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
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119. Mukhopadhyay S, Nathani S, Yusuf J, et al. Clinical efficacy of phosphodiesterase-5 inhibitor tadalafil in Eisenmenger syndrome--a randomized,
placebo-controlled, double-blind crossover study. Congenit Heart Dis 2011; 6: 424-431.
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Coll Cardiol 2002; 39: 1496-1502.
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104. Galie N, Beghetti M, Gatzoullis MA, et al. Bosentan therapy inpatients with Eisenmenger syndrome: a multicenter, double-blind, randomized,
placebo-controlled study. Circulation 2006; 114: 48-54.
105. Galie N, Rubin LJ, Hoeper M, et al. Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a
double-blind, randomized controlled trial. Lancet 2008; 371: 2093-2100.
106. 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.
107. Provencher S, Sitbon O, Humbert M, et al. Long-term outcome with first-line bosentan therapy in idiopathic pulmonary arterial hypertension. Eur Heart
J 2006; 27: 589- 595.
108. Galié N, Badesch D, Oudiz R, et al. Ambrisentan therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2005; 46: 529-535.
109. Galie N, Olschewski H, Oudiz R, et al. Ambrisentan for the treatment of pulmonary hypertension: results of ambrisentan inpulmonary arterial
hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and2. Circulation 2008; 117: 3010-3019.
110. Oudiz RJ, Galie N, Olschewski H, et al. Long-term ambrisentan therapy for the treatment of pulmonary arterial hypertension. J Am Coll Cardiol 2009;
54: 1971-1981.
111. Michelakis ED, Tymchak W, Noga M, et al. Long-term treatment with oral sildenafil is safe and improves functional capacity and hemodynamics in
patients with pulmonary arterial hypertension. Circulation 2003; 108: 2066-2069.
112. Ghofrani HA, Schermuly RT, Rose F, et al. Sildenafil for long-term treatment of nonoperable chronic thromboembolic pulmonary hypertension. Am J
Respir Crit Care Med 2003; 167: 1139-1141.
113. Galiè N, Ghofrani HA, Torbicki A, et al. Sildenafil Use in Pulmonary Arterial Hypertension (SUPER) Study Group: Sildenafil citrate therapy for
pulmonary arterial hypertension. N Eng J Med 2005; 353: 2148-2157.
114. Ghofrani HA, Pepke-Zaba J, Barbera JA, et al. Nitric oxide pathway and phosphdiesterase inhibitors in pulmonary arterial hypertension. J Am Coll
Cardiol 2004; 43: 68S-72S.
115. Ghofrani HA, Wiedemann R, Rose F, et al. Sildenafil for treatment of lung fibrosis and pulmonary hypertension: a randomized controlled trial. Lancet
2002; 360: 895-900.
116. Rubin LJ, Badesch DB, Fleming TR, et al. Long-term treatment with sildenafil citrate in pulmonary arterial hypertension: the SUPER-2 study. Chest
2011; 140: 1274- 1283.
117. Galiè N, Brundage BH, Ghofrani HA, et al. Pulmonary Arterial Hypertension and Response to Tadalafil (PHIRST) Study Group. Circulation 2009; 119:
2894-2903.
118. Oudiz RJ, Brundage BH, Galiè N, et al. Tadalafil for the treatment of pulmonary arterial hypertension: a double-blind 52-week uncontrolled extension
study. J Am Coll Cardiol 2012; 60: 768-774.
119. Mukhopadhyay S, Nathani S, Yusuf J, et al. Clinical efficacy of phosphodiesterase-5 inhibitor tadalafil in Eisenmenger syndrome--a randomized,
placebo-controlled, double-blind crossover study. Congenit Heart Dis 2011; 6: 424-431.
120. Moncada S, Gryglewski R, Bunting S, et al. An enzyme isolated from arteries transforms prostaglandin endoperoxides to an unstable substance that
inhibits platelet aggregation. Nature 1976; 263: 663-665.
121. Christman BW, McPherson CD, Newman JH, et al. An imbalance between the excretion of thromboxane and prostacyclin metabolites in pulmonary
hypertension.N Engl J Med 1992; 327: 70-75.
122. Tuder RM, Cool CD, Geraci MW, et al. Prostacyclin synthase expression is decreased in lungs from patients with severe pulmonary hypertension.
Am J Respir Crit Care Med 1999; 159: 1925-1932.
123. Rubin LJ, 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: 485-491.
124. Barst RJ, Rubin LJ, McGoon MD, et al. Survival in primary pulmonary hypertension with long-term continuous intravenous prostacyclin.Ann Intern Med
1994; 121: 409-415.
125. 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.
126. McLaughlin VV, Genthner DE, Panella MM, et al. Reduction in pulmonary vascular resistance with long-term epoprostenol (prostacyclin) therapy in
primary pulmonary hypertension. N Engl J Med 1998; 338: 273-277.
127. Badesch DB, Tapson VF, McGoon MD, et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of
diseased: a randomized, controlled trial. Ann Intern Med 2000; 132: 425-434.
128. Ryerson CJ, Nayar S, Swiston JR, et al. Pharmacotherapy in pulmonary arterial hypertension: a systematic review and meta-analysis. Respir Res
2010; 11: 12.
129. McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation 2002; 106: 1477-
1482.
130. Yung D, Widlitz AC, Rosenzweig EB, et al. Outcome in Children with idiopathic pulmonary hypertension. Circulation 2004; 110: 660-665.
131. Badesch DB, McLaughlin VV, Delcroix M, et al. Prostanoid therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2004; 43: 56S-61S.
132. Akagi S, Nakamura K, Miyaji K, et al. Marked hemodynamic improvements by high-dose epoprostenol therapy in patients with idiopathic pulmonary
arterial hypertension. Circ J 2010; 74: 2200-2205.
133. 国枝武義，粕川禮司，西間木友衛，他．TRK-100の原 発性肺高血圧症，膠原病に合併する肺高血圧症に対する臨 床試験．臨床と研究 1997; 74: 205-223.
134. Nagaya N, Uematsu M, Okano Y, et al. Effect of orally active prostacyclin analogue on survival of outpatients with primary pulmonary hypertension. J
Am Coll Cardiol 1999; 34: 1188-1192.
135. Galie N, Humbert M, Vachiery JL, et al. Arterial Pulmonary Hypertension and Beraprost European (ALPHABET) Study Group: Effects of beraprost
sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled trial. J Am
Coll Cardiol 2002; 39: 1496-1502.
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