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）
文献
201. Polin RA, Spitzer AR. Fetal & neonatal secrets 2nd edn. Philadelphia, Elsevier Mosby 2001: 413-416.
202. Doyle RL, McCrory D, Channick RN, et al. Surgical treatments / interventions for pulmonary arterial hypertension: ACCP evidence-based clinical
practice guidelines. Chest 2004; 126: 63S-71S.
203. Glanville AR, Burke CM, Theodore J, et al. Primary pulmonary hypertension. Length of survival in patients referred for heart-lung transplantation. Chest
1987; 91: 675-681.
204. Christie JD, Edwards LB, Kucheryavaya AY, et al. The Registry of the International Society for Heart and Lung Transplantation: twenty-seventh official
adult lung and heartlung transplant report--2010. J Heart Lung Transplant 2010; 29: 1104-1118.
205. Bando T, Date H, Minami M, et al. First registry report: lung transplantation in Japan: The Japanese Society of Lung and Heart-Lung Transplantation.
Gen Thorac Cardiovasc Surg. 2008; 56: 17-21.
206. Bando K, Armitage JM, Paradis IL, et al. Indications for and results of single, bilateral, and heart-lung transplantation for pulmonary hypertension. J
Thorac Cardiovasc Surg 1994; 108: 1056-1065.
207. Waddell TK, et al. Heart-lung or lung transplantation for Eisenmenger syndrome. J Heart Lung Transplant 2002; 21:731-737.
208. Pigula FA, et al. Cardiopulmonary transplantation for congenital heart disease in the adult. J Heart Lung Transplant 2001; 20: 297-303.
209. Reichart B, et al. Improved results after heart-lung transplantation: a 17-year experience. Transplantation 2003; 75: 127-132.
210. Spray TL, et al. Pediatric lung transplantation for pulmonary hypertension and congenital heart disease. Ann Thorac Surg 1992; 54: 216-223.
211. Choong CK, et al. Repair of congenital heart lesions combined with lung transplantation for the treatment of severe pulmonary hypertension: a 13-year
experience. J Thorac Cardiovasc Surg 2005; 129: 661-669.
212. Trulock EP, et al. International Society for Heart and Lung Transplantation. 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 2006; 25: 880-892.
213. Keogh A, et al. Interventional and surgical modalities of treatment in pulmonary arterial hypertension. J Am Coll Cardiol 2009; 54: S67-S77.
214. Daliento L, et al. Eisenmenger syndrome. Factors relating to deterioration and death. Eur Heart J 1998; 19: 1845-1855.
215. Maurer JR, Frost AE, Estenne M, et al. International guidelines for the selection of lung transplant candidates. The International Society for Heart and
Lung Transplantation, the American Thoracic Society, the American Society of Transplant Physicians, the European Respiratory Society. J
Heart Lung Transplant 1998. 17: 703-709.
216. Guglin M, Khan H. Pulmonary hypertension in heart failure. J Card Fail 2010; 16: 461-474.
217. Abramson SV, Burke JF, Kelly JJ Jr, et al. Pulmonary hypertension predicts mortality and morbidity in patients with dilated cardiomyopathy. Ann
Intern Med 1992; 11: 888-895.
218. Ghio S, Gavazzi A, Campana C, et al. Independent and additive prognostic value of right ventricular systolic function and pulmonary artery pressure in
patients with chronic heart failure. J Am Coll Cardiol 2001; 1: 183-188.
219. Lam CS, Roger VL, Rodeheffer RJ, et al. Pulmonary hypertension in heart failure with preserved ejection fraction: a communitybased study. J Am Coll
Cardiol 2009; 13: 1119- 1126.
220. Leung CC, V Moondra, Catherwood E, et al. Prevalence and risk factors of pulmonary hypertension in patients with elevated pulmonary venous
pressure and preserved ejection fraction. Am J Cardiol 2010; 106: 284-286.
221. Kjaergaard J, Akkan D, Iversen KK, et al. Prognostic importance of pulmonary hypertension in patients with heart failure. Am J Cardiol 2007; 8: 1146-
1150.
222. Butler J, Chomsky DB, Wilson JR. Pulmonary hypertension and exercise intolerance in patients with heart failure. J Am Coll Cardiol 1999; 34: 1802-
1806.
223. Costard-Jäckle A, Fowler MB. Influence of preoperative pulmonary artery pressure on mortality after heart transplantation: testing of potential
reversibility of pulmonary hypertension with nitroprusside is useful in defining a high risk group. J Am Coll Cardiol 1992; 19: 48-54.
224. Abramson SV, Burke JF, Kelly JJ Jr, et al. Pulmonary hypertension predicts mortality and morbidity in patients with dilated cardiomyopathy. Ann
Intern Med 1992; 116: 888-895.
225. Erickson KW, Costanzo-Nordin MR, O’Sullivan EJ, et al. Influence of preoperative transpulmonary gradient on late mortality after orthotopic heart
transplantation. J Heart Transplant 1990; 9: 526-637.
226. Chen JM, Levin HR, Michler RE, et al. Reevaluating the significance of pulmonary hypertension before cardiac transplantation: determination of
optimal thresholds and quantification of the effect of reversibility on perioperative mortality. J Thorac Cardiovasc Surg 1997; 114: 627-634.
