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

CTEPHに対する薬物療法—抗凝固療法も含めて

著者： 細川和也¹

所属機関： ¹九州大学病院循環器内科

ページ範囲：P.556 - P.561

POINT
●CTEPHに対する抗凝固療法としてはビタミンK拮抗薬が推奨され，直接経口抗凝固薬のエビデンスは不足している．
●CTEPHに対する肺血管拡張薬としてはリオシグアトとセレキシパグが承認されている．
●抗凝固療法に加え外科治療，BPA，肺血管拡張薬による集学的治療が推奨されており，戦略の最適化が試みられている．

参考文献

1）Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 2023 ; 61 : 2200879.

2）Hosokawa K, Abe K, Funakoshi K, et al. Long-term outcome of chronic thromboembolic pulmonary hypertension using direct oral anticoagulants and warfarin : a Japanese prospective cohort study. J Thromb Haemost 2023 ; 21 : 2151-62.

3）Jensen KW, Kerr KM, Fedullo PF, et al. Pulmonary hypertensive medical therapy in chronic thromboembolic pulmonary hypertension before pulmonary thromboendarterectomy. Circulation 2009 ; 120 : 1248-54.

4）Delcroix M, Lang I, Pepke-Zaba J, et al. Long-Term Outcome of Patients With Chronic Thromboembolic Pulmonary Hypertension : Results From an International Prospective Registry. Circulation 2016 ; 133 : 859-71.

5）Bunclark K, Newnham M, Chiu YD, et al. A multicenter study of anticoagulation in operable chronic thromboembolic pulmonary hypertension. J Thromb Haemost 2019 ; 18 : 114-22.

6）Sena S, Bulent M, Derya K, et al. Real-life data of direct anticoagulant use, bleeding risk and venous thromboembolism recurrence in chronic thromboembolic pulmonary hypertension patients : an observational retrospective study. Pulm Circ 2020 ; 10 : 2045894019873545.

7）Humbert M, Simonneau G, Pittrow D, et al. Oral anticoagulants（NOAC and VKA）in chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant 2022 ; 41 : 716-21.

8）Hosokawa K, Abe K, Kishimoto J, et al. Efficacy and safety of edoxaban in patients with chronic thromboembolic pulmonary hypertension : protocol for a multicentre, randomised, warfarin-controlled, parallel group trial-KABUKI trial. BMJ Open 2022 ; 12 : e061225.

9）Ghofrani HA, D'Armini AM, Grimminger F, et al ; CHEST-1 Study Group. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med 2013 ; 369 : 319-29.

10）Ogo T, Shimokawahara H, Kinoshita H, et al. Selexipag for the treatment of chronic thromboembolic pulmonary hypertension. Eur Respir J 2022 ; 60 : 2101694.

11）Sadushi-Kolici R, Jansa P, Kopec G, et al. Subcutaneous treprostinil for the treatment of severe non-operable chronic thromboembolic pulmonary hypertension（CTREPH） : a double-blind, phase 3, randomised controlled trial. Lancet Respir Med 2019 ; 7 : 239-48.

12）Ghofrani HA, Simonneau G, D'Armini AM, et al ; MERIT study investigators. Macitentan for the treatment of inoperable chronic thromboembolic pulmonary hypertension（MERIT-1） : results from the multicentre, phase 2, randomised, double-blind, placebo-controlled study. Lancet Respir Med 2017 ; 5 : 785-94.

13）Reichenberger F, Voswinckel R, Enke B, et al. Long-term treatment with sildenafil in chronic thromboembolic pulmonary hypertension. Eur Respir J 2007 ; 30 : 922-7.

14）Jais X, D'Armini AM, Jansa P, et al. Bosentan for treatment of inoperable chronic thromboembolic pulmonary hypertension : BENEFiT（Bosentan Effects in iNopErable Forms of chronIc Thromboembolic pulmonary hypertension）, a randomized, placebo-controlled trial. J Am Coll Cardiol 2008 ; 52 : 2127-34.

15）Tanabe N, Ogo T, Hatano M, et al. Safety and effectiveness of riociguat for chronic thromboembolic pulmonary hypertension in real-world clinical practice : interim data from post-marketing surveillance in Japan. Pulm Circ 2020 ; 10 : 2045894020938986.

16）Kawakami T, Matsubara H, Shinke T, et al. Balloon pulmonary angioplasty versus riociguat in inoperable chronic thromboembolic pulmonary hypertension（MR BPA） : an open-label, randomised controlled trial. Lancet Respir Med 2022 ; 10 : 949-60.

17）Jais X, Brenot P, Bouvaist H, et al. Balloon pulmonary angioplasty versus riociguat for the treatment of inoperable chronic thromboembolic pulmonary hypertension（RACE） : a multicentre, phase 3, open-label, randomised controlled trial and ancillary follow-up study. Lancet Respir Med 2022 ; 10 : 961-71.