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D\u2019Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, et al. Survival in patients with primary pulmonary hypertension: Results from a national prospective registry. <i>Ann Intern Med<\/i> 1991; 115: 343\u2013349.","DOI":"10.7326\/0003-4819-115-5-343"},{"key":"2","doi-asserted-by":"crossref","unstructured":"2. Ogawa A, Ejiri K, Matsubara H. Long-term patient survival with idiopathic\/heritable pulmonary arterial hypertension treated at a single center in Japan. <i>Life Sci<\/i> 2014; 118: 414\u2013419.","DOI":"10.1016\/j.lfs.2014.01.077"},{"key":"3","doi-asserted-by":"crossref","unstructured":"3. Ogawa A, Satoh T, Tamura Y, Fukuda K, Matsubara H. Survival of Japanese patients with idiopathic\/heritable pulmonary arterial hypertension. <i>Am J Cardiol<\/i> 2017; 119: 1479\u20131484.","DOI":"10.1016\/j.amjcard.2017.01.015"},{"key":"4","doi-asserted-by":"crossref","unstructured":"4. Gali\u00e8 N, Barber\u00e0 JA, Frost AE, Ghofrani HA, Hoeper MM, McLaughlin VV, et al. Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension. <i>N Engl J Med<\/i> 2015; 373: 834\u2013844.","DOI":"10.1056\/NEJMoa1413687"},{"key":"5","doi-asserted-by":"crossref","unstructured":"5. Pulido T, Adzerikho I, Channick RN, Delcroix M, Gali\u00e8 N, Ghofrani HA, et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. <i>N Engl J Med<\/i> 2013; 369: 809\u2013818.","DOI":"10.1056\/NEJMoa1213917"},{"key":"6","doi-asserted-by":"crossref","unstructured":"6. Sitbon O, Channick R, Chin KM, Frey A, Gaine S, Gali\u00e8 N, et al. Selexipag for the treatment of pulmonary arterial hypertension. <i>N Engl J Med<\/i> 2015; 373: 2522\u20132533.","DOI":"10.1056\/NEJMoa1503184"},{"key":"7","doi-asserted-by":"crossref","unstructured":"7. Fox BD, Shtraichman O, Langleben D, Shimony A, Kramer MR. Combination therapy for pulmonary arterial hypertension: A systematic review and meta-analysis. <i>Can J Cardiol<\/i> 2016; 32: 1520\u20131530.","DOI":"10.1016\/j.cjca.2016.03.004"},{"key":"8","doi-asserted-by":"crossref","unstructured":"8. Galie N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, 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). <i>Eur Heart J<\/i> 2016; 37: 67\u2013119.","DOI":"10.1093\/eurheartj\/ehv317"},{"key":"9","doi-asserted-by":"crossref","unstructured":"9. Yoshida S, Shirato K, Shimamura R, Nakahara N, Iwase T, Nakajima H. Efficacy, safety, and pharmacokinetics of ambrisentan in Japanese adults with pulmonary arterial hypertension. <i>Curr Med Res Opin<\/i> 2011; 27: 1827\u20131834.","DOI":"10.1185\/03007995.2011.605440"},{"key":"10","doi-asserted-by":"crossref","unstructured":"10. Takahashi T, Hayata S, Kobayashi A, Onaka Y, Ebihara T, Hara T. Surveillance on the safety and efficacy of ambrisentan (Volibris tablet 2.5 mg) in patients with pulmonary arterial hypertension in real clinical practice: Post-marketing surveillance (Interim analysis report). <i>Clin Drug Investig<\/i> 2018; 38: 219\u2013229.","DOI":"10.1007\/s40261-017-0602-4"},{"key":"11","unstructured":"11. Fukuda K, Itoh H, Ueda H, Ogino H, Kuwana M, Sato T, et al. Guidelines for treatment of pulmonary hypertension (JCS2017\/JPCPHS 2017). http:\/\/www.j-circ.or.jp\/guideline\/pdf\/JCS2017_fukuda_h.pdf#search=%27%E8%82%BA%E9%AB%98%E8%A1%80%E5%9C%A7%E7%97%87+%E3%82%AC%E3%82%A4%E3%83%89%E3%83%A9%E3%82%A4%E3%83%B3%27 (accessed February 8, 2019) (in Japanese)."},{"key":"12","unstructured":"12. Bachetti C, Manes A, Dardi F, Palazzini M, Mazzanti G, Rinnaldi A, et al. Comparison between initial combination therapy and initial monotherapy in pulmonary arterial hypertension: A single centre blinded evaluation of patients enrolled in the AMBITION study. <i>Am J Respir Crit Care Med<\/i> 2015; 191: A4779."},{"key":"13","doi-asserted-by":"crossref","unstructured":"13. Hoeper MM, McLaughlin VV, Barber\u00e1 JA, Frost AE, Ghofrani HA, Peacock AJ, et al. Initial combination therapy with ambrisentan and tadalafil and mortality in patients with pulmonary arterial hypertension: A secondary analysis of the results from the randomised, controlled AMBITION study. <i>Lancet Respir Med<\/i> 2016; 4: 894\u2013901.","DOI":"10.1016\/S2213-2600(16)30307-1"},{"key":"14","doi-asserted-by":"crossref","unstructured":"14. Tamura Y, Kumamaru H, Satoh T, Miyata H, Ogawa A, Tanabe N, et al. Effectiveness and outcome of pulmonary arterial hypertension-specific therapy in Japanese patients with pulmonary arterial hypertension. <i>Circ J<\/i> 2018; 82: 275\u2013282.","DOI":"10.1253\/circj.CJ-17-0139"},{"key":"15","doi-asserted-by":"crossref","unstructured":"15. Sitbon O, Sattler C, Bertoletti L, Savale L, Cottin V, Ja\u00efs X, et al. 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