227. Delgado JF, Gomez-Sanchez MA, Saenz de la Calzada C, et al. Impact of mild pulmonary hypertension on mortality and pulmonary artery pressure
profile after heart transplantation. J Heart Lung Transplant 2001; 20: 942-928.
228. Chang PP, Longenecker JC, Wang NY, et al. Mild vs severe pulmonary hypertension before heart transplantation: different effects on
osttransplantation pulmonary hypertension and mortality. J Heart Lung Transplant 2005; 24: 998-1007.
229. Alfonso F, Macaya C, Hernandez R, et al. Percutaneous mitral valvuloplasty with severe pulmonary artery hypertension. Am J Cardiol 1993; 72: 325-
330.
230. Fawzy ME, Mimish L, Sivanandam V, et al. Immediate and long-term effect of mitral balloon valvotomy on severe pulmonary hypertension in patients
with mitral stenosis. Am Heart J 1996; 131: 89-93.
231. Zenner JC, Hancock EW, Shumway NE, et al. Regression of extreme pulmonary hypertension after mitral valve surgery. Am. J Cardiol 1972; 30: 820-
826.
232. Ward C, Hancock BW. Extreme Pulmonary hypertension caused by mitral valve disease. Natural history and results of surgery. Br Heart 1975;
37: 74- 78.
233. Vincens JJ, Temizer D, Post JR, et al. Long-term outcome of cardiac surgery in patients with mitral stenosis and severe pulmonary hypertension.
Circulation 1995; 92(9 Suppl): II137-142.
234. Tandon HD, Kasturi J. Pulmonary vascular changes associated with isolated mitral stenosis in India. Br Heart J 1975; 37: 26-36.
235. Goodale W, Sanchez G, Friedlich AL, et al. Correlation of pulmonary arteriolar resistance with pulmonary vascular changes in patients with mitral
stenosis before and aftervalvulotomy. N Engl J Med 1955; 252: 979-983.
236. Califf RM, Adams KF, McKenna WJ, et al. A randomized controlled trial of epoprostenol therapy for severe congestive heart failure: The Flolan
International Randomized Survival Trial (FIRST). Am Heart J 1997; 134: 44-54.
237. Mylona P, Cleland JG. Update of REACH-1 and MERIT HF clinical trials in heart failure. Cardio. net Editorial Team. Eur J Heart Fail 1999; 1: 197-200.
238. Packer M, McMurray J, Massie BM, et al. Clinical effects of endothelin receptor antagonism with bosentan in patients with severe chronic heart
failure: results of a pilot study. J Card Fail 2005; 11: 12-20.
239. Kalra PR, Moon JC, Coats AJ. Do results of the ENABLE (Endothelin Antagonist Bosentan for Lowering Cardiac Events in Heart Failure) study spell
the end for non-selective endothelin antagonism in heart failure? Int J Cardiol 2002; 85: 195-197.
240. Torre-Amione G, Young JB, Colucci WS, et al. Hemodynamic and clinical effects of tezosentan, an intravenous dual endothelin receptor antagonist, in
patients hospitalized for acute decompensated heart failure. J Am Coll Cardiol 2003; 42: 140-147.
241. Anand I, McMurray J, Cohn JN et al. Long-term effects of darusentan on left-ventricular remodelling and clinical outcomes in the EndothelinA Receptor
Antagonist Trial in Heart Failure (EARTH): randomised, double-blind, placebocontrolled trial. Lancet 2004; 364: 347-354.
242. Lewis GD, Shah R, Shahzad K, et al. Sildenafil improves exercise capacity and quality of life in patients with systolic heart failure and secondary
pulmonary hypertension. Circulation 2007; 116: 1555-1562.
243. Behling A, Rohde LE, Colombo FC, et al. Effects of 5’-phosphodiesterase four-week long inhibition with sildenafil in patients with chronic heart failure:
a double-blind, placebocontrolled clinical trial. J Card Fail 2008; 14: 189-197.
244. Guazzi M, Vicenzi M, Arena R, et al. PDE5 Inhibition With Sildenafil Improves Left Ventricular Diastolic Function, Cardiac Geometry, and Clinical
Status in Patients With Stable Systolic Heart Failure. Results of a 1-Year, Prospective, Randomized, Placebo-Controlled Study. Circ Heart Fail
2011; 4: 8-17.
245. Elliott CG, Palevsky HI. Treatment with epoprostenol of pulmonary arterial hypertension following mitral valve replacement for mitral stenosis. Thorax
2004; 59: 536-537.
246. Trachte AL, MD, Lobato EB, Urdaneta F, et al. Oral Sildenafil Reduces Pulmonary Hypertension After Cardiac Surgery. Ann Thorac Surg 2005; 79:
194 -197.
247. 日本呼吸器学会COPDガイドライン第3版作成委員会.COPD（慢性閉塞性肺疾患）診断と治療のためのガイドライン．第3版．東京，日本呼吸器学会 2009．
248. Barberà JA, Peinado VI, Santos S. Pulmonary hypertension in chronic obstructive pulmonary disease. Eur Respir J 2003;21: 892-905.
