摘要目的 明确先天性体肺分流型心脏病相关性肺动脉高压(PAH-CHD)在先天性心脏病患者中的比例及其影响因素.方法 自2007年5月至2008年12月,前瞻、连续纳入首次就诊于阜外心血管病医院并住院治疗的先天性心脏病患者,行多普勒超声心动图检查,以肺动脉收缩压＞40 mm Hg(1 mm Hg=0.133 kPa)作为判断PAH-CHD的标准,出现双向分流或右向左分流作为判断存在艾森曼格综合征的标准,比较合并与未合并PAH及艾森曼格综合征患者的临床特征.应用多因素Logistic回归分析先天性心脏病患者合并PAH的危险因素.结果 共纳入先天性心脏病患者692例,房间隔缺损、室间隔缺损和动脉导管未闭分别为187例(27.0％)、456例(65.9％)及49例(7.1％).PAH-CHD和艾森曼格综合征患者分别为329例(47.5％)和105例(15.2％),后者占PAH-CHD患者的31.9％.先天件心脏病患者发生肺动脉高压的可能性随着心脏间隔缺损内径的增加而增加.先天性心脏病患者合并肺动脉高压的可能性随着年龄的增长而增加( OR=1.04,P＜0.001),室间隔缺损和动脉导管未闭患者更易发生肺动脉高压(OR=2.78,P＜0.001和OR=2.50,P＜0.001).结论 肺动脉高压是先天性心脏病患者的常见合并症,室间隔缺损是引起PAH-CHD的最常见类型.年龄增加、室间隔缺损和动脉导管末闭是PAH发生的危险因素.

abstractsObjective To determine the prevalence and influencing factors of pulmonary arterial hypertension (PAH) in patients with congenital heart disease (CHD).Methods Between May 2007 and December 2008,a total of 692 CHD patients admitted into Fuwai Hospital were prospectively enrolled.Doppler echocardiograpy was employed to measure the level of pulmonary artery systolic pressure (PASP).PAH occurred when there was an increase in PASP ＞ 40 mm Hg (1 mm Hg =0.133 kPa) at rest.Eisenmenger syndrome was defined when there was a reversed (pulmonary-to-systemic) or bidirectional shunt.The clinical characteristics between the patients with/without PAH and Eisenmenger syndrome were compared and their risk factors analyzed with a multivariate Logistic model. Results The underlyingconditions included atrial septal defect ( n - 187,27.0％ ),ventricular septal defect ( n =456,65.9％ )and patent ductus arteriosus ( n =49,7.1％ ).The numbers of patients with PAH-CHD and Eisenmenger syndrome were 329 (47.5％ ) and 105 (15.2％) respectively.Amongthe PAH-CHD patients,31.9％ of them had Eisenmenger syndrome.The patients with large shunts were at an elevated risk of PAH.Logistic regression analysis showed that advanced age was an independent risk factor of PAH ( OR =1.04,P ＜0.001 ).Compared with atrial septal defect,ventricular septal defect and patent ductus arteriosus increased the risks of PAH (OR=2.78,P＜0.001 and OR=2.50,P＜0.001 respectively).Conclusions PAH is a common complication in CHD patients.And ventricular septal defect is the most common pathogenic type of PAH.Advanced age,ventricular septal defect and patent ductus arteriosus are the risk factors of PAH.