J Am Coll Cardiol 54 2009 S67 S77
71. O. Sitbon, M. Humbert, and H. Nunes Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival J Am Coll Cardiol 40 2002 780 788
72. V.V. McLaughlin, A. Shillington, and S. Rich Survival in primary pulmonary hypertension: the impact of epoprostenol therapy Circulation 106 2002 1477 1482
73. E.P. Trulock, L.B. Edwards, and D.O. Taylor Registry of the International Society for Heart and Lung Transplantation: twenty-third official adult lung and heart lung transplantation report - 2006 J Heart Lung Transplant 25 2006 880 892
74. Y. Toyoda, J. Thacker, and R. Santos Long-term outcome of lung and heart-lung transplantation for idiopathic pulmonary arterial hypertension Ann Thorac Surg 86 2008 1116 1122
75. E. Fadel, O. Mercier, and S. Mussot Long-term outcome of double-lung and heart-lung transplantation for pulmonary hypertension: a comparative retrospective study of 219 patients Eur J Cardiothorac Surg 38 2010 277 284
76. M. de Perrot, J.T. Granton, and K. McRae Outcome of patients with pulmonary arterial hypertension referred for lung transplantation: a 14-year single-center experience J Thorac Cardiovasc Surg 143 2012 910 918
77. J.D. Christie, L.B. Edwards, and A.Y. Kucheryavaya The Registry of the International Society for Heart and Lung Transplantation: 29th official adult lung and heart-lung transplant report - 2012 J Heart Lung Transplant 31 2012 1073 1086
78. T.K. Waddell, L. Bennett, R. Kennedy, T.R. Todd, and S.H. Keshavjee Heart-lung or lung transplantation for Eisenmenger syndrome J Heart Lung Transplant 21 2002 731 737
79. C.K. Choong, S.C. Sweet, and T.J. Guthrie Repair of congenital heart lesions combined with lung transplantation for the treatment of severe pulmonary hypertension: a 13-year experience J Thorac Cardiovasc Surg 129 2005 661 669
80. T. Fuehner, C. Kuehn, and J. Hadem Extracorporeal membrane oxygenation in awake patients as bridge to lung transplantation Am J Respir Crit Care Med 185 2012 763 768
81. J. Sandoval, J. Gaspar, and T. Pulido Graded balloon dilation atrial septostomy in severe primary pulmonary hypertension. A therapeutic alternative for patients nonresponsive to vasodilator treatment J Am Coll Cardiol 32 1998 297 304
82. M. Kurzyna, M. Dabrowski, and D. Bielecki Atrial septostomy in treatment of end-stage right heart failure in patients with pulmonary hypertension Chest 131 2007 977 983
83. T.F. Althoff, F. Knebel, and A. Panda Long-term follow-up of a fenestrated Amplatzer atrial septal occluder in pulmonary arterial hypertension Chest 133 2008 283 285
84. T.R. Fleming, and J.H. Powers Biomarkers and surrogate endpoints in clinical trials Statist Med 31 2012 2973 2984
85. T.R. Fleming Surrogate endpoints and FDA accelerated approval process Health Affairs 24 2005 67 78
86. G. Savarese, S. Paolillo, and P. Costanzo Do changes of 6-minute walk distance predict clinical events in patients with pulmonary arterial hypertension?: A meta-analysis of 22 randomized trials J Am Coll Cardiol 60 2012 1192 1201