ABSTRACT
<p><b>OBJECTIVE</b>To investigate the clinical characteristics of patients with idiopathic pulmonary arterial hypertension (IPAH) in China.</p><p><b>METHODS</b>A total of 161 patients diagnosed as IPAH in Shanghai Pulmonary Hospital from June 2008 to June 2010 were retrospective analyzed.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSIONS</b>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.</p>