循環器ジャーナル66巻3号

2018年07月発行

特集 肺高血圧症Cutting Edge Ⅴ．肺高血圧症のトピックスあるいはコントラバーシ

リバースリモデリングは達成可能か？

著者： 細川和也¹ 阿部弘太郎¹

所属機関： ¹九州大学病院循環器内科

ページ範囲：P.452 - P.459

Point
・肺動脈性肺高血圧症（PAH）は，中膜肥厚，求心性新生内膜増殖，叢状病変（plexiform lesion）といったリモデリング病変の進行を認める．
・高用量エポプロステノールも含めた多剤併用療法により肺動脈圧の低下を達成できた症例の予後が極めて良好であることが示されているが，リバースリモデリングが起こったか不明である．
・血行動態ストレス軽減によるリバースリモデリングの可能性について，PAH疾患モデル動物と実臨床例から解説する．

参考文献

1) Sitbon O, Jais X, Savale L, et al:Upfront triple combination therapy in pulmonary arterial hypertension:a pilot study. Eur Respir J 43:1691-1697, 2014

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 82:275-282, 2017

3) Galiè N, Humbert M, Vachiery JL, et al:2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension:The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology（ESC）and the European Respiratory Society（ERS）:Endorsed by:Association for European Paediatric and Congenital Cardiology（AEPC）, International Society for Heart and Lung Transplantation（ISHLT）. Eur Respir J 46:903-975, 2015

4) HEATH D, EDWARDS JE:The pathology of hypertensive pulmonary vascular disease;a description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects. Circulation 18:533-547, 1958

5) White RJ, Meoli DF, Swarthout RF, et al:Plexiform-like lesions and increased tissue factor expression in a rat model of severe pulmonary arterial hypertension. Am J Physiol Lung Cell Mol Physiol 293:L583-590, 2007

6) Tanaka Y, Shuster DP, Davis EC, et al:The role of vascular injury and hemodynamics in rat pulmonary artery remodeling. J Clin Invest 98:434-442, 1996

7) Rai PR, Cool CD, King JA, et al:The cancer paradigm of severe pulmonary arterial hypertension. Am J Respir Crit Care Med 178:558-564, 2008

8) Wagenvoort CA, Wangenvoort N, Draulans-Noe Y:Reversibility of plexogenic pulmonary arteriopathy following banding of the pulmonary artery. J Thorac Cardiovasc Sug 87:876-886, 1984

9) Levy NT, Liapis H, Eisenberg PR, et al:Pathologic regression of primary pulmonary hypertension in left native lung following right single-lung transplantation. J Heart Lung Transplant 20:381-384, 2001

10) Stenmark KR, Meyrick B, Galie N, et al:Animal models of pulmonary arterial hypertension:the hope for etiological discovery and pharmacological cure. Am J Physiol Lung Cell Mol Physiol. 297:L1013-1032, 2009

11) Abe K, Toba M, Alzoubi A, et al:Formation of plexiform lesions in experimental severe pulmonary arterial hypertension. Circulation 121:2747-2754, 2010

12) Abe K, Shioda M, Tanaka M, et al:Haemodynamic unloading reverses occlusive vascular lesions in severe pulmonary hypertension. Cardiovasc Res 111:16-25, 2016

13) Kodama Y, Abe K, Hosokawa K, et al:Reversal of diffuse patchy pattern in lung perfusion scan in a case of severe pulmonary arterial hypertension. Heart Lung 44:451-452, 2015

14) Piazza G, Goldhaber SZ:Chronic thromboembolic pulmonary hypertension. N Engl J Med 364:351-360, 2011

15) Yi ES, Kim H, Ahn H, et al:Distribution of obstructive intimal lesions and their cellular phenotypes in chronic pulmonary hypertension. A morphometric and immunohistochemical study. Am J Respir Crit Care Med 162:1577-1586, 2000

16) Hosokawa K, Abe K, Horimoto K, et al:Balloon pulmonary angioplasty relieves hemodynamic stress towards untreated-side pulmonary vasculature and ameliorates its resistance in patients with chronic thromboembolic pulmonary hypertension. EuroIntervention, 2018［Epub ahead of print］