目的：比较系统性红斑狼疮（SLE）相关肺动脉高压（SLE-APAH）及特发性肺动脉高压（IPAH）患者的血流动力学和右心功能以及二者对雾化吸入伊洛前列素的反应。方法入选北京协和医院2010年11月至2012年9月临床诊断SLE-APAH和IPAH的患者58例，所有患者均进行右心导管检查（RHC）及经胸超声心动图检查（TTE），并于雾化吸入20μg伊洛前列素后复查RHC及TTE。结果58例患者中SLE-APAH患者40例，均为女性，年龄（33.6±7.9）岁；IPAH患者18例，女性15例，年龄（37.6±12.2）岁。两组患者RHC所测肺动脉平均压（mPAP）、肺血管阻力（PVR）、心排血量（CO）、肺毛细血管楔压（PCWP）以及TTE测量的左心室舒张末容积、左心室射血分数无显著性差异（P＞0.05）。与SLE-APAH患者相比，IPAH患者右房、右心室增大及右心室壁增厚更为显著（P＜0.05），两组超声所测右心室功能（右心室心肌工作指数，右心室面积变化率，三尖瓣瓣环收缩期位移，三尖瓣瓣环收缩期峰值运动速度）无区别（P＞0.05）。30例SLE-APAH患者及10例IPAH患者完成雾化吸入20μg伊洛前列素，两组患者在吸入伊洛前列素后mPAP及PVR均明显降低，CO、PCWP及TTE所测前述右心室功能指标无明显变化。结论 IPAH患者与SLE-APAH患者相比右心增大及右心室增厚更为明显，提示IPAH患者右心重构更为严重；吸入伊洛前列素可明显降低两组患者的mPAP及PVR，但CO及右心室功能无改善。%Objective To compare the hemodynamics, right heart function and their response to the inhaled iloprost between the patients with systematic lupus erythematosus associated pulmonary arterial hypertension (SLE-APAH) and idiopathic pulmonary arterial hypertension (IPAH). Methods A total of 58 consecutive patients with SLE-APAH or IPAH admitted into Peking Union Medical College were enrolled in the study. All patients underwent right heart catheterization (RHC), transthoracic echocardiograpy (TTE) and inhaled 20 μg iloprost. Results Among the 58 patients, 40 [all females, age(33.6±7.9)years] patients were diagnosed as SLE-APAH and 18[15 females, age (37.6±12.2)years]were diagnosed as IPAH. There were no differences between two groups in pulmonary arterial mean pressure (PAPmean), pulmonary vessel resistance (PVR), cardiac output (CO), and pulmonary capillary wedge pressure (PCWP) obtained by RHC, and end diastolic volume of left ventriculer and left ventricular ejection fraction obtained by echocardiography (P>0.05). Compared with the patients with SLE-APAH, patients with IPAH had larger right atrium and right ventricle, and thicker RV wall(P<0.05), but similar right ventricular function assessed by echocardiography. 30 patients of SLE-APAH and 10 patients of IPAH finished the inhaled iloprost. After inhaled iloprost, PAPmean and PVR of two groups were all obviously improved;but CO, PCWP and parameters of right ventricular function remained the same. Conclusions At the similar level of PAPmean and PVR, patients with IPAH had larger right heart and thicker RV wall than those with SLE-APAH, which might indicated severer right heart remodeling. PVR and PAPmean will improved after inhaled single dose of iloprost, but no improvement could be observed in right heart function.
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