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？ Ⅰ.総論：肺高血圧症の診断と治療

肺高血圧症の診断と治療アルゴリズム

著者： 矢尾板信裕¹ 安田聡¹

所属機関： ¹東北大学病院循環器内科

ページ範囲：P.480 - P.488

POINT
●肺高血圧症は治療の進歩により予後が改善しつつある一方で，早期診断・早期治療の必要性が増している．そのためには，肺高血圧症を疑う症状や身体所見から肺高血圧症に必要な検査に進まないといけない．
●本稿では肺高血圧症を疑う症状と，そこから肺高血圧症の鑑別に必要な検査を日欧のガイドラインからわかりやすく解説する．
●また，肺動脈性肺高血圧症は3系統の肺血管拡張薬を組み合わせて行われる一方で，慢性血栓塞栓性肺高血圧症は手術やカテーテル治療，肺血管拡張薬を組み合わせて行われる．このように治療に関しても様々な選択肢があるため，治療に関してもわかりやすく解説する．

参考文献

1）Colman R, Whittingham H, Tomlinson G, et al. Utility of the physical examination in detecting pulmonary hypertension. A mixed methods study. PLoS One 2014 ; 9 : e108499.

2）Rich JD, Thenappan T, Freed B, et al. QTc prolongation is associated with impaired right ventricular function and predicts mortality in pulmonary hypertension. Int J Cardiol 2013 ; 167 : 669-76.

3）Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2022 ; 43 : 3618-731.

4）Huston JH, Maron BA, French J, et al. Association of mild echocardiographic pulmonary hypertension with mortality and right ventricular function. JAMA Cardiol 2019 ; 4 : 1112-21.

5）Hughes JM, Pride NB. Examination of the carbon monoxide diffusing capacity（DL（CO））in relation to its KCO and VA components. Am J Respir Crit Care Med 2012 ; 186 : 132-9.

6）Trip P, Nossent EJ, de Man FS, et al. Severely reduced diffusion capacity in idiopathic pulmonary arterial hypertension : patient characteristics and treatment responses. Eur Respir J 2013 ; 42 : 1575-85.

7）Sitbon O, Humbert M, Jaïs X, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005 ; 111 : 3105-11.

8）Galiè N, Muller K, Scalise AV, et al. PATENT PLUS : a blinded, randomised and extension study of riociguat plus sildenafil in pulmonary arterial hypertension. Eur Respir J 2015 ; 45 : 1314-22.

9）Clozel M, Maresta A, Humbert M. Endothelin receptor antagonists. Handb Exp Pharmacol 2013 ; 218 : 199-227.

10）Galiè N, Manes A, Branzi A. Prostanoids for pulmonary arterial hypertension. Am J Respir Med 2003 ; 2 : 123-37.

11）Sitbon O, Humbert M, Jais X, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005 ; 111 : 3105-11.

12）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.

13）Miyamichi-Yamamoto S, Fukumoto Y, Sugimura K, et al. Intensive immunosuppressive therapy improves pulmonary hemodynamics and long-term prognosis in patients with pulmonary arterial hypertension associated with connective tissue disease. Circ J 2011 ; 75 : 2668-74.

14）Waxman A, Restrepo-Jaramillo R, Thenappan T, et al. Inhaled treprostinil in pulmonary hypertension due to interstitial lung disease. N Engl J Med 2021 ; 384 : 325-34.

15）Vitulo P, Stanziola A, Confalonieri M, et al. Sildenafil in severe pulmonary hypertension associated with chronic obstructive pulmonary disease : a randomized controlled multicenter clinical trial. J Heart Lung Transplant 2017 ; 36 : 166-74.

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）Aoki T, Sugimura K, Nochioka K, et al. Comprehensive evaluation of the effectiveness and safety of balloon pulmonary angioplasty for inoperable chronic thrombo-embolic pulmonary hypertension : long-term effects and procedure-related complications. Eur Heart J 2017 ; 38 : 3152-9.