1 Galiè N, Barbera JA, Frost AE, et al. Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension. N Engl J Med. 2015;373(9):834–844. doi:10.1056/NEJMoa1413687.
2 Sitbon O, Jaïs X, Savale L, et al. Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study. Eur Respir J. 2014;43(6):1691–1697. doi:10.1183/09031936.00116313.
3 Ogawa A, Satoh T, Tamura Y, Fukuda K, Matsubara H. Survival of Japanese Patients With Idio- pathic/Heritable Pulmonary Arterial Hypertension. Am J Cardiol. 2017;119(9):1479–1484. doi:10.1016/j. amjcard.2017.01.015.
4 Kondo T, Okumura M, Adachi S, Murohara T. Pulmonary Hypertension: Diagnosis, Management, and Treatment. Nagoya J Med Sci. 2019;81(1):19–30. doi:10.18999/nagjms.81.1.19.
5 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 Heart J. 2016;37(1):67–119. doi:10.1093/eurheartj/ehv317.
6 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. 2015;46(4):903–975. doi:10.1183/13993003.01032-2015.
7 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. 2017;50(2): 1700740. doi:10.1183/13993003.00740-2017.
8 Boucly A, Weatherald J, Savale L, et al. Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension. Eur Respir J. 2017;50(2): 1700889. doi:10.1183/13993003.00889-2017.
9 Weatherald J, Sitbon O, Humbert M. Validation of a risk assessment instrument for pulmonary arterial hypertension. Eur Heart J. 2018;39(47):4182–4185. doi:10.1093/eurheartj/ehx301.
10 Wasserman K, Van Kessel AL, Burton GG. Interaction of physiological mechanisms during exercise. J Appl Physiol. 1967;22(1):71–85. doi:10.1152/jappl.1967.22.1.71.
11 Zafrir B. Exercise training and rehabilitation in pulmonary arterial hypertension: rationale and current data evaluation. J Cardiopulm Rehabil Prev. 2013;33(5):263–273. doi:10.1097/HCR.0b013e3182a0299a.
12 Breda AP, Pereira de Albuquerque AL, Jardim C, et al. Skeletal muscle abnormalities in pulmonary arterial hypertension. PLoS One. 2014;9(12):e114101. doi:10.1371/journal.pone.0114101.
13 Liang F, Yang S, Yao L, Belardinelli L, Shryock J. Ambrisentan and tadalafil synergistically relax endothelin-induced contraction of rat pulmonary arteries. Hypertension. 2012;59(3):705–711. doi:10.1161/ HYPERTENSIONAHA.111.182261.
14 Hirashiki A, Adachi S, Nakano Y, et al. Effects of bosentan on peripheral endothelial function in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. Pulm Circ. 2016;6(2):168–173. doi:10.1086/685715.
15 Yamazaki M, Sato H. Human Walking With Reference to Step Length, Cadence, Speed and Energy Expenditure[in Japanese]. J Anthrop Soc. Nippon. 1990;98(4):385–401.doi:10.1537/ase1911.98.385.
16 Miyamoto S, Nagaya N, Satoh T, et al. Clinical correlates and prognostic significance of six-minute walk test in patients with primary pulmonary hypertension. Comparison with cardiopulmonary exercise testing. Am J Respir Crit Care Med. 2000;161(2 Pt 1):487–492. doi:10.1164/ajrccm.161.2.9906015.
17 de Man FS, Handoko ML, Groepenhoff H, et al. Effects of exercise training in patients with idiopathic pulmonary arterial hypertension. Eur Respir J. 2009;34(3):669–675. doi:10.1183/09031936.00027909.
18 Mereles D, Ehlken N, Kreuscher S, et al. Exercise and respiratory training improve exercise capacity and quality of life in patients with severe chronic pulmonary hypertension. Circulation. 2006;114(14):1482–1489. doi:10.1161/CIRCULATIONAHA.106.618397.
19 Fox BD, Kassirer M, Weiss I, et al. Ambulatory rehabilitation improves exercise capacity in patients with pulmonary hypertension. J Card Fail. 2011;17(3):196–200. doi:10.1016/j.cardfail.2010.10.004.