title: Survival of Chinese patients with pulmonary arterial hypertension in the modern treatment era.
abstract: BACKGROUND
  In a previous study of Chinese patients with idiopathic pulmonary arterial hypertension (IPAH) in the nontargeted therapy era (defined as the time before 2006 when new pulmonary arterial hypertension-specific drugs were not available in China), we reported 1- and 3-year survival estimates of only 68% and 39%, respectively. However, it is not yet known whether the survival of patients with pulmonary arterial hypertension is improved in the modern treatment era (defined in China as after 2006).
  
  
  METHODS
  A retrospective cohort study was undertaken in 276 consecutive patients with newly diagnosed incident IPAH and connective tissue disease-related pulmonary arterial hypertension (CTDPAH) who were referred between 2007 and 2009. Baseline characteristics and survival rates in the two groups were compared.
  
  
  RESULTS
  The 1- and 3-year survival estimates were 92.1% and 75.1%, respectively, in patients with IPAH, and 85.4% and 53.6%, respectively, in patients with CTDPAH. Patients with CTDPAH had a significantly lower mean pulmonary artery pressure, more pericardial effusion, and more severe impairment of the diffusion capacity of the lung for carbon monoxide than patients with IPAH. A diagnosis of CTDPAH, World Health Organization functional class III or IV, single-breath diffusion capacity of the lung for carbon monoxide < 80% predicted, and the presence of pericardial effusion were independent predictors of mortality. The 1- and 3-year survival rates of male patients were 93.5% and 77.5%, respectively, in those with IPAH, and 71.1% and 47.4%, respectively, in those with CTDPAH.
  
  
  CONCLUSIONS
  The survival rates of patients with pulmonary arterial hypertension have improved in China in the modern treatment era, despite the high costs of treatment and financial constraints. However, the survival rates of patients with CTDPAH are inferior to those of patients with IPAH. Our study also indicates poorer survival rates in male patients with CTDPAH.
authors.affiliation: Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China.
authors.forename: Rui
authors.initials: R
authors.lastname: Zhang
authors.affiliation: Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China.
authors.forename: Li-Zhi
authors.initials: LZ
authors.lastname: Dai
authors.affiliation: Department of Respiratory Medicine, the First Affiliated Hospital of Nanjing Medical University, Nanjing, China.
authors.forename: Wei-Ping
authors.initials: WP
authors.lastname: Xie
authors.affiliation: Department of Cardiology, Xiangya Hospital of Central-South University, Changsha, China.
authors.forename: Zai-Xin
authors.initials: ZX
authors.lastname: Yu
authors.affiliation: Department of Cardiology, the First Affiliated Hospital of Harbin Medical University, Harbin, China.
authors.forename: Bing-Xiang
authors.initials: BX
authors.lastname: Wu
authors.affiliation: Department of Respiratory Medicine, Beijing Shijitan Hospital, Beijing, China.
authors.forename: Lei
authors.initials: L
authors.lastname: Pan
authors.affiliation: Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China.
authors.forename: Ping
authors.initials: P
authors.lastname: Yuan
authors.affiliation: Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China.
authors.forename: Xin
authors.initials: X
authors.lastname: Jiang
authors.affiliation: Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China.
authors.forename: Jing
authors.initials: J
authors.lastname: He
authors.affiliation: INSERM U999 Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine Béclère, Assistance Publique Hôpitaux de Paris, Université Paris-Sud 11, Clamart, France.
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
authors.affiliation: Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China. Electronic address: jingzhicheng@gmail.com.
authors.forename: Zhi-Cheng
authors.initials: ZC
authors.lastname: Jing
mesh_terms: D000328:Adult; D002681:China / Q000453:epidemiology; D015331:Cohort Studies; D003240:Connective Tissue Diseases / Q000150:complications; D004452:Echocardiography; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000188:drug therapy / Q000401:mortality* / Q000503:physiopathology; D008297:Male; D010490:Pericardial Effusion / Q000150:complications; D012129:Respiratory Function Tests; D015996:Survival Rate