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However, few reports have included the characteristics and treatments for patients with PoPH of Asian population; thus, we investigated the clinical characteristics, treatment, and survival of these patients in a Japanese cohort.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>Pulmonary arterial hypertension (PAH) has been included in the National Research Project on Intractable Disease in Japan; therefore, we extracted data of patients with PoPH from the forms of newly registered cases of the project from 2012 to 2013 (for 2 years), and updated cases of the project in 2013 (Study 1, n\u2009=\u200936 newly registered forms, n\u2009=\u200946 updated forms). Additionally, for Study 2, we performed a retrospective, observational cohort study at Chiba University Hospital (n\u2009=\u200911). We compared the characteristics between patients with PoPH and those with idiopathic\/heritable PAH (I\/H-PAH).<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>Both studies showed higher cardiac outputs (COs) and cardiac indexes (CIs), lower pulmonary vascular resistance (PVR), and less treated with combination therapy in patients with PoPH than those with I\/H-PAH. In Study 2, the overall and disease-specific survival between PoPH and I\/H-PAH were similar. Conversely, many patients (45%) had to change their PAH-specific medicine because of adverse effects.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusion<\/jats:title>\n                <jats:p>As seen in western countries, Japanese patients with PoPH showed higher COs and CIs, better exercise tolerance, and lower PVRs than patients with I\/H-PAH. Further studies are needed to improve PoPH treatments.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s12890-021-01452-3","type":"journal-article","created":{"date-parts":[[2021,4,9]],"date-time":"2021-04-09T12:33:54Z","timestamp":1617971634000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["The clinical characteristics, treatment, and survival of portopulmonary hypertension in Japan"],"prefix":"10.1186","volume":"21","author":[{"given":"Yukiko","family":"Takahashi","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Keiko","family":"Yamamoto","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Seiichiro","family":"Sakao","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takao","family":"Takeuchi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rika","family":"Suda","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nobuhiro","family":"Tanabe","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Koichiro","family":"Tatsumi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2021,3,16]]},"reference":[{"key":"1452_CR1","doi-asserted-by":"crossref","unstructured":"Swanson KL, Krowka MJ: Screen for portopulmonary hypertension, especially in liver transplant candidates. 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The need for informed consent was waived based on the Japanese legislation (Study 1). The Research Ethics Committee of Chiba University School of Medicine approved the study\u2019s protocol (approval number 2,584). Since 2009, all survivors provided written informed consent for a prospective cohort study (approval number 826). Deaths before 2008 were not required to provide written informed consent as per the guidelines for retrospective studies in Japan and the ethics committee of Chiba University Hospital permit it (Study 2). The study database was anonymized. All experiments were performed in accordance with the relevant guidelines and regulations.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors have no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"89"}}