249. Santos S, Peinado VI, Ramírez J, et al. Characterization of pulmonary vascular remodelling in smokers and patients with mild COPD. Eur Respir J
2002; 19: 632-638.
250. 平賀俊尚，岸不盡彌，川上義和，他．慢性呼吸不全患者 における低酸素血症の肺循環に及ぼす影響 －動脈血酸素分圧・基礎疾患別検討－．昭和62年,
厚生省特定疾患呼吸不全調査研究班（編）：昭和62年度研究報告書. 135-140.
251. Stevens D, Sharma K, Szidon P, et al. Severe pulmonary hypertension associated with COPD. Ann Transplant 2000; 5:8-12.
252. Scharf SM, Iqbal M, Keller C, et al. Hemodynamic characterization of patients with severe emphysema. Am J Respir Crit Care Med 2002; 166: 314-
322.
253. Incalzi RA, Fuso L, De Rosa M, et al. Electrocardiographic signs of chronic cor pulmonale: A negative prognostic finding in chronic obstructive
pulmonary disease. Circulation 1999;99: 1600-6105.
254. Burrows B, et al. Patterns of cardiovascular dysfunction in chronic obstructive lung disease. N Engl J Med 1972; 286: 912-918.
255. Fletcher E.C and Levin DC. Cardiopulmona ry hemodynamics during sleep in subjects with chronic obstructive pulmonary disease. The effect of
short- and longterm oxygen. Chest 1984; 85: 6-14.
256. Zenker G, Forche G, Harnoncourt K. Two-dimensional echocardiography using a subcostal approach in patients with COPD. Chest 1985; 88: 722-725.
257. Marcus JT, Vonk Noordegraaf A, De Vries PM, et al. MRI evaluation of right ventricular pressure overload in chronic obstructive pulmonary disease. J
Magn Reson Imaging 1998; 8: 999-1005.
258. Yamaoka S, Yonekura Y, Koide H, et al. Noninvasive method to assess cor pulmonale in patients with chronic obstructive pulmonary disease Chest
1987; 92: 10-17.
259. Morrison DA, Pulmonary hypertension in chronic obstructive pulmonary disease: the right ventricular hypothesis. Chest 1987; 92: 387-389.
260. Oswald-Mammosser M, Weitzenblum E, Quoix E, et al. Prognostic factors in COPD patients receiving long-term oxygen therapy. Importance of
pulmonary artery pressure. Chest 1995; 107: 1193-1198.
261. Kessler R, Faller M, Fourgaut G, et al. Predictive factors of hospitalization for acute exacerbation in a series of 64 patients with chronic obstructive
pulmonary disease. Am J Respir Crit Care Med 1999; 159: 158-164.
262. Minai OA, Chaouat A, Adnot S. Pulmonary hypertension in COPD: epidemiology, significance, and management: pulmonary vascular disease: the
global perspective. Chest 2010; 137(Suppl): 39S-51S.
263. Wells JM, Washko GR, Han MK, et al. Pulmonary arterial enlargement and acute exacerbations of COPD. N Engl J Med 2012; 367: 913-921.
264. No authors listed. Long term domiciliary oxygen therapy in chronic hypoxic cor pulmonale complicating chronic bronchitis and emphysema. Report of
the Medical Research Council Working Party. Lancet 1981;1: 681-686.
265. No authors listed. Continuous or nocturnal oxygen therapy in hypoxemic chronic obstructive lung disease: a clinical trial. Nocturnal Oxygen Therapy
Trial Group. Ann Intern Med 1980; 93: 391-398.
266. Krop HD, Block AJ, Cohen E.. Neuropsychologic effects of continuous oxygen therapy in chronic obstructive pulmonary disease. Chest 1973;
64: 317- 322.
267. Weitzenblum E, Sautegeau A, Ehrhart M, et al. Long-term oxygen therapy can reverse the progression of pulmonary hypertension in patients with
chronic obstructive pulmonary disease. Am Rev Respir Dis 1985; 131: 493-498.
268. Chaouat A, Weitzenblum E, Kessler R, et al. A randomized trial of nocturnal oxygen therapy in chronic obstructive pulmonary disease patients.
Eur Respir J 1999; 14: 1002-1008.
269. Blanco I, Gimeno E, Munoz PA, et al. Hemodynamic and gas exchange effects of sildenafil in patients with chronic obstructive pulmonary disease
and pulmonary hypertension. Am J Respir Crit Care Med 2010; 181: 270-278.
270. Barberà JA, Roger N, Roca J, et al. Worsening of pulmonary gas exchange with nitric oxide inhalation in chronic obstructive pulmonary disease.
Lancet 1996; 347:436-440.
271. Ashutosh K, Phadke K, Jackson JF, et al. Use of nitric oxide inhalation in chronic obstructive pulmonary disease. Thorax 2000 ; 55: 109-113.
272. Vonbank K, Ziesche R, Higenbottam TW, et al. Controlled prospective randomised trial on the effects on pulmonary haemodynamics of the
ambulatory long term use of nitric oxide and oxygen in patients with severe COPD. Thorax 2003; 58: 289-293.
