循環器ジャーナル66巻1号

2018年01月発行

特集 循環器診療　薬のギモン—エキスパートに学ぶ薬物治療のテクニック Ⅴ．肺高血圧症診療でのギモン

upfront治療って実際にどうするの？

著者： 上田仁¹ 大郷剛¹

所属機関： ¹国立循環器病研究センター肺循環科

ページ範囲：P.132 - P.140

Point
・肺高血圧症の原因，病態は多岐にわたるため，適切な治療を行うには，正確な確定診断，病型分類，重症度評価が必要である．
・肺高血圧症の各疾患は，同一疾患とは思えないほど多彩な臨床像を示すことがあるため，診断後は，早期に肺高血圧症診療の経験豊富な専門施設への紹介が望ましいと推奨されている．
・肺血管拡張薬は，3系統の薬剤が存在し，主にGroup 1である肺動脈性肺高血圧症に対して使用されている．
・肺血管拡張薬の使用に当たっては，各々の特性と副作用，薬剤間相互作用を十分に理解することが重要である．

参考文献

1) 日本循環器学会：肺高血圧症治療ガイドライン（2012年改訂版）．

2) Sitbon O, Humbert M, Jaïs X, et al:Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 111:3105-3111, 2005

3) Galiè N, Humbert M, Vachiery JL, et al:2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 46:903-975, 2015

4) Simonneau G, Gatzoulis MA, Adatia I, et al:Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 62:D34-41, 2013

5) Humbert M, Sitbon O, Chaouat A, et al:Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation 122:156-163, 2010

6) Hoeper MM, Markevych I, Spiekerkoetter E, et al:Goal-oriented treatment and combination therapy for pulmonary arterial hypertension. Eur Respir J 26:858-863, 2005

7) Simonneau G, Rubin LJ, Galiè N, et al:Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension:a randomized trial. Ann Intern Med 149:521-530, 2008

8) McLaughin VV, Oudiz RJ, Frost A, et al:Randomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertension. Am J Respir Crit Care Med 174:1257-1263, 2006

9) Hoeper MM, Leuchte H, Halank M, et al:Combining inhaled iloprost with bosentan in patients with idiopathic pulmonary arterial hypertension. Eur Respir J 28:691-694, 2006

10) Gruenig E, Michelakis E, Vachiéry JL, et al:Acute hemodynamic effects of single-dose sildenafil when added to established bosentan therapy in patients with pulmonary arterial hypertension:results of the COMPASS-1 study. J Clin Pharmacol 49:1343-1352, 2009

11) McLaughlin V, Channick RN, Ghofrani HA, et al:Bosentan added to sildenafil therapy in patients with pulmonary arterial hypertension. Eur Respir J 46:405-413, 2015

12) McLaughlin VV, Benza RL, Rubin LJ, et al:Addition of inhaled treprostinil to oral therapy for pulmonary arterial hypertension:a randomized controlled clinical trial. J Am Coll Cardiol 55:1915-1922, 2010

13) Pulido T, Adzerikho I, Channick RN, et al:Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 369:809-818, 2013

14) Rubin LJ, Badesch DB, Fleming TR, et al:Long term treatment with sildenafil citrate in pulmonary arterial hypertension:the SUPER-2 study. Chest 140:1274-1283, 2011

15) Humbert M, Barst RJ, Robbins IM, et al:Combination of bosentan with epoprostenol in pulmonary arterial hypertension:BREATHE-2. Eur Respir J 24:353-359, 2004

16) Kemp K, Savale L, O'Callaghan DC, et al:Usefulness of first-line combination therapy with epoprostenol and bosentan in pulmonary arterial hypertension:an observational study. J Heart Lung Transplant 31:150-158, 2012

17) Galiè N, Barberá JA, Frost AE, et al:Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension. N Engl J Med 373:834-844, 2015

18) Hoeper MM, McLaughin VV, Barberá JA, et al:Initial combination therapy with ambrisentan and tadalafil and mortality in patients with pulmonary arterial hypertension:a secondary analysis of the results from the randomized, controlled AMBITION study. Lancet Respir Med 4:894-901, 2016

19) Tamura Y, Kumamaru H, Satoh T, et al:Effectiveness and outcome of pulmonary arterial hypertension-specific therapy in Japanese patients with pulmonary arterial hypertension. Circ J doi:10.1253/circj.CJ-17-0139, 2017

20) Sitbon O, Sattler C, Bertoletti L, et al:Initial dual oral combination therapy in pulmonary arterial hypertension. Eur Respir J 47:1727-1736, 2016

21) Sitbon O, Jaïs X, Savale L, et al:Upfront triple combination therapy in pulmonary arterial hypertension:a pilot study. Eur Respir J 43:1691-1697, 2014