循環器ジャーナル69巻3号

2021年07月発行

特集 成人先天性心疾患 エキスパートコンセンサス Ⅲ．成人先天性心疾患の病態別の治療指針

肺高血圧症を合併した患者に対する管理と治療

著者： 赤木達¹

所属機関： ¹岡山大学大学院医歯薬学総合研究科循環器内科学

ページ範囲：P.471 - P.477

Point
・肺動脈性肺高血圧症は先天性心疾患における重要な併存疾患である．
・肺動脈性肺高血圧症に対し，特異的肺血管拡張薬が有効な症例が存在する．
・Treat and Repairは，シャント性心疾患関連肺動脈性肺高血圧症の治療戦略である．

参考文献

1) Baumgartner H, De Backer J, Babu-Narayan SV, et al:2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J 42:563-645, 2021

2) Stout KK, Daniels CJ, Aboulhosn JA, et al:2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease:Executive Summary:A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol 73:1494-1563, 2019

3) Simonneau G, Gatzoulis MA, Adatia I, et al:Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 62:D34-D41, 2013

4) Kaemmerer H, Gorenflo M, Huscher D, et al:Pulmonary hypertension in adults with congenital heart disease:Real-world data from the international COMPERA-CHD Registry. J Clin Med 9:1456, 2020

5) Manes A, Palazzini M, Leci E, et al:Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease:a comparison between clinical subgroups. Eur Heart J 35:716-724, 2014

6) Akagi S, Matsubara H, Nakamura K, et al:Modern treatment to reduce pulmonary arterial pressure in pulmonary arterial hypertension. J Cardiol 72:466-472, 2018

7) Galiè N, Channick RN, Frantz RP, et al:Risk stratification and medical therapy of pulmonary arterial hypertension. Eur Respir J 53:1801889, 2019

8) Galiè N, Humbert M, Vachiery JL, et al:2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 37:67-119, 2015

9) Benza RL, Gomberg-Maitland M, Elliott CG, et al:Predicting Survival in Patients With Pulmonary Arterial Hypertension. Chest 156:323-337, 2019

10) Kijima Y, Akagi T, Takaya Y, et al:Treat and Repair Strategy in Patients With Atrial Septal Defect and Significant Pulmonary Arterial Hypertension. Circ J 80:227-234, 2016

11) Bradley EA, Ammash N, Martinez SC, et al:“Treat-to-close”:Non-repairable ASD-PAH in the adult:Results from the North American ASD-PAH（NAAP）Multicenter Registry. Int J Cardiol 291:127-133, 2019

12) Yan C, Pan X, Wan L, et al:Combination of F-ASO and Targeted Medical Therapy in Patients With Secundum ASD and Severe PAH. JACC Cardiovasc Interv 13:2024-2034, 2020

13) Hu Z, Xie B, Zhai X, et al:Midterm results of “treat and repair” for adults with non-restrictive ventricular septal defect and severe pulmonary hypertension. J Thorac Dis 7:1165-1173, 2015

14) Akagi S, Kasahara S, Sarashina T, et al:Treat-and-repair strategy is a feasible therapeutic choice in adult patients with severe pulmonary arterial hypertension associated with a ventricular septal defect:case series. Euro Heart J Case Rep 2:yty033, 2018

15) Galiè N, Beghetti M, Gatzoulis MA, et al:Bosentan therapy in patients with Eisenmenger syndrome:a multicenter, double-blind, randomized, placebo-controlled study. Circulation 114:48-54, 2006

16) Mulder BJ:Changing demographics of pulmonary arterial hypertension in congenital heart disease. Eur Respir Rev 19:308-313, 2010