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

成人先天性心疾患に合併する肺高血圧症の管理

著者： 齊藤暁人¹ 相馬桂¹

所属機関： ¹東京大学医学部附属病院循環器内科

ページ範囲：P.538 - P.542

POINT
●成人先天性心疾患に合併する肺高血圧症は，肺血流量増加に伴うシャント性の肺動脈性肺高血圧症が多い．
●構造的に未修復のシャント性肺動脈性肺高血圧症に対しては専門施設でのtreat ＆ repair strategyが検討される．
●肺動脈低形成による区域性肺動脈性肺高血圧症やFontan手術後の肺血管抵抗上昇に対する治療アプローチは今後の課題である．

参考文献

1）Shiina Y, Toyoda T, Kawasoe Y, et al. Prevalence of adult patients with congenital heart disease in Japan. Int J Cardiol 2011 ; 146 : 13-6.

2）Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease : A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation 2019 ; 139 : e698-800.

3）Naidu P, Grigg L, Zentner D. Mortality in adults with congenital heart disease. Int J Cardiol 2017 ; 245 : 125-30.

4）Diller GP, Kempny A, Alonso-Gonzalez R, et al. Survival Prospects and Circumstances of Death in Contemporary Adult Congenital Heart Disease Patients Under Follow-Up at a Large Tertiary Centre. Circulation 2015 ; 132 : 2118-25.

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

6）Arvind B, Relan J, Kothari SS. “Treat and repair” strategy for shunt lesions : a critical review. Pulm Circ 2020 ; 10 : 2045894020917885.

7）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 2019 ; 291 : 127-33.

8）Yao A. “Treat-and-Repair” Strategy for Atrial Septal Defect and Associated Pulmonary Arterial Hypertension. Circ J 2016 ; 80 : 69-71.

9）Dimopoulos K, Diller GP, Opotowsky AR, et al. Definition and Management of Segmental Pulmonary Hypertension. J Am Heart Assoc 2018 ; 7 : e008587.

10）Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2013 ; 62（25 Suppl） : D34-41.

11）Rosenzweig EB, Abman SH, Adatia I, et al. Paediatric pulmonary arterial hypertension : updates on definition, classification, diagnostics and management. Eur Respir J 2019 ; 53 : 1801916.

12）Mori H, Park IS, Yamagishi H, et al. Sildenafil reduces pulmonary vascular resistance in single ventricular physiology. Int J Cardiol 2016 ; 221 : 122-7.

13）Hill KD, Maharaj AR, Li JS, et al. A Randomized, Controlled Pharmacokinetic and Pharmacodynamics Trial of Ambrisentan After Fontan Surgery. Pediatr Crit Care Med 2020 ; 21 : e795-803.

14）Agnoletti G, Gala S, Ferroni F, et al. Endothelin inhibitors lower pulmonary vascular resistance and improve functional capacity in patients with Fontan circulation. J Thorac Cardiovasc Surg 2017 ; 153 : 1468-75.