[The prognosis study of 108 idiopathic pulmonary arterial hypertension patients].
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.
Ni
Xin-hai
XH
Center of Diagnosis and Treatment of Pulmonary Vascular Diseases, Fu Wai Cardiovascular Hospital, Beijing 100037, China. xin-haini@163.com
Tao
Xin-cao
XC
Zhang
Jian-qiang
JQ
He
Jian-guo
JG
Liu
Zhi-hong
ZH
Xiong
Chang-ming
CM
Luo
Qin
Q
Zhang
Hong-liang
HL
Liu
Yang-qing
YQ
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