title: [The prognosis study of 108 idiopathic pulmonary arterial hypertension patients].
abstract: OBJECTIVE
  To analyze the characteristics of idiopathic pulmonary arterial hypertension (IPAH) and predict the prognosis of IPAH by Echocardiogram and Right Heart Catheterization.
  
  
  METHODS
  108 IPAH patients who was diagnosed as IPAH in Fuwai hospital from Jan 2003 to Jun 2007 were retrospectively analyzed and followed up to June 2008, the statistical analysis software was SPSS 13.0.
  
  
  RESULTS
  The mean age of the 108 IPAH patients was (32.5 +/- 12.6) years, the ratio of female and male was 2.86:1, the average time from appearing symptom to diagnosis was (34.9 +/- 36.3) months, the incidence of syncope, hepatomegaly, hemoptysis and Edema of lower extremity was 32.4%, 27.8%, 15.7% and 14.8% respectively. A total of 94 patients (87.0%) were followed up for (27.6 +/- 14.7) months, forty-two patients (38.9%) died of right heart failure, the mean death time was (11.6 +/- 9.4) months after diagnosis, the survival rate of 1, 2 and 3 years was 74%, 58% and 44% respectively. These following variables could predict survival in univariate analysis: Systolic Blood Pressure (SBP) (P < 0.01, RR = 0.937), pulse Pressure (PP) (P < 0.01, RR = 0.930), left ventricular End-Diastolic Dimension (LVEDD) (P = 0.004, RR = 0.924), right ventricular End-Diastolic Dimension (RVEDD) (P = 0.029, RR = 1.045), RVEDD/LVEDD (P = 0.003, RR = 3.222), Pulmonary Arterial Systolic Pressure (PASP) measured by echocardiogram (P = 0.002, RR = 1.022), PASP (P = 0.030, RR = 1.026), mean Pulmonary Arterial Pressure (mPAP) (P = 0.031, RR = 1.037) and Pulmonary Vascular Resistance (PVR) measured by right heart catheter (P = 0.019, RR = 1.001). in multivariate analysis, PP (P < 0.01, RR = 0.923), RVEDD/LVEDD (P = 0.006, RR = 3.126) and PASP measured by Echocardiogram (P = 0.002, RR = 1.022), PASP, mPAP, PVR measured by right heart catheter (P = 0.017, RR = 1.001) were predictive.
  
  
  CONCLUSIONS
  IPAH was prone to attack young women with a poor prognosis, the diagnosis of IPAH at an early stage was difficult because of nonspecific symptom, syncope, hepatomegaly and Edema of lower extremity can predict the severity of IPAH, PVR measured by right heart catheterization, PP and PASP were independently predictive factors for IPAH.
authors.affiliation: Center of Diagnosis and Treatment of Pulmonary Vascular Diseases, Fu Wai Cardiovascular Hospital, Beijing 100037, China. xin-haini@163.com
authors.forename: Xin-hai
authors.initials: XH
authors.lastname: Ni
authors.forename: Xin-cao
authors.initials: XC
authors.lastname: Tao
authors.forename: Jian-qiang
authors.initials: JQ
authors.lastname: Zhang
authors.forename: Jian-guo
authors.initials: JG
authors.lastname: He
authors.forename: Zhi-hong
authors.initials: ZH
authors.lastname: Liu
authors.forename: Chang-ming
authors.initials: CM
authors.lastname: Xiong
authors.forename: Qin
authors.initials: Q
authors.lastname: Luo
authors.forename: Hong-liang
authors.initials: HL
authors.lastname: Zhang
authors.forename: Yang-qing
authors.initials: YQ
authors.lastname: Liu
mesh_terms: D000328:Adult; D006328:Cardiac Catheterization; D018618:Echocardiography, Doppler, Color; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000628:therapy; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D055815:Young Adult