title: [Clinical characteristics of 161 Chinese patients with idiopathic pulmonary arterial hypertension].
abstract: OBJECTIVE
  To investigate the clinical characteristics of patients with idiopathic pulmonary arterial hypertension (IPAH) in China.
  
  
  METHODS
  A total of 161 patients diagnosed as IPAH in Shanghai Pulmonary Hospital from June 2008 to June 2010 were retrospective analyzed.
  
  
  RESULTS
  The mean diagnostic age was (33 ± 15) years old and 70.2% patients were female. The median duration from symptoms onset to diagnostic right heart catheterization was 12 months. Incidence of NYHA class III to IV was 56.5% at diagnosis and the mean six minutes walk distance was limited to (398 ± 108) meters. Incidence of mild obstructive, restrictive and diffusing impairment in pulmonary function test was 7.8%, 42.2% and 82.2% patients with IPAH, respectively. Right heart catheterization demonstrated severe elevated mean pulmonary arterial pressure [(63 ± 17) mm Hg (1 mm Hg = 0.133 kPa)] and pulmonary vascular resistance index [(25 ± 12) Wood U/m(2)] in this patient cohort. The response rate of acute pulmonary vasoreactivity testing was 8.7% in this cohort. Compared with non-responders, responders to acute pulmonary vasoreactivity testing were younger and with less severe pulmonary hypertension. Among non-responders, 89% patients were treated by one specific anti-pulmonary arterial hypertension drug and 27% patients received combined anti-pulmonary arterial hypertension medications.
  
  
  CONCLUSIONS
  Young female was predominantly involved in patients with IPAH in China. The diagnosis of IPAH is often made at advanced disease stage and majority patients with IPAH received specific anti-pulmonary arterial hypertension therapies in this patient cohort.
authors.affiliation: Department of Cardio-Pulmonary Circulation, Tongji University School of Medicine, Shanghai, China.
authors.forename: Wei-bin
authors.initials: WB
authors.lastname: Jia
authors.forename: Xin
authors.initials: X
authors.lastname: Jiang
authors.forename: Xiang-rong
authors.initials: XR
authors.lastname: Zuo
authors.forename: Jing
authors.initials: J
authors.lastname: He
authors.forename: Qin-hua
authors.initials: QH
authors.lastname: Zhao
authors.forename: Rong
authors.initials: R
authors.lastname: Jiang
authors.forename: Zhi-cheng
authors.initials: ZC
authors.lastname: Jing
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D055815:Young Adult