肺高血圧症治療ガイドライン（2012年改訂版）
Guidelines for Treatment of Pulmonary Hypertension（JCS2012）
Guidelines for Treatment of Pulmonary Hypertension（JCS2012）
1. Barst RJ, Gibbs JSR, Ghofrani HA, et al. Updated evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Col Cardiol 2009; 54
(suppl): S78-84.
2. McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/ AHA 2009 expert consensus document on pulmonary hypertension: a report of the American
College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: developed in collaboration with
the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association. Circulation 2009; 119:
2250-2294.
3. Galie N, Hoeper MM, Humbert M, et al. 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: 2493-2537.
4. Task Force for Diagnosis and Treatment of Pulmonary Hypertension of European Society of Cardiology (ESC); European Respiratory Society (ERS);
International Society of Heart and Lung Transplantation (ISHLT), Galiè N, HoeperMM, Humbert M,et al: Guidelines for the diagnosis and treatment
of pulmonary hypertension. Euro Respir J 2009; 34: 1219-1263.
5. Badesch DB, Champion HC, Sanchez MA, et al. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol 2009; 54(1 Suppl):
S55-66.
6. Kovacs G, Berghold A, Scheidl S, et al. Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review. Eur Respir J
2009; 34: 888-894.
7. Simonneau G, Galiè N, Rubin LJ, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol 2004; 43(12 Suppl S): 5S-12S.
8. Rubin LJ. Diagnosis and management of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004; 126
(1 Suppl ): 7S-10S.
9. Simonneau G, Robbins IM, Beghetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009; 54(1 Suppl): S43-54.
10. 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: 81-84.
11. 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: 325-334.
12. 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.
13. Hachulla E, Gressin V, Guillevin L, et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective
multicenter study. Arthritis Rheum 2005; 52: 3792-3800.
14. 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: 1088-1093.
15. 吉田俊治，深谷修作．膠原病性肺高血圧症の頻度と病態 の解析．厚生労働科学研究費補助金免疫アレルギー疾患予防・治療研究事業 全身性自己免疫疾患
における難治性病態の診断と治療法に関する研究班平成15年度総括・分担研究報告書 2004: 40-43.
16. Sitbon O, Lascoux-Combe C, Delfraissy JF, et al. Prevalence of HIV-related pulmonary arterial hypertension in the current antiretroviral therapy era. Am
J Respir Crit CareMed 2008; 177: 108-113.
17. Krowka MJ, Swanson KL, Frantz RP, et al. Portopulmonary hypertension: Results from a 10-year screening algorithm.Hepatology 2006; 44: 1502-1510.
18. Hervé P, Lebrec D, Brenot F, et al. Pulmonary vascular disorders in portal hypertension.Eur Respir J 1998; 11: 1153- 1166.
19. Galie N, Manes A, Palazzini M, et al. Management of Pulmonary Arterial Hypertension Associated with Congenital Systemic-to-Pulmonary Shunts and
Eisenmenger’s Syndrome. Drugs 2008; 68: 1049-1066.
20. Chaves E. The pathology of the arterial pulmonary vasculature in Manson’s schistosomiasis. Chest 1966; 50: 72-77.
21. Montani D, Price LC, Dorfmuller P, et al. Pulmonary venoocclusive disease. Eur Respir J 2009; 33: 189-200.
22. Chaouat A, Bugnet AS, Kadaoui N, et al. Severe pulmonary hypertension and chronic obstructive pulmonary disease. Am J Respir Crit Care Med 2005;
172: 189-194.
23. 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- 870.
24. Williams MH, Handler CE, Akram R, et al. Role of N-terminal brain natriuretic peptide (N-TproBNP) in scleroderma-associated pulmonary arterial
hypertension. Eur Heart J 2006; 27: 1485-1494.
25. Fijalkowska A, Kurzyna M, Torbicki A, et al. Serum N-terminal brain natriuretic peptide as a prognostic parameter in patients with pulmonary
hypertension. Chest 2006; 129:1313-1321.
26. Andreassen AK, Wergeland R, Simonsen S, et al. N-terminal pro-B-type natriuretic peptide as an indicator of disease severity in a heterogeneous group
of patients with chronic precapillary pulmonary hypertension. Am J Cardiol2006; 98: 525-529.
27. Nagaya N, Uematsu M, Satoh T, et al. Serum uric acid levels correlate with the severity and the mortality of primary pulmonary hypertension. Am J
Respir Crit Care Med 1999;160: 487-492.