273. Hurdman J, Condliffe R, Elliot CA, et al. Pulmonary hypertension in COPD: results from the ASPIRE Registry. Eur Respir J 2012 Sep 27.
[Epub ahead of print]
274. Criner GJ, Scharf SM, Falk JA., et al. Effect of lung volume reduction surgery on resting pulmonary hemodynamics in severe emphysema.
Am J Respir Crit Care Med 2007; 176: 253-260.
275. 日本呼吸器学会COPDガイドライン第3版作成委員会.COPD（慢性閉塞性肺疾患）診断と治療のためのガイドライン 第3版. 2009, 東京: 日本呼吸器学会.
276. Rogers TK, Howard P. Howard, Pulmonary hemodynamics and physical training in patients with chronic obstructive pulmonary disease. Chest 1992;
101(5 Suppl): 289S-292S.
277. Anthonisen NR, Skeans MA, Wise RA, et al. The effects of a smoking cessation intervention on 14.5-year mortality: a randomized clinical trial. Ann
Intern Med 2005; 142: 233-239.
278. Sliwin ´ski P, Hawrylkiewicz I, Górecka D, et al. Acute effect of oxygen on pulmonary arterial pressure does not predict survival on long-term oxygen
therapy in patients with chronic obstructive pulmonary disease. Am Rev Respir Dis 1992; 146: 665-669.
279. Leuchte HH, Baumgartner RA, Nounou ME, et al. Brain natriuretic peptide is a prognostic parameter in chronic lung disease. Am J Respir Crit Care
Med 2006; 173: 744-750.
280. Nathan SD, Shlobin OA, Ahmad S, et al. Pulmonary hypertension and pulmonary function testing in idiopathic pulmonary fibrosis. Chest 2007; 131:
657-663.
281. Handa T, Nagai S, Miki S, et al. Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis. Chest 2006; 129: 1246-
1252.
282. Shorr AF, Helman DL, Davies DB, et al. Pulmonary hypertension in advanced sarcoidosis: epidemiology and clinical characteristics. Eur Respir J
2005; 25: 783-788.
283. Harari S, Simonneau G, De Juli E, et al. Prognostic value of pulmonary hypertension in patients with chronic interstitial lung disease referred for lung
or heart-lung transplantation. J Heart Lung Transplant 1997; 16: 460-463.
284. Le Pavec J, Lorillon G, Jais X, et al. Pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension: clinical characteristics and impact
of pulmonary arterial hypertension therapies. Chest 2012; 142: 1150-1157.
285. Weitzenblum E, Ehrhart M, Rasaholinjanahary J, et al. Pulmonary hemodynamics in idiopathic pulmonary fibrosis and other interstitial pulmonary
diseases. Respiration 1983; 44: 118-127.
286. Renzoni EA, Walsh DA, Salmon M, et al. Interstitial vascularity in fibrosing alveolitis. Am J Respir Crit Care Med 2003; 167: 438-443.
287. Lettieri CJ, athan SD, Barnett SD, et al. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest
2006; 129: 746-752.
288. King TE Jr, Tooze JA, Schwarz MI, et al. Predicting survival in idiopathic pulmonary fibrosis: scoring system and survival model. Am J Respir Crit Care
Med 2001; 164: 1171-1181.
289. Nadrous HF, Pellikka PA, Krowka MJ, et al. Pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Chest 2005; 128: 2393-2399.
290. Nunes H, Humbert M, Capron F, et al. Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis. Thorax
2006; 61: 68-74.
291. Sturani C, Papiris S, Galavotti V, et al. Pulmonary vascular responsiveness at rest and during exercise in idiopathic pulmonary fibrosis: effects of
oxygen and nifedipine. Respiration 1986; 50: 117-129.
292. King TE Jr, Behr J, Brown KK., et al. BUILD-1: a randomized placebo-controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit
Care Med 2008; 177: 75-81.
293. 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: 600-607.
294. Strange C, Bolster M, Mazur J, et al. Hemodynamic effects of epoprostenol in patients with systemic sclerosis and pulmonary hypertension. Chest
2000; 118: 1077-1082.
295. Health Canada Endorsed Important Safety Information on Volibris. Contraindication regarding the use of Volibris (ambrisentan) in patients with
Idiopathic Pulmonary Fibrosis (IPF), 2012.
296. Cottin V, Nunes H, Brillet PY,et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognized entity.Eur Respir J 2005; 26: 586-593.
297. Cottin V, Pavec JLe, Prévot G, et al. Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome. Eur Respir
J 2010; 35: 105-111.
298. 巽浩一郎，佐久間哲也，栗山喬之，他．結核後遺症-現時点における総括-．肺循環障害と睡眠呼吸障害 病態と治療．結核 2005; 80: 663-666.
299. 佐々木結花，山岸文雄，鈴木公典，他．在宅酸素療法を施行した肺結核後遺症症例における予後および肺循環諸量の変化の検討．日胸疾会誌 1997; 35:
511-517.
300. American Academy of Sleep Medicine. In: International classification of sleep disorders, 2nd ed.: Diagnostic and coding manual. American Academy
of Sleep Medicine.Westcheter, IL 2005.
