{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T18:03:38Z","timestamp":1792692218912,"version":"4.5.0"},"reference-count":30,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2012,7,1]],"date-time":"2012-07-01T00:00:00Z","timestamp":1341100800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2012,7,1]],"date-time":"2012-07-01T00:00:00Z","timestamp":1341100800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Pulm. circ."],"published-print":{"date-parts":[[2012,7]]},"abstract":"<jats:p>The survival rates of Chinese patients with idiopathic pulmonary arterial hypertension (IPAH) and familial pulmonary arterial hypertension (PAH) on conventional therapy at 1 and 3 years were 68.0% and 38.9%, respectively. Our aim was to update recent knowledge on the demographics, clinical course, hemodynamic features, disease management, and survival of adult patients with IPAH. This retrospective and observational study was conducted at the largest tertiary referral center in China. Ninety patients with IPAH who underwent initial evaluation at Fu Wai Hospital from January 2006 through November 2009 were retrospectively enrolled. The primary outcome was death. Statistical analyses used included independent sample <jats:italic>t<\/jats:italic> test, nonparametric test, Kaplan\u2010Meier method, and Cox proportional hazards analysis. Of the 90 patients enrolled, the median age was 32 years with female predominance. The median interval from onset of symptoms to diagnosis was 14\u00a0months. Patients exhibited severe exercise limitation and hemodynamic abnormalities at diagnosis. Only 10.6% had a positive vasoreactivity test, while calcium channel blockers were given to 22.2% of patients. Fifty\u2010nine patients (65.6%) received PAH\u2010targeted therapies during follow\u2010up. Our survival rates of 84.1%, 73.7%, and 70.6% at 1\u2010, 2\u2010, and 3\u2010years compared favorably with predicted survival based on the National Institutes of Health equation which showed 1\u2010, 2\u2010, and 3\u2010years survival rates of 67.7%, 55.9%, and 47%, respectively. For the patients receiving conventional therapy solely, the 1\u2010 and 3\u2010years survival rates were 67.0% and 49.3%, respectively. Younger age, lower body mass index, presence of pericardial effusion, and absence of PAH\u2010targeted therapy were independently associated with mortality. We concluded that patients with IPAH were still diagnosed too late, and while survival rates have improved in the modern treatment era, there is still room for improvement.<\/jats:p>","DOI":"10.4103\/2045-8932.101655","type":"journal-article","created":{"date-parts":[[2012,9,26]],"date-time":"2012-09-26T21:03:51Z","timestamp":1348693431000},"page":"373-378","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":14,"title":["The Impact of Pulmonary Arterial Hypertension\u2010Targeted Therapy on Survival in Chinese Patients with Idiopathic Pulmonary Arterial Hypertension"],"prefix":"10.1002","volume":"2","author":[{"given":"Wei\u2010Jie","family":"Zeng","sequence":"first","affiliation":[{"name":"Department of Cardiology Cardiovascular Institute and Fu Wai Hospital Chinese Academy of Medical Sciences and Peking Union Medical College Beijing China"},{"name":"Department of Cardiology The First Affiliated Hospital of Jinan University Guangzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yun\u2010Juan","family":"Sun","sequence":"additional","affiliation":[{"name":"Department of Cardiology Cardiovascular Institute and Fu Wai Hospital Chinese Academy of Medical Sciences and Peking Union Medical College Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qing","family":"Gu","sequence":"additional","affiliation":[{"name":"Department of Cardiology Cardiovascular Institute and Fu Wai Hospital Chinese Academy of Medical Sciences and Peking Union Medical College Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chang\u2010Ming","family":"Xiong","sequence":"additional","affiliation":[{"name":"Department of Cardiology Cardiovascular Institute and Fu Wai Hospital Chinese Academy of Medical Sciences and Peking Union Medical College Beijing 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