28. Voelkel MA, Wynne KM, Badech DB, et al. Hyperuricemia in severe pulmonary hypertension. Chest 2000; 117: 19-24.
29. Farber HW, Foreman AJ, Miller DP, et al. REVEAL Registry: correlation of right heart catheterization and echocardiography in patients with pulmonary
arterial hypertension. Congest Heart Fail 2011; 17: 56-64.
30. Raymond RJ, Hinderliter AL, Willis PW, et al. Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. J Am Coll Cardiol
2002; 39: 1214-1219.
31. Forfia PR, Fisher MR, Mathai SC, et al. Tricuspid annular displacement predicts survival in pulmonary hypertension. Am J Respir Crit Care Med 2006;
174: 1034-1041.
32. Yeo TC, Dujardin KS, Tei C, et al. Value of a Dopplerderived index combining systolic and diastolic time intervals in predicting outcome in primary
pulmonary hypertension. Am J Cardiol 1998; 81: 1157-1161.
33. Steen V, Medsger TA Jr. Predictors of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement. Arthritis
Rheum 2003; 48: 516-522.
34. Hoeper MM, Lee SH, Voswinckel R, et al. Complications of right heart catheterization procedures in patients with pulmonary hypertension in
experienced centers. J Am Coll Cardiol 2006; 48: 2546-2552.
35. Ranu H, Smith K, Nimako K, et al. A retrospective review to evaluate the safety of right heart catheterization via internal jugular vein in the assessment
of pulmonary hypertension. Clin Cardiol 2010; 33: 303-306.
36. Taylor CJ, Derrick G, McEwan A, et al. Risk of cardiac cathererization under anesthesia in children with pulmonary hypertension. Br J Anaesth 2007;
98: 657-661.
37. Sitbon O, Humbert M, Jais X, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005;
111: 3105-3111.
38. Hofmann LV, Lee DS, Gupta A, et al. Safety and hemodynamic effects of pulmonary angiography in patients with pulmonary hypertension: 10-year
single-center experience. Am J Roentgenol 2004; 183: 779-786.
39. Montani D, Achouh L, Dorfmuller P, et al. Pulmonary veno-occlusive disease: clinical, functional, radiologic, and hemodynamics and outcome of 24
cases confirmed by histology. Medicine 2008; 87: 220-233.
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hypertension. Comparison with cardiopulmonary exercise testing. Am J Respir Crit Care Med 2000; 161: 487-492.
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Am Coll Cardiol 2002; 40: 780-788.
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2. McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/ AHA 2009 expert consensus document on pulmonary hypertension: a report of the American
College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: developed in collaboration with
the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association. Circulation 2009; 119:
2250-2294.
3. Galie N, Hoeper MM, Humbert M, et al. 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: 2493-2537.
4. Task Force for Diagnosis and Treatment of Pulmonary Hypertension of European Society of Cardiology (ESC); European Respiratory Society (ERS);
International Society of Heart and Lung Transplantation (ISHLT), Galiè N, HoeperMM, Humbert M,et al: Guidelines for the diagnosis and treatment
of pulmonary hypertension. Euro Respir J 2009; 34: 1219-1263.
5. Badesch DB, Champion HC, Sanchez MA, et al. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol 2009; 54(1 Suppl):
S55-66.
6. Kovacs G, Berghold A, Scheidl S, et al. Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review. Eur Respir J
2009; 34: 888-894.
7. Simonneau G, Galiè N, Rubin LJ, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol 2004; 43(12 Suppl S): 5S-12S.
8. Rubin LJ. Diagnosis and management of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004; 126
(1 Suppl ): 7S-10S.
9. Simonneau G, Robbins IM, Beghetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009; 54(1 Suppl): S43-54.
10. 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: 81-84.
11. 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: 325-334.
12. 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.
13. Hachulla E, Gressin V, Guillevin L, et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective
multicenter study. Arthritis Rheum 2005; 52: 3792-3800.
14. 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: 1088-1093.
15. 吉田俊治，深谷修作．膠原病性肺高血圧症の頻度と病態 の解析．厚生労働科学研究費補助金免疫アレルギー疾患予防・治療研究事業 全身性自己免疫疾患
における難治性病態の診断と治療法に関する研究班平成15年度総括・分担研究報告書 2004: 40-43.
16. Sitbon O, Lascoux-Combe C, Delfraissy JF, et al. Prevalence of HIV-related pulmonary arterial hypertension in the current antiretroviral therapy era. Am
J Respir Crit CareMed 2008; 177: 108-113.