202. Doyle RL, McCrory D, Channick RN, et al. Surgical treatments / interventions for pulmonary arterial hypertension: ACCP evidence-based clinical
practice guidelines. Chest 2004; 126: 63S-71S.
203. Glanville AR, Burke CM, Theodore J, et al. Primary pulmonary hypertension. Length of survival in patients referred for heart-lung transplantation. Chest
1987; 91: 675-681.
204. Christie JD, Edwards LB, Kucheryavaya AY, et al. The Registry of the International Society for Heart and Lung Transplantation: twenty-seventh official
adult lung and heartlung transplant report--2010. J Heart Lung Transplant 2010; 29: 1104-1118.
205. Bando T, Date H, Minami M, et al. First registry report: lung transplantation in Japan: The Japanese Society of Lung and Heart-Lung Transplantation.
Gen Thorac Cardiovasc Surg. 2008; 56: 17-21.
206. Bando K, Armitage JM, Paradis IL, et al. Indications for and results of single, bilateral, and heart-lung transplantation for pulmonary hypertension. J
Thorac Cardiovasc Surg 1994; 108: 1056-1065.
207. Waddell TK, et al. Heart-lung or lung transplantation for Eisenmenger syndrome. J Heart Lung Transplant 2002; 21:731-737.
208. Pigula FA, et al. Cardiopulmonary transplantation for congenital heart disease in the adult. J Heart Lung Transplant 2001; 20: 297-303.
209. Reichart B, et al. Improved results after heart-lung transplantation: a 17-year experience. Transplantation 2003; 75: 127-132.
210. Spray TL, et al. Pediatric lung transplantation for pulmonary hypertension and congenital heart disease. Ann Thorac Surg 1992; 54: 216-223.
211. Choong CK, et al. Repair of congenital heart lesions combined with lung transplantation for the treatment of severe pulmonary hypertension: a 13-year
experience. J Thorac Cardiovasc Surg 2005; 129: 661-669.
212. Trulock EP, et al. International Society for Heart and Lung Transplantation. 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 2006; 25: 880-892.
213. Keogh A, et al. Interventional and surgical modalities of treatment in pulmonary arterial hypertension. J Am Coll Cardiol 2009; 54: S67-S77.
214. Daliento L, et al. Eisenmenger syndrome. Factors relating to deterioration and death. Eur Heart J 1998; 19: 1845-1855.
215. Maurer JR, Frost AE, Estenne M, et al. International guidelines for the selection of lung transplant candidates. The International Society for Heart and
Lung Transplantation, the American Thoracic Society, the American Society of Transplant Physicians, the European Respiratory Society. J
Heart Lung Transplant 1998. 17: 703-709.
216. Guglin M, Khan H. Pulmonary hypertension in heart failure. J Card Fail 2010; 16: 461-474.
217. Abramson SV, Burke JF, Kelly JJ Jr, et al. Pulmonary hypertension predicts mortality and morbidity in patients with dilated cardiomyopathy. Ann
Intern Med 1992; 11: 888-895.
218. Ghio S, Gavazzi A, Campana C, et al. Independent and additive prognostic value of right ventricular systolic function and pulmonary artery pressure in
patients with chronic heart failure. J Am Coll Cardiol 2001; 1: 183-188.
219. Lam CS, Roger VL, Rodeheffer RJ, et al. Pulmonary hypertension in heart failure with preserved ejection fraction: a communitybased study. J Am Coll
Cardiol 2009; 13: 1119- 1126.
220. Leung CC, V Moondra, Catherwood E, et al. Prevalence and risk factors of pulmonary hypertension in patients with elevated pulmonary venous
pressure and preserved ejection fraction. Am J Cardiol 2010; 106: 284-286.
221. Kjaergaard J, Akkan D, Iversen KK, et al. Prognostic importance of pulmonary hypertension in patients with heart failure. Am J Cardiol 2007; 8: 1146-
1150.
222. Butler J, Chomsky DB, Wilson JR. Pulmonary hypertension and exercise intolerance in patients with heart failure. J Am Coll Cardiol 1999; 34: 1802-
1806.
223. Costard-Jäckle A, Fowler MB. Influence of preoperative pulmonary artery pressure on mortality after heart transplantation: testing of potential
reversibility of pulmonary hypertension with nitroprusside is useful in defining a high risk group. J Am Coll Cardiol 1992; 19: 48-54.
224. Abramson SV, Burke JF, Kelly JJ Jr, et al. Pulmonary hypertension predicts mortality and morbidity in patients with dilated cardiomyopathy. Ann
Intern Med 1992; 116: 888-895.
225. Erickson KW, Costanzo-Nordin MR, O’Sullivan EJ, et al. Influence of preoperative transpulmonary gradient on late mortality after orthotopic heart
transplantation. J Heart Transplant 1990; 9: 526-637.
226. Chen JM, Levin HR, Michler RE, et al. Reevaluating the significance of pulmonary hypertension before cardiac transplantation: determination of
optimal thresholds and quantification of the effect of reversibility on perioperative mortality. J Thorac Cardiovasc Surg 1997; 114: 627-634.
