Integrated care and optimal management of pulmonary arterial hypertension

Journal of Multidisciplinary Healthcare

Volumn 2, Issue , 2009, Pages 67-78

  Strange, Geoff ^a   Fowler, Robin ^b   Jary, Corina ^b   Dalton, Brad ^c   Stewart, Simon ^d   Gabbay, Eli ^e  

^a MONASH UNIVERSITY   (Australia)

^b ROYAL PERTH HOSPITAL   (Australia)

^c UNIVERSITY OF TASMANIA   (Australia)

^d BAKER IDI HEART AND DIABETES INSTITUTE   (Australia)

^e UNIVERSITY OF WESTERN AUSTRALIA   (Australia)

Author keywords

Diagnostic protocol; Multidisciplinary care; Pulmonary hypertension; Systemic sclerosis

Indexed keywords

AMBRISENTAN; BERAPROST; BOSENTAN; CALCIUM CHANNEL BLOCKING AGENT; DIGOXIN; DIURETIC AGENT; ILOPROST; PLACEBO; PROSTACYCLIN; SILDENAFIL; SITAXSENTAN; TREPROSTINIL; WARFARIN;

ADD ON THERAPY; CONTINUOUS INFUSION; DIAGNOSTIC TEST; DISEASE CLASSIFICATION; DOPPLER ECHOCARDIOGRAPHY; DRUG EFFICACY; ELECTROCARDIOGRAM; EXERCISE; HEART CATHETERIZATION; HEART FAILURE; HEART LUNG TRANSPLANTATION; HUMAN; LUNG FUNCTION TEST; LUNG TRANSPLANTATION; MORBIDITY; PATHOPHYSIOLOGY; PHYSICAL EXAMINATION; PULMONARY HYPERTENSION; REVIEW; SCREENING; THORAX RADIOGRAPHY;

EID: 85009776041     PISSN: None     EISSN: 11782390     Source Type: Journal    
DOI: 10.2147/JMDH.S3085     Document Type: Review

References (85)