17. Krowka MJ, Swanson KL, Frantz RP, et al. Portopulmonary hypertension: Results from a 10-year screening algorithm.Hepatology 2006; 44: 1502-1510.
18. Hervé P, Lebrec D, Brenot F, et al. Pulmonary vascular disorders in portal hypertension.Eur Respir J 1998; 11: 1153- 1166.
19. Galie N, Manes A, Palazzini M, et al. Management of Pulmonary Arterial Hypertension Associated with Congenital Systemic-to-Pulmonary Shunts and
Eisenmenger’s Syndrome. Drugs 2008; 68: 1049-1066.
20. Chaves E. The pathology of the arterial pulmonary vasculature in Manson’s schistosomiasis. Chest 1966; 50: 72-77.
21. Montani D, Price LC, Dorfmuller P, et al. Pulmonary venoocclusive disease. Eur Respir J 2009; 33: 189-200.
22. Chaouat A, Bugnet AS, Kadaoui N, et al. Severe pulmonary hypertension and chronic obstructive pulmonary disease. Am J Respir Crit Care Med 2005;
172: 189-194.
23. 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- 870.
24. Williams MH, Handler CE, Akram R, et al. Role of N-terminal brain natriuretic peptide (N-TproBNP) in scleroderma-associated pulmonary arterial
hypertension. Eur Heart J 2006; 27: 1485-1494.
25. Fijalkowska A, Kurzyna M, Torbicki A, et al. Serum N-terminal brain natriuretic peptide as a prognostic parameter in patients with pulmonary
hypertension. Chest 2006; 129:1313-1321.
26. Andreassen AK, Wergeland R, Simonsen S, et al. N-terminal pro-B-type natriuretic peptide as an indicator of disease severity in a heterogeneous group
of patients with chronic precapillary pulmonary hypertension. Am J Cardiol2006; 98: 525-529.
27. Nagaya N, Uematsu M, Satoh T, et al. Serum uric acid levels correlate with the severity and the mortality of primary pulmonary hypertension. Am J
Respir Crit Care Med 1999;160: 487-492.
28. Voelkel MA, Wynne KM, Badech DB, et al. Hyperuricemia in severe pulmonary hypertension. Chest 2000; 117: 19-24.
29. Farber HW, Foreman AJ, Miller DP, et al. REVEAL Registry: correlation of right heart catheterization and echocardiography in patients with pulmonary
arterial hypertension. Congest Heart Fail 2011; 17: 56-64.
30. Raymond RJ, Hinderliter AL, Willis PW, et al. Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. J Am Coll Cardiol
2002; 39: 1214-1219.
31. Forfia PR, Fisher MR, Mathai SC, et al. Tricuspid annular displacement predicts survival in pulmonary hypertension. Am J Respir Crit Care Med 2006;
174: 1034-1041.
32. Yeo TC, Dujardin KS, Tei C, et al. Value of a Dopplerderived index combining systolic and diastolic time intervals in predicting outcome in primary
pulmonary hypertension. Am J Cardiol 1998; 81: 1157-1161.
33. Steen V, Medsger TA Jr. Predictors of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement. Arthritis
Rheum 2003; 48: 516-522.
34. Hoeper MM, Lee SH, Voswinckel R, et al. Complications of right heart catheterization procedures in patients with pulmonary hypertension in
experienced centers. J Am Coll Cardiol 2006; 48: 2546-2552.
35. Ranu H, Smith K, Nimako K, et al. A retrospective review to evaluate the safety of right heart catheterization via internal jugular vein in the assessment
of pulmonary hypertension. Clin Cardiol 2010; 33: 303-306.
36. Taylor CJ, Derrick G, McEwan A, et al. Risk of cardiac cathererization under anesthesia in children with pulmonary hypertension. Br J Anaesth 2007;
98: 657-661.
37. Sitbon O, Humbert M, Jais X, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005;
111: 3105-3111.
38. Hofmann LV, Lee DS, Gupta A, et al. Safety and hemodynamic effects of pulmonary angiography in patients with pulmonary hypertension: 10-year
single-center experience. Am J Roentgenol 2004; 183: 779-786.
39. Montani D, Achouh L, Dorfmuller P, et al. Pulmonary veno-occlusive disease: clinical, functional, radiologic, and hemodynamics and outcome of 24
cases confirmed by histology. Medicine 2008; 87: 220-233.
40. 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: 487-492.
41. 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.
42. 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: 647-652.
43. 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: 319-324.
44. Oudiz RJ, Barst RJ, Hansen JE, et al. Cardiopulmonary exercise testing and six-minute walk correlations in pulmonary arterial hypertension. Am J
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