227. Delgado JF, Gomez-Sanchez MA, Saenz de la Calzada C, et al. Impact of mild pulmonary hypertension on mortality and pulmonary artery pressure
profile after heart transplantation. J Heart Lung Transplant 2001; 20: 942-928.
228. Chang PP, Longenecker JC, Wang NY, et al. Mild vs severe pulmonary hypertension before heart transplantation: different effects on
osttransplantation pulmonary hypertension and mortality. J Heart Lung Transplant 2005; 24: 998-1007.
229. Alfonso F, Macaya C, Hernandez R, et al. Percutaneous mitral valvuloplasty with severe pulmonary artery hypertension. Am J Cardiol 1993; 72: 325-
330.
230. Fawzy ME, Mimish L, Sivanandam V, et al. Immediate and long-term effect of mitral balloon valvotomy on severe pulmonary hypertension in patients
with mitral stenosis. Am Heart J 1996; 131: 89-93.
231. Zenner JC, Hancock EW, Shumway NE, et al. Regression of extreme pulmonary hypertension after mitral valve surgery. Am. J Cardiol 1972; 30: 820-
826.
232. Ward C, Hancock BW. Extreme Pulmonary hypertension caused by mitral valve disease. Natural history and results of surgery. Br Heart 1975;
37: 74- 78.
233. Vincens JJ, Temizer D, Post JR, et al. Long-term outcome of cardiac surgery in patients with mitral stenosis and severe pulmonary hypertension.
Circulation 1995; 92(9 Suppl): II137-142.
234. Tandon HD, Kasturi J. Pulmonary vascular changes associated with isolated mitral stenosis in India. Br Heart J 1975; 37: 26-36.
235. Goodale W, Sanchez G, Friedlich AL, et al. Correlation of pulmonary arteriolar resistance with pulmonary vascular changes in patients with mitral
stenosis before and aftervalvulotomy. N Engl J Med 1955; 252: 979-983.
236. Califf RM, Adams KF, McKenna WJ, et al. A randomized controlled trial of epoprostenol therapy for severe congestive heart failure: The Flolan
International Randomized Survival Trial (FIRST). Am Heart J 1997; 134: 44-54.
237. Mylona P, Cleland JG. Update of REACH-1 and MERIT HF clinical trials in heart failure. Cardio. net Editorial Team. Eur J Heart Fail 1999; 1: 197-200.
238. Packer M, McMurray J, Massie BM, et al. Clinical effects of endothelin receptor antagonism with bosentan in patients with severe chronic heart
failure: results of a pilot study. J Card Fail 2005; 11: 12-20.
239. Kalra PR, Moon JC, Coats AJ. Do results of the ENABLE (Endothelin Antagonist Bosentan for Lowering Cardiac Events in Heart Failure) study spell
the end for non-selective endothelin antagonism in heart failure? Int J Cardiol 2002; 85: 195-197.
240. Torre-Amione G, Young JB, Colucci WS, et al. Hemodynamic and clinical effects of tezosentan, an intravenous dual endothelin receptor antagonist, in
patients hospitalized for acute decompensated heart failure. J Am Coll Cardiol 2003; 42: 140-147.
241. Anand I, McMurray J, Cohn JN et al. Long-term effects of darusentan on left-ventricular remodelling and clinical outcomes in the EndothelinA Receptor
Antagonist Trial in Heart Failure (EARTH): randomised, double-blind, placebocontrolled trial. Lancet 2004; 364: 347-354.
242. Lewis GD, Shah R, Shahzad K, et al. Sildenafil improves exercise capacity and quality of life in patients with systolic heart failure and secondary
pulmonary hypertension. Circulation 2007; 116: 1555-1562.
243. Behling A, Rohde LE, Colombo FC, et al. Effects of 5’-phosphodiesterase four-week long inhibition with sildenafil in patients with chronic heart failure:
a double-blind, placebocontrolled clinical trial. J Card Fail 2008; 14: 189-197.
244. Guazzi M, Vicenzi M, Arena R, et al. PDE5 Inhibition With Sildenafil Improves Left Ventricular Diastolic Function, Cardiac Geometry, and Clinical
Status in Patients With Stable Systolic Heart Failure. Results of a 1-Year, Prospective, Randomized, Placebo-Controlled Study. Circ Heart Fail
2011; 4: 8-17.
245. Elliott CG, Palevsky HI. Treatment with epoprostenol of pulmonary arterial hypertension following mitral valve replacement for mitral stenosis. Thorax
2004; 59: 536-537.
246. Trachte AL, MD, Lobato EB, Urdaneta F, et al. Oral Sildenafil Reduces Pulmonary Hypertension After Cardiac Surgery. Ann Thorac Surg 2005; 79:
194 -197.
247. 日本呼吸器学会COPDガイドライン第3版作成委員会.COPD（慢性閉塞性肺疾患）診断と治療のためのガイドライン．第3版．東京，日本呼吸器学会 2009．
248. Barberà JA, Peinado VI, Santos S. Pulmonary hypertension in chronic obstructive pulmonary disease. Eur Respir J 2003;21: 892-905.
