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

レジストリー構築の意義，重要性—日本発のエビデンスを国際的ガイドラインへ反映させるために

著者： 田村雄一¹

所属機関： ¹国際医療福祉大学医学部循環器内科

ページ範囲：P.460 - P.466

Point
・希少疾患におけるレジストリーは疾病予後だけではなくリスク評価や新たな治療戦略などの情報をもたらす．
・本邦のレジストリーによると十分な多剤併用療法を行うことで肺動脈性肺高血圧症（PAH）の予後は極めて良い．

参考文献

1) McGoon MD, Krichman A, Farber HW, et al:Design of the REVEAL registry for US patients with pulmonary arterial hypertension. Mayo Clin Proc 83:923-931, 2008

2) Humbert M, Sitbon O, Chaouat A, et al:Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation 122:156-163, 2010

3) Hoeper MM, Huscher D, Ghofrani HA, et al:Elderly patients diagnosed with idiopathic pulmonary arterial hypertension:results from the COMPERA registry. Int J Cardiol 168:871-880, 2013

4) Farber HW, Miller DP, Poms AD, et al:Five-Year outcomes of patients enrolled in the REVEAL Registry. Chest 148:1043-1054, 2015

5) Jing ZC, Xu XQ, Han ZY, et al:Registry and survival study in Chinese patients with idiopathic and familial pulmonary arterial hypertension. Chest 132:373-379, 2007

6) Ling Y, Johnson MK, Kiely DG, et al:Changing demographics, epidemiology and survival of incident pulmonary arterial hypertension:results from the pulmonary hypertension registry of the United Kingdom and Ireland. Am J Respir Crit Care Med 186:790-796, 2012

7) 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

8) D' Alonzo GE, Barst RJ, Ayres SM, et al:Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 115:343-349, 1991

9) Benza RL, Gomberg-Maitland M, Miller DP, et al:The REVEAL Registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension. Chest 141:354-362, 2012

10) Benza RL, Miller DP, Foreman AJ, et al:Prognostic implications of serial risk score assessments in patients with pulmonary arterial hypertension:a Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management（REVEAL）analysis. J Heart Lung Transplant 34:356-361, 2015

11) 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

12) Kylhammar D, Kjellström B, Hjalmarsson C, et al;SveFPH and SPAHR:A comprehensive risk stratification at early follow-up determines prognosis in pulmonary arterial hypertension. Eur Heart J, 2017［Epub ahead of print］

13) Hoeper MM, Kramer T, Pan Z, et al:Mortality in pulmonary arterial hypertension:prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. Eur Respir J 50:1700740, 2017

14) Hoeper MM, Markevych I, Spiekerkoetter E, et al:Goal-oriented treatment and combination therapy for pulmonary arterial hypertension. Eur Respir J 26:858-863, 2005

15) Sitbon O, Humbert M, Nunes H, et al:Long-term intravenous epoprostenol infusion in primary pulmonary hypertension:prognostic factors and survival. J Am Coll Cardiol 40:780-788, 2002

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

17) Galiè N, Barberà JA, Frost AE, et al:Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension. N Engl J Med 373:834-844, 2015

18) Montani D, Bergot E, Günther S, et al:Pulmonary arterial hypertension in patients treated by dasatinib. Circulation 125:2128-2137, 2012

19) Guignabert C, Phan C, Seferian A, et al:Dasatinib induces lung vascular toxicity and predisposes to pulmonary hypertension. J Clin Invest 126:3207-3218, 2016

20) Perros F, Günther S, Ranchoux B, et al:Mitomycin-Induced Pulmonary Veno-Occlusive Disease:Evidence From Human Disease and Animal Models. Circulation 132:834-847, 2015