{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T20:04:03Z","timestamp":1790193843319,"version":"4.1.0"},"reference-count":18,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2022,12,13]],"date-time":"2022-12-13T00:00:00Z","timestamp":1670889600000},"content-version":"vor","delay-in-days":73,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pulm. circ."],"published-print":{"date-parts":[[2022,10]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Pulmonary veno\u2010occlusive disease (PVOD) or pulmonary capillary hemangiomatosis (PCH) is a rare subtype of pulmonary hypertension with dismal prognosis. Limited data are available on the efficacy and safety of orally administered pulmonary vasodilators for PVOD\/PCH. Whether and how systemic sclerosis (SSc) affects the clinical outcomes of PVOD\/PCH is also unknown. This study aimed to determine the clinical and hemodynamic efficacy and safety of oral pulmonary vasodilators for PVOD\/PCH and clarify the possible effects of SSc on the clinical presentation of PVOD\/PCH. We retrospectively analyzed the clinical data of 15 patients with PVOD\/PCH treated with oral pulmonary vasodilators in our department since 2001. Six of them had SSc. Oral pulmonary vasodilators were administered either as single agents (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200910) or in combination (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u20095). Treatment improved the functional class of five patients, and pulmonary arterial pressure and pulmonary vascular resistance decreased by 10\u2009\u00b1\u200912\u2009mmHg and 36\u2009\u00b1\u200919%, respectively (\n                    <jats:italic>p<\/jats:italic>\n                    \u2009&lt;\u20090.05 for both,\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200913), whereas pulmonary edema developed in three patients. The mean survival was 3.9 years, and the 1\u2010 and 3\u2010year survival rates were 93% and 65%, respectively. The clinical presentation, including survival, was similar between patients with and without SSc. In our PVOD\/PCH cohort, oral pulmonary vasodilators caused pulmonary edema in 20% of patients, but more than 80% of patients experienced significant pulmonary vasodilatory effects, and the overall prognosis was better than that previously reported. SSc does not adversely affect the clinical outcomes of PVOD\/PCH.\n                  <\/jats:p>","DOI":"10.1002\/pul2.12168","type":"journal-article","created":{"date-parts":[[2022,12,3]],"date-time":"2022-12-03T02:55:18Z","timestamp":1670036118000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Efficacy and safety of oral pulmonary vasodilators in pulmonary veno\u2010occlusive disease"],"prefix":"10.1002","volume":"12","author":[{"given":"Junichi","family":"Nakamura","sequence":"first","affiliation":[{"name":"Department of Respiratory Medicine, Faculty of Medicine Hokkaido University Sapporo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0196-9572","authenticated-orcid":false,"given":"Ichizo","family":"Tsujino","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Faculty of Medicine Hokkaido University Sapporo Japan"},{"name":"Division of Respiratory and Cardiovascular Innovative Research, Faculty of Medicine Hokkaido University Sapporo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hideki","family":"Shima","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Faculty of Medicine Hokkaido University Sapporo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshitaka","family":"Nakaya","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Faculty of Medicine Hokkaido University Sapporo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ayako","family":"Sugimoto","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Faculty of Medicine Hokkaido University Sapporo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takahiro","family":"Sato","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Faculty of Medicine Hokkaido University Sapporo Japan"},{"name":"Division of Respiratory and Cardiovascular Innovative Research, Faculty of Medicine Hokkaido University Sapporo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Taku","family":"Watanabe","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Faculty of Medicine Hokkaido University Sapporo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroshi","family":"Ohira","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Faculty of Medicine Hokkaido University Sapporo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masaru","family":"Suzuki","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Faculty of Medicine Hokkaido University Sapporo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satonori","family":"Tsuneta","sequence":"additional","affiliation":[{"name":"Department of Diagnostic and Interventional Radiology Hokkaido University Hospital Sapporo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryo","family":"Hisada","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine Hokkaido University  Sapporo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masaru","family":"Kato","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine Hokkaido University  Sapporo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoshi","family":"Konno","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Faculty of Medicine Hokkaido University Sapporo Japan"},{"name":"Division of Respiratory and Cardiovascular Innovative Research, Faculty of Medicine Hokkaido University Sapporo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,12,13]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00026-2016"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01032-2015"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.67.4.487"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(16)30438-6"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.1253\/circj.CJ-66-0158"},{"key":"e_1_2_12_7_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01913-2018"},{"key":"e_1_2_12_8_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00017809"},{"key":"e_1_2_12_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.resinv.2018.10.004"},{"key":"e_1_2_12_10_1","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0000000000027334"},{"key":"e_1_2_12_11_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12890-019-1031-3"},{"key":"e_1_2_12_12_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00168-2018"},{"key":"e_1_2_12_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.34501"},{"key":"e_1_2_12_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.38098"},{"key":"e_1_2_12_15_1","doi-asserted-by":"publisher","DOI":"10.1253\/circj.cj-11-0973"},{"key":"e_1_2_12_16_1","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0b013e31818193bb"},{"key":"e_1_2_12_17_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kex351"},{"key":"e_1_2_12_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-020-04953-4"},{"key":"e_1_2_12_19_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00106008"}],"container-title":["Pulmonary Circulation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pul2.12168","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/pul2.12168","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pul2.12168","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,17]],"date-time":"2023-08-17T19:08:39Z","timestamp":1692299319000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/pul2.12168"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,10]]},"references-count":18,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2022,10]]}},"alternative-id":["10.1002\/pul2.12168"],"URL":"https:\/\/doi.org\/10.1002\/pul2.12168","archive":["Portico"],"relation":{},"ISSN":["2045-8940","2045-8940"],"issn-type":[{"value":"2045-8940","type":"print"},{"value":"2045-8940","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,10]]},"assertion":[{"value":"2022-08-29","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-12-02","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-12-13","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e12168"}}