249. Santos S, Peinado VI, Ramírez J, et al. Characterization of pulmonary vascular remodelling in smokers and patients with mild COPD. Eur Respir J
2002; 19: 632-638.
250. 平賀俊尚，岸不盡彌，川上義和，他．慢性呼吸不全患者 における低酸素血症の肺循環に及ぼす影響 －動脈血酸素分圧・基礎疾患別検討－．昭和62年,
厚生省特定疾患呼吸不全調査研究班（編）：昭和62年度研究報告書. 135-140.
251. Stevens D, Sharma K, Szidon P, et al. Severe pulmonary hypertension associated with COPD. Ann Transplant 2000; 5:8-12.
252. Scharf SM, Iqbal M, Keller C, et al. Hemodynamic characterization of patients with severe emphysema. Am J Respir Crit Care Med 2002; 166: 314-
322.
253. Incalzi RA, Fuso L, De Rosa M, et al. Electrocardiographic signs of chronic cor pulmonale: A negative prognostic finding in chronic obstructive
pulmonary disease. Circulation 1999;99: 1600-6105.
254. Burrows B, et al. Patterns of cardiovascular dysfunction in chronic obstructive lung disease. N Engl J Med 1972; 286: 912-918.
255. Fletcher E.C and Levin DC. Cardiopulmona ry hemodynamics during sleep in subjects with chronic obstructive pulmonary disease. The effect of
short- and longterm oxygen. Chest 1984; 85: 6-14.
256. Zenker G, Forche G, Harnoncourt K. Two-dimensional echocardiography using a subcostal approach in patients with COPD. Chest 1985; 88: 722-725.
257. Marcus JT, Vonk Noordegraaf A, De Vries PM, et al. MRI evaluation of right ventricular pressure overload in chronic obstructive pulmonary disease. J
Magn Reson Imaging 1998; 8: 999-1005.
258. Yamaoka S, Yonekura Y, Koide H, et al. Noninvasive method to assess cor pulmonale in patients with chronic obstructive pulmonary disease Chest
1987; 92: 10-17.
259. Morrison DA, Pulmonary hypertension in chronic obstructive pulmonary disease: the right ventricular hypothesis. Chest 1987; 92: 387-389.
260. Oswald-Mammosser M, Weitzenblum E, Quoix E, et al. Prognostic factors in COPD patients receiving long-term oxygen therapy. Importance of
pulmonary artery pressure. Chest 1995; 107: 1193-1198.
261. Kessler R, Faller M, Fourgaut G, et al. Predictive factors of hospitalization for acute exacerbation in a series of 64 patients with chronic obstructive
pulmonary disease. Am J Respir Crit Care Med 1999; 159: 158-164.
262. Minai OA, Chaouat A, Adnot S. Pulmonary hypertension in COPD: epidemiology, significance, and management: pulmonary vascular disease: the
global perspective. Chest 2010; 137(Suppl): 39S-51S.
263. Wells JM, Washko GR, Han MK, et al. Pulmonary arterial enlargement and acute exacerbations of COPD. N Engl J Med 2012; 367: 913-921.
264. No authors listed. Long term domiciliary oxygen therapy in chronic hypoxic cor pulmonale complicating chronic bronchitis and emphysema. Report of
the Medical Research Council Working Party. Lancet 1981;1: 681-686.
265. No authors listed. Continuous or nocturnal oxygen therapy in hypoxemic chronic obstructive lung disease: a clinical trial. Nocturnal Oxygen Therapy
Trial Group. Ann Intern Med 1980; 93: 391-398.
266. Krop HD, Block AJ, Cohen E.. Neuropsychologic effects of continuous oxygen therapy in chronic obstructive pulmonary disease. Chest 1973;
64: 317- 322.
267. Weitzenblum E, Sautegeau A, Ehrhart M, et al. Long-term oxygen therapy can reverse the progression of pulmonary hypertension in patients with
chronic obstructive pulmonary disease. Am Rev Respir Dis 1985; 131: 493-498.
268. Chaouat A, Weitzenblum E, Kessler R, et al. A randomized trial of nocturnal oxygen therapy in chronic obstructive pulmonary disease patients.
Eur Respir J 1999; 14: 1002-1008.
269. Blanco I, Gimeno E, Munoz PA, et al. Hemodynamic and gas exchange effects of sildenafil in patients with chronic obstructive pulmonary disease
and pulmonary hypertension. Am J Respir Crit Care Med 2010; 181: 270-278.
270. Barberà JA, Roger N, Roca J, et al. Worsening of pulmonary gas exchange with nitric oxide inhalation in chronic obstructive pulmonary disease.
Lancet 1996; 347:436-440.
271. Ashutosh K, Phadke K, Jackson JF, et al. Use of nitric oxide inhalation in chronic obstructive pulmonary disease. Thorax 2000 ; 55: 109-113.
272. Vonbank K, Ziesche R, Higenbottam TW, et al. Controlled prospective randomised trial on the effects on pulmonary haemodynamics of the
ambulatory long term use of nitric oxide and oxygen in patients with severe COPD. Thorax 2003; 58: 289-293.
