title: Prognostic factors in pulmonary arterial hypertension with Dana Point group 1.
abstract: OBJECTIVE
  To clarify the prognosis and prognostic factors in pulmonary arterial hypertension (PAH) patients in real-world medical practice in the Tokai area in Japan.
  
  
  METHODS
  We conducted a retrospective, multicenter observational study. The data of 81 patients diagnosed with Dana Point group 1 or 1' PAH was collected from January 2005 to January 2013. The primary outcome was all-cause death.
  
  
  RESULTS
  The patients consisted of 34 cases of idiopathic PAH (IPAH), 28 of connective tissue-associated PAH (CTD-PAH), 16 of congenital heart disease-associated PAH (CHD-PAH) and others. Mean age was 51 years and mean observation period was 46 months. The systolic blood pressure (BPs) was 117±23 mm Hg. Pericardial effusion was observed in 27.0% of patients. The mean right atrial pressure (mRAP) was 10.2±7.3 mm Hg. In the univariate Cox regression analysis, WHO-FCS III & IV, a cardiac index (CI)<2.5 L/min/m(2), and the presence of pericardial effusion at baseline were significantly associated with all-cause death. In the multivariate analysis, the pericardial effusion (HR 3.3, 95% CI 1.03-10.63, p=0.04) and mRAP (HR 3.2, 95% CI 1.03-9.83, p=0.04) or CI<2.5 L/min/m(2) (HR 3.89, 95% CI 1.05-14.45, p=0.04) were the independent predictors of mortality.
  
  
  CONCLUSIONS
  The presence of pericardial effusion and mRAP or CI<2.5 L/min/m(2) at diagnosis indicated high mortality.
authors.affiliation: Department of Cardiology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8560, Japan.
authors.forename: Shiro
authors.initials: S
authors.lastname: Adachi
authors.affiliation: Department of Advanced Medicine in Cardiopulmonary Disease, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8560, Japan.
authors.forename: Akihiro
authors.initials: A
authors.lastname: Hirashiki
authors.affiliation: Department of Cardiology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8560, Japan.
authors.forename: Yoshihisa
authors.initials: Y
authors.lastname: Nakano
authors.affiliation: Department of Cardiology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8560, Japan.
authors.forename: Shuzo
authors.initials: S
authors.lastname: Shimazu
authors.affiliation: Department of Cardiology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8560, Japan.
authors.forename: Toyoaki
authors.initials: T
authors.lastname: Murohara
authors.affiliation: Department of Advanced Medicine in Cardiopulmonary Disease, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8560, Japan. Electronic address: takahisa@med.nagoya-u.ac.jp.
authors.forename: Takahisa
authors.initials: T
authors.lastname: Kondo
mesh_terms: D001794:Blood Pressure; D065627:Familial Primary Pulmonary Hypertension / Q000150:complications / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology; D005260:Female; D006439:Hemodynamics; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D010490:Pericardial Effusion / Q000150:complications / Q000503:physiopathology; D011379:Prognosis; D016016:Proportional Hazards Models
keywords: Cardiac index; Mean right atrial pressure; Pericardial effusion; Pulmonary arterial hypertension; Right ventricular function