Clinical characteristics of 161 Chinese patients with idiopathic pulmonary arterial hypertension
10.3760/cma.j.issn.0253-3758.2012.08.006
- VernacularTitle:特发性肺动脉高压161例临床特点及治疗情况分析
- Author:
Wei-Bin JIA
1
;
Xin JIANG
;
Xiang-Rong ZUO
;
Jing HE
;
Qin-Hua ZHAO
;
Rong JIANG
;
Zhi-Cheng JING
Author Information
1. 山东省临清市人民医院
- Keywords:
Hypertension,pulmonary;
Disease attributes;
Drug therapy
- From:
Chinese Journal of Cardiology
2012;40(8):652-656
- CountryChina
- Language:Chinese
-
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/m2 ] 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.