1. Shafazand S, Goldstein MK, Doyle RL, Hlatky MA, Gould MK. Health-related quality of life in patients with pulmonary arterial hypertension. Chest. 2004;126:1452–1459.
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;115:343–349.
3. McLaughlin VV, Sitbon O, Badesch DB, et al. Survival with first-line bosentan in patients with primary pulmonary hypertension. Eur Respir J. 2005;25:244–249.
4. Williams MH, Das C, Handler CE, et al. Systemic sclerosis associated pulmonary hypertension: improved survival in the current era. Heart. 2006;92:926–932.
5. Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med. 1987;107:216–223.
6. Simonneau G, Galie N, Rubin LJ, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004;43:5S–12S.
7. Galie N, Torbicki A, Barst R, et al. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology. Eur Heart J. 2004;25:2243–2278.
8. Rubin LJ. Diagnosis and management of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004;126:7S–10S.
9. Runo JR, Loyd JE. Primary pulmonary hypertension. Lancet. 2003;361:1533–1544.
10. Rich S. Executive summary from the world symposium on primary pulmonary hypertension. Available at http://www.who.int/ncd/cvd/pph.htm. 1998.
11. Magliano M, Isenberg DA, Hillson J. Pulmonary hypertension in autoimmune rheumatic diseases: where are we now? Arthritis Rheum. 2002;46:1997–2009.
12. Fuster V, Steele PM, Edwards WD, Gersh BJ, McGoon MD, Frye RL. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation. 1984;70:580–587.
13. Hoeper MM, Galie N, Simonneau G, Rubin LJ. New treatments for pulmonary arterial hypertension. Am J Respir Crit Care Med. 2002;165:1209–1216.
14. Kim NH, Rubin LJ. Endothelin in health and disease: endothelin receptor antagonists in the management of pulmonary artery hypertension. J Cardiovasc Pharmacol Ther. 2002;7:9–19.
15. Rubin LJ, Roux S. Bosentan: a dual endothelin receptor antagonist. Expert Opin Investig Drugs. 2002;11:991–1002.
16. Roux S, Breu V, Ertel SI, Clozel M. Endothelin antagonism with bosentan: a review of potential applications. J Mol Med. 1999;77:364–376.
17. Humbert M, Morrell NW, Archer SL, et al. Cellular and molecular pathobiology of pulmonary arterial hypertension. J Am Coll Cardiol. 2004;43:13S–24S.
18. Barst RJ. PDGF signaling in pulmonary arterial hypertension. J Clin Invest. 2005;115:2691–2694.
19. McGoon M, Gutterman D, Steen V, et al. Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004;126:14S–34S.
20. Proudman SM, Stevens WM, Sahhar J, Celermajer D. Pulmonary arterial hypertension in systemic sclerosis: the need for early detection and treatment. Intern Med J. 2007;37:485–494.
21. Hachulla E, Coghlan JG. A new era in the management of pulmonary arterial hypertension related to scleroderma: endothelin receptor antagonism. Ann Rheum Dis. 2004;63:1009–1014.
22. 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;173:1023–1030.
23. Peacock AJ, Murphy NF, McMurray JJ, Caballero L, Stewart S. An epidemiological study of pulmonary arterial hypertension. Eur Respir J. 2007;30:104–109.
24. Rubin LJ. Primary pulmonary hypertension. N Engl J Med. 1997;336:111–117.
25. Gaine SP, Rubin LJ. Primary pulmonary hypertension. Lancet. 1998;352:719–725.
26. Galie N, Manes A, Uguccioni L, et al. Primary pulmonary hypertension: insights into pathogenesis from epidemiology. Chest. 1998;114: 184S–194S.
27. Battle RW, Davitt MA, Cooper SM, et al. Prevalence of pulmonary hypertension in limited and diffuse scleroderma. Chest. 1996;110:1515–1519.
28. Koh ET, Lee P, Gladman DD, Abu-Shakra M. Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients. Br J Rheumatol. 1996;35:989–993.
29. MacGregor AJ, Canavan R, Knight C, et al. Pulmonary hypertension in systemic sclerosis: risk factors for progression and consequences for survival. Rheumatology (Oxford). 2001;40:453–459.
30. Pope JE, Lee P, Baron M, et al. Prevalence of elevated pulmonary arterial pressures measured by echocardiography in a multicenter study of patients with systemic sclerosis. J Rheumatol. 2005;32:1273–1278.
31. Schachna L, Wigley FM, Chang B, White B, Wise RA, Gelber AC. Age and risk of pulmonary arterial hypertension in scleroderma. Chest. 2003;124:2098–2104.
