摘要目的：调查先天性心脏病（ CHD）患者中肺动脉高压（ PAH）的发生率并分析其可能的危险因素。方法连续纳入2007年5月至2008年12月首次在阜外心血管病医院住院诊治的CHD患者704例，采集临床资料，全部患者接受右心导管检查，以右心导管测定的肺动脉平均压≥25 mmHg（1 mmHg＝0.133 kPa）作为PAH的诊断标准。用多因素Logistic回归分析PAH存在的独立影响因素。结果704例患者中男319例，女385例，中位年龄5岁；包括房间隔缺损185例、室间隔缺损452例、动脉导管未闭48例及上述畸形合并存在者19例；其中，合并PAH者280例（39.8％），未合并PAH者424例（60.2％）；年龄（OR＝1.017，95％CI：1.001～1.033，P＝0.040）、体动脉收缩压（OR＝0.959，95％CI：0.944～0.975，P＜0.001）、室间隔缺损（OR＝4.402，95％CI：2.615～7.411，P＜0.001）、动脉导管未闭（OR＝6.417，95％CI：3.079～13.373，P＜0.001）、复合畸形（OR＝10.513，95％CI：3.641～30.355，P＜0.001）是CHD患者发生PAH的独立危险因素。结论 PAH是CHD常见的并发症，年龄、体动脉收缩压及CHD类型是发生PAH的独立危险因素。

abstractsObjective To explore the prevalence of pulmonary arterial hypertension ( PAH ) in congenital heart disease (CHD) and analyze its independent risk factors.Methods During May 2007 to December 2008, a total of 704 consecutive CHD patients initially admitted into Fuwai Hospital were recruited.Their baseline data were collected and right heart catheterization ( RHC) was performed.Those with a mean pulmonary arterial pressure ( mPAP) ≥25 mmHg (1 mmHg=0.133 kPa) during RHC were diagnosed as PAH.Multiple Logistic regression was performed to analyze the independent risk factors of PAH.Results There were 319 males and 385 females with a median age of 5 years.The causes included atrial septal defect (n=185), ventricular septal defect (VSD, n=452), patent ductus arteriosus (PDA, n=48) and a combinations of the above lesions (n=19).And 280 (39.8%) CHD patients had PAH.Logistic regression analysis revealed that age , systemic artery systolic blood pressure , VSD, PDA and combined lesions were independent risk factors of PAH in CHD ( age: OR =1.017, 95%CI: 1.001 -1.033, P=0.040; systemic artery systolic blood pressure: OR=0.959, 95%CI: 0.944 -0.975, P<0.001;VSD:OR=4.402, 95%CI:2.615 -7.411, P<0.001; PDA: OR=6.417, 95%CI:3.079 -13.373, P <0.001; combined lesions: OR =10.513, 95%CI: 3.641 -30.355, P <0.001 ).Conclusions PAH is a common complication of CHD.Age, peripheral artery systolic blood pressure and type of lesion are independent risk factors of PAH in CHD.