循環器ジャーナル71巻4号

2023年10月発行

特集 変革期を迎えた肺高血圧症—次世代の病態理解，診断，治療とは？ Ⅱ.各論：各種肺高血圧症の診断と治療

PAH治療薬③：プロスタサイクリン系薬剤

著者： 竹内かおり¹

所属機関： ¹杏林大学医学部付属病院循環器内科

ページ範囲：P.515 - P.522

POINT
●肺高血圧症の併用療法におけるプロスタサイクリン系薬剤の位置付けと，使用方法についてまとめた．
●治療により改善した症例に対して，次のステップとしてどのように減薬を判断するか，当院での経験をまとめた．

参考文献

1）Okada O, Tanabe N, Yasuda J, et al. Prediction of life expectancy in patients with primary pulmonary hypertension. A retrospective nationwide survey from 1980-1990. Intern Med 1999 ; 38 : 12-6.

2）Tamura Y, Kumamaru H, Satoh T, et al. Japan PH Registry（JAPHR）Network. Effectiveness and Outcome of Pulmonary Arterial Hypertension-Specific Therapy in Japanese Patients With Pulmonary Arterial Hypertension. Circ J 2017 ; 82 : 275-82.

3）Ogawa A, Ejiri K, Matsubara H. Long-term patient survival with idiopathic/heritable pulmonary arterial hypertension treated at a single center in Japan : Life Sci 2014 ; 118 : 414-9.

4）Lang IM, Gaine SP. Recent advances in targeting the prostacyclin pathway in pulmonary arterial hypertension. Eur Respir Rev 2015 ; 24 : 630-41.

5）Barst RJ, McGoon M, McLaughlin V, et al ; Beraprost Study Group. Beraprost therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2003 ; 41 : 2119-25.

6）Sitbon O, Channick R, Chin KM, et al ; GRIPHON Investigators. Selexipag for the Treatment of Pulmonary Arterial Hypertension. N Engl J Med 2015 ; 373 : 2522-33.

7）Chin KM, Sitbon O, Doelberg M, et al. Three-Versus Two-Drug Therapy for Patients With Newly Diagnosed Pulmonary Arterial Hypertension. J Am Coll Cardiol 2021 ; 78 : 1393-403.

8）Tokunaga N, Ogawa A, Ito H, et al. Rapid and high-dose titration of epoprostenol improves pulmonary hemodynamics and clinical outcomes in patients with idiopathic and heritable pulmonary arterial hypertension. J Cardiol 2016 ; 68 : 542-7.

9）Sitbon O, Manes A, Jais X, et al. Rapid switch from intravenous epoprostenol to intravenous treprostinil in patients with pulmonary arterial hypertension. J Cardiovasc Pharmacol 2007 ; 49 : 1-5.

10）Shirai Y, Tamura Y, Yasuoka H, et al. IgG4-related disease in pulmonary arterial hypertension on long-term epoprostenol treatment. Eur Respir J 2014 ; 43 : 1516-9.

11）Izumi K, Inami T, Takeuchi K, et al. Reversible Cardiac Hypertrophy in Pulmonary Arterial Hypertension Treated With High-Dose Epoprostenol. CJC Open 2022 ; 4 : 816-9.

12）Miura Y, Kataoka M, Chiba T, et al. Giant fold gastritis induced by epoprostenol infusion in patients with pulmonary arterial hypertension. Circ J 2018 ; 82 : 2676-7.

13）Tamura Y, Furukawa A, Tamura Y, et al. Long-term safety of a structured transition protocol from parenteral prostanoids to selexipag in pulmonary arterial hypertension. Pulm Circ 2022 ; 12 : e12058.

14）Takeuchi K, Goda A, Ito J, et al : Successful epoprostenol withdrawal and termination with an aid of the exercise stress test in pulmonary arterial hypertension. Int J Cardiol 2022 ; 346 : 80-5.