32. Gabbay E, Yeow W, Playford D. Pulmonary arterial hypertension is an uncommon cause of pulmonary hypertension in an unselected population: The Armadale echocardiography study. Proceedings of the American Thoracic Society Annual Meeting; 2007 May 18–23; San Francisco, California.
33. Consensus statement on the management of pulmonary hypertension in clinical practice in the UK and Ireland. Heart. 2008;94 Suppl 1:i1–41.
34. Nauser TD, Stites SW. Diagnosis and treatment of pulmonary hypertension. Am Fam Physician. 2001;63:1789–1798.
35. Barst RJ, McGoon M, Torbicki A, et al. Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2004;43:40S–47S.
36. Borgeson DD, Seward JB, Miller FA, Jr, Oh JK, Tajik AJ. Frequency of Doppler measurable pulmonary artery pressures. J Am Soc Echocardiogr. 1996;9:832–837.
37. Barst RJ, editor. Pulmonary arterial hypertension: Evidence-based treatment. Wiltshire: Wiley; 2008.
38. Phung S, Strange G, Chung LP, et al. Prevalence of pulmonary arterial hypertension in an Australian scleroderma population: Screening allows for earlier diagnosis. Intern Med J. 2008.
39. Badesch DB, Abman SH, Ahearn GS, et al. Medical therapy for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004;126:35S–62S.
40. Doyle RL, McCrory D, Channick RN, Simonneau G, Conte J. Surgical treatments/interventions for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004;126:63S–71S.
41. McLaughlin VV, Presberg KW, Doyle RL, et al. Prognosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004;126:78S–92S.
42. Ogata M, Ohe M, Shirato K, Takishima T. Effects of a combination therapy of anticoagulant and vasodilator on the long-term prognosis of primary pulmonary hypertension. Jpn Circ J. 1993;57:63–69.
43. Rich S, Kaufmann E, Levy PS. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med. 1992;327:76–81.
44. Barst RJ, Rubin LJ, Long WA, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group. N Engl J Med. 1996;334:296–302.
45. Stewart S, Strange G, editors. A clinician’s guide to pulmonary arterial hypertension. Second edition: Informa Healthcare; 2008.
46. Galie N, Manes A, Branzi A. The endothelin system in pulmonary arterial hypertension. Cardiovasc Res. 2004;61:227–237.
47. Stewart DJ, Levy RD, Cernacek P, Langleben D. Increased plasma endothelin-1 in pulmonary hypertension: marker or mediator of disease? Ann Intern Med. 1991;114:464–469.
48. Channick RN, Simonneau G, Sitbon O, et al. Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study. Lancet. 2001;358:1119–1123.
49. Jais X, D’Armini AM, Jansa P, et al. Bosentan for treatment of inoperable chronic thromboembolic pulmonary hypertension: BENEFiT (Bosentan Effects in iNopErable Forms of chronIc Thromboembolic pulmonary hypertension), a randomized, placebo-controlled trial. J Am Coll Cardiol. 2008;52:2127–2134.
50. Gatzoulis MA, Beghetti M, Galie N, et al. Longer-term bosentan therapy improves functional capacity in Eisenmenger syndrome: results of the BREATHE-5 open-label extension study. Int J Cardiol. 2008;127:27–32.
51. Galie N, Rubin L, Hoeper M, et al. Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial. Lancet. 2008;371:2093–2100.
52. Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med. 2002;346:896–903.
53. 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;73:372–382.
54. Sitbon O, Gressin V, Speich R, et al. Bosentan for the treatment of human immunodeficiency virus-associated pulmonary arterial hypertension. Am J Respir Crit Care Med. 2004;170:1212–1217.
55. Denton CP, Pope JE, Peter HH, et al. Long-term effects of bosentan on quality of life, survival, safety and tolerability in pulmonary arterial hypertension related to connective tissue diseases. Ann Rheum Dis. 2008;67:1222–1228.
56. 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;41:1380–1386.
57. Humbert M, Segal ES, Kiely DG, Carlsen J, Schwierin B, Hoeper MM. Results of European post-marketing surveillance of bosentan in pulmonary hypertension. Eur Respir J. 2007;30:338–344.
58. Barst RJ, Langleben D, Badesch D, et al. Treatment of pulmonary arterial hypertension with the selective endothelin-A receptor antagonist sitaxsentan. J Am Coll Cardiol. 2006;47:2049–2056.
