Ni Xin-hai, Tao Xin-cao, Zhang Jian-qiang, He Jian-guo, Liu Zhi-hong, Xiong Chang-ming, Luo Qin, Zhang Hong-liang, Liu Yang-qing
Center of Diagnosis and Treatment of Pulmonary Vascular Diseases, Fu Wai Cardiovascular Hospital, Beijing 100037, China.
Zhonghua Xin Xue Guan Bing Za Zhi. 2009 Aug;37(8):708-11.
To analyze the characteristics of idiopathic pulmonary arterial hypertension (IPAH) and predict the prognosis of IPAH by Echocardiogram and Right Heart Catheterization.
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.
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.
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.
分析特发性肺动脉高压（IPAH）的特征，并通过超声心动图和右心导管检查预测IPAH的预后。
回顾性分析2003年1月至2007年6月在阜外医院确诊为IPAH的108例患者，并随访至2008年6月，统计分析软件为SPSS 13.0。
108例IPAH患者的平均年龄为（32.5±12.6）岁，男女比例为2.86:1，从出现症状到诊断的平均时间为（34.9±36.3）个月，晕厥、肝肿大、咯血和下肢水肿的发生率分别为32.4%、27.8%、15.7%和14.8%。共94例患者（87.0%）随访了（27.6±14.7）个月，42例患者（38.9%）死于右心衰竭，诊断后平均死亡时间为（11.6±9.4）个月，1年、2年和3年生存率分别为74%、58%和44%。在单因素分析中，以下变量可预测生存：收缩压（SBP）（P<0.01，RR=0.937）、脉压（PP）（P<0.01，RR=0.930）、左心室舒张末期内径（LVEDD）（P=0.004，RR=0.924）、右心室舒张末期内径（RVEDD）（P=0.029，RR=1.04）、RVEDD/LVEDD（P=0.003，RR=3.222）、超声心动图测量的肺动脉收缩压（PASP）（P=0.002，RR=1.022）、右心导管测量的PASP（P=0.030，RR=1.026）、平均肺动脉压（mPAP）（P=0.031，RR=1.037）和肺血管阻力（PVR）（P=0.019，RR=1.00）。多因素分析中，PP（P<0.01，RR=0.923）、RVEDD/LVEDD（P=0.006，RR=3.126）以及超声心动图测量的PASP（P=0.002，RR=1.022）、右心导管测量的PASP、mPAP、PVR（P=0.017，RR=1.00）具有预测性。
IPAH易发生于年轻女性，预后较差，由于症状不特异，IPAH早期诊断困难，晕厥、肝肿大和下肢水肿可预测IPAH的严重程度，右心导管测量的PVR、PP和PASP是IPAH的独立预测因素。