273. Hurdman J, Condliffe R, Elliot CA, et al. Pulmonary hypertension in COPD: results from the ASPIRE Registry. Eur Respir J 2012 Sep 27.
[Epub ahead of print]
274. Criner GJ, Scharf SM, Falk JA., et al. Effect of lung volume reduction surgery on resting pulmonary hemodynamics in severe emphysema.
Am J Respir Crit Care Med 2007; 176: 253-260.
275. 日本呼吸器学会COPDガイドライン第3版作成委員会.COPD（慢性閉塞性肺疾患）診断と治療のためのガイドライン 第3版. 2009, 東京: 日本呼吸器学会.
276. Rogers TK, Howard P. Howard, Pulmonary hemodynamics and physical training in patients with chronic obstructive pulmonary disease. Chest 1992;
101(5 Suppl): 289S-292S.
277. Anthonisen NR, Skeans MA, Wise RA, et al. The effects of a smoking cessation intervention on 14.5-year mortality: a randomized clinical trial. Ann
Intern Med 2005; 142: 233-239.
278. Sliwin ´ski P, Hawrylkiewicz I, Górecka D, et al. Acute effect of oxygen on pulmonary arterial pressure does not predict survival on long-term oxygen
therapy in patients with chronic obstructive pulmonary disease. Am Rev Respir Dis 1992; 146: 665-669.
279. Leuchte HH, Baumgartner RA, Nounou ME, et al. Brain natriuretic peptide is a prognostic parameter in chronic lung disease. Am J Respir Crit Care
Med 2006; 173: 744-750.
280. Nathan SD, Shlobin OA, Ahmad S, et al. Pulmonary hypertension and pulmonary function testing in idiopathic pulmonary fibrosis. Chest 2007; 131:
657-663.
281. Handa T, Nagai S, Miki S, et al. Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis. Chest 2006; 129: 1246-
1252.
282. Shorr AF, Helman DL, Davies DB, et al. Pulmonary hypertension in advanced sarcoidosis: epidemiology and clinical characteristics. Eur Respir J
2005; 25: 783-788.
283. Harari S, Simonneau G, De Juli E, et al. Prognostic value of pulmonary hypertension in patients with chronic interstitial lung disease referred for lung
or heart-lung transplantation. J Heart Lung Transplant 1997; 16: 460-463.
284. Le Pavec J, Lorillon G, Jais X, et al. Pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension: clinical characteristics and impact
of pulmonary arterial hypertension therapies. Chest 2012; 142: 1150-1157.
285. Weitzenblum E, Ehrhart M, Rasaholinjanahary J, et al. Pulmonary hemodynamics in idiopathic pulmonary fibrosis and other interstitial pulmonary
diseases. Respiration 1983; 44: 118-127.
286. Renzoni EA, Walsh DA, Salmon M, et al. Interstitial vascularity in fibrosing alveolitis. Am J Respir Crit Care Med 2003; 167: 438-443.
287. Lettieri CJ, athan SD, Barnett SD, et al. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest
2006; 129: 746-752.
288. King TE Jr, Tooze JA, Schwarz MI, et al. Predicting survival in idiopathic pulmonary fibrosis: scoring system and survival model. Am J Respir Crit Care
Med 2001; 164: 1171-1181.
289. Nadrous HF, Pellikka PA, Krowka MJ, et al. Pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Chest 2005; 128: 2393-2399.
290. Nunes H, Humbert M, Capron F, et al. Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis. Thorax
2006; 61: 68-74.
291. Sturani C, Papiris S, Galavotti V, et al. Pulmonary vascular responsiveness at rest and during exercise in idiopathic pulmonary fibrosis: effects of
oxygen and nifedipine. Respiration 1986; 50: 117-129.
292. King TE Jr, Behr J, Brown KK., et al. BUILD-1: a randomized placebo-controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit
Care Med 2008; 177: 75-81.
293. 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: 600-607.
294. Strange C, Bolster M, Mazur J, et al. Hemodynamic effects of epoprostenol in patients with systemic sclerosis and pulmonary hypertension. Chest
2000; 118: 1077-1082.
295. Health Canada Endorsed Important Safety Information on Volibris. Contraindication regarding the use of Volibris (ambrisentan) in patients with
Idiopathic Pulmonary Fibrosis (IPF), 2012.
296. Cottin V, Nunes H, Brillet PY,et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognized entity.Eur Respir J 2005; 26: 586-593.
297. Cottin V, Pavec JLe, Prévot G, et al. Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome. Eur Respir
J 2010; 35: 105-111.
298. 巽浩一郎，佐久間哲也，栗山喬之，他．結核後遺症-現時点における総括-．肺循環障害と睡眠呼吸障害 病態と治療．結核 2005; 80: 663-666.
299. 佐々木結花，山岸文雄，鈴木公典，他．在宅酸素療法を施行した肺結核後遺症症例における予後および肺循環諸量の変化の検討．日胸疾会誌 1997; 35:
511-517.
300. American Academy of Sleep Medicine. In: International classification of sleep disorders, 2nd ed.: Diagnostic and coding manual. American Academy
of Sleep Medicine.Westcheter, IL 2005.