59. Barst RJ, Langleben D, Frost A, et al. Sitaxsentan therapy for pulmonary arterial hypertension. Am J Respir Crit Care Med. 2004;169: 441–447.
60. Benza RL, Barst RJ, Galie N, et al. Sitaxsentan for the treatment of pulmonary arterial hypertension: a 1-year, prospective, open-label observation of outcome and survival. Chest. 2008;134:775–782.
61. Galie N, Badesch D, Oudiz R, et al. Ambrisentan therapy for pulmonary arterial hypertension. J Am Coll Cardiol. 2005;46:529–535.
62. Barst RJ, Rubin LJ, McGoon MD, Caldwell EJ, Long WA, Levy PS. Survival in primary pulmonary hypertension with long-term continuous intravenous prostacyclin. Ann Intern Med. 1994;121:409–415.
63. McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation. 2002;106:1477–1482.
64. Sitbon O, Humbert M, Nunes H, et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol. 2002;40:780–788.
65. Galie N, Ghofrani HA, Torbicki A, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med. 2005;353: 2148–2157.
66. McLaughlin VV, Gaine SP, Barst RJ, et al. Efficacy and safety of treprostinil: an epoprostenol analog for primary pulmonary hypertension. J Cardiovasc Pharmacol. 2003;41:293–299.
67. Provencher S, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension with bosentan: from pathophysiology to clinical evidence. Expert Opin Pharmacother. 2005;6:1337–1348.
68. Hoeper MM, Markevych I, Spiekerkoetter E, Welte T, Niedermeyer J. Goal-oriented treatment and combination therapy for pulmonary arterial hypertension. Eur Respir J. 2005;26:858–863.
69. Opitz CF, Wensel R, Winkler J, et al. Clinical efficacy and survival with first-line inhaled iloprost therapy in patients with idiopathic pulmonary arterial hypertension. Eur Heart J. 2005;26:1895–1902.
70. Simonneau G, Rubin LJ, Galie N, et al. Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial. Ann Intern Med. 2008;149:521–530.
71. Recommendations on the management of pulmonary hypertension in clinical practice. Heart. 2001;86 Suppl 1:I1–13.
72. Sandoval J, Rothman A, Pulido T. Atrial septostomy for pulmonary hypertension. Clin Chest Med. 2001;22:547–560.
73. Taichman DB, Shin J, Hud L, et al. Health-related quality of life in patients with pulmonary arterial hypertension. Respir Res. 2005;6:92.
74. Archibald CJ, Auger WR, Fedullo PF, et al. Long-term outcome after pulmonary thromboendarterectomy. Am J Respir Crit Care Med. 1999;160:523–528.
75. Mikhail GW, Prasad SK, Li W, et al. Clinical and haemodynamic effects of sildenafil in pulmonary hypertension: acute and mid-term effects. Eur Heart J. 2004;25:431–436.
76. Olschewski H, Simonneau G, Galie N, et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med. 2002;347:322–329.
77. Sastry BK, Narasimhan C, Reddy NK, Raju BS. Clinical efficacy of sildenafil in primary pulmonary hypertension: a randomized, placebo-controlled, double-blind, crossover study. J Am Coll Cardiol. 2004;43:1149–1153.
78. 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;165:800–804.
79. Souza R, Jardim C, Martins B, et al. Effect of bosentan treatment on surrogate markers in pulmonary arterial hypertension. Curr Med Res Opin. 2005;21:907–911.
80. Strange G, Gabbay E, Williams T, Wlodarczyk J, McNeil K, Keogh A. Bosentan therapy in patients with pulmonary arterial hypertension: the relationship between improvements in six-minute walk test and quality of life. Respirology. 2008;13:674–682.
81. Mereles D, Ehlken N, Kreuscher S, et al. Exercise and respiratory training improve exercise capacity and quality of life in patients with severe chronic pulmonary hypertension. Circulation. 2006;114:1482–1489.
82. McDonald K, Ledwidge M, Cahill J, et al. Heart failure management: multidisciplinary care has intrinsic benefit above the optimization of medical care. J Card Fail. 2002;8:142–148.
83. Organised inpatient (stroke unit) care for stroke. Stroke Unit Trialists’ Collaboration. Cochrane Database Syst Rev. 2000:CD000197.
84. Hankey GJ, Warlow CP. Treatment and secondary prevention of stroke: evidence, costs, and effects on individuals and populations. Lancet. 1999;354:1457–1463.
85. August DA, Carpenter LC, Harness JK, et al. Benefits of a multidisciplinary approach to breast care. J Surg Oncol. 1993;53:161–167.