108例特发性肺动脉高压预后的研究

The prognosis study of 108 idiopathic pulmonary arterial hypertension patients

目的分析特发性肺动脉高压（IPAH）的发病特点并评价超声心动图和右心导管检查指标在判断IPAH预后中的作用。方法回顾性分析2003年1月到2007年6月在阜外心血管病医院住院并诊断为IPAH的108例患者，对这些患者生存情况进行随访，数据分析采用SPSS13．0软件包。结果108例IPAH患者中女性和男性比为2．86：1，确诊的平均年龄为（32．5±12．6）岁，出现症状到确诊的平均时间为（34．9±36．3）个月，晕厥、肝脏肿大、咯血和下肢水肿的发生率分别为32．4％、27．8％、15．7％和14．8％。随访截止时间为2008年6月，平均随访（27．6±14．7）个月，随访到94例（87．0％），42例（38．9％）在随访期间死于右心衰竭，死亡病例平均存活时间为确诊后（11．6±9．4）个月，出院后1、2、3年的生存率分别为74％、58％、44％。单因素分析显示对生存有影响的指标有：肱动脉收缩压（P〈0．01，RR=0．937）、脉压（P〈0．01，RR=0．930）、右左心室舒张末径比（P=0．003，RR=3．222）、超声测得的肺动脉收缩压（P=0．002，RR=1．022）、右心导管检测的肺动脉收缩压（P=0．030，RR=1．026）、肺动脉平均压（P=0．031，RR=1．037）和全肺血管阻力（P=0．019，RR=1．001）；多因素COX回归分析显示脉压（P〈0．01，RR=0．923）、超声测得的右左心室舒张末径比值（P=0．006，RR=3．126）和肺动脉收缩压（P=0．002，RR=1．022）、右心导管检测得的肺动脉收缩压（P=0．016，解=1．033）、肺动脉平均压（P=0．038，RR=1．038）和全肺血管阻力（P=0．017，RR=1．001）是IPAH患者预后的独立预测因子。结论IPAH多发生于中青年女性，症状缺乏特异性，不易确诊且预后极差，晕厥、肝脏肿大和下肢水肿提示患者病情较重，晕厥最常见，超声心动图是测量肺动脉压力较准确的方法，右心导管测得的全肺血管阻力、脉压和超声测定的肺动脉收缩压是IPAH患者预后的独立预测因子。

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. O. Result 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. Conclusion 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.