摘要目的:研究纤维素性纵隔炎（FM）合并肺动脉高压（PH）的临床及影像学特征。方法:回顾性收集2015年9月至2022年6月确诊为FM的13例患者，其中男4例，女9例，年龄28~82（64.08±14.37）岁，全部行右心导管检查，确诊合并PH的患者（FM-PH组）与不合并PH的患者（FM组）分别采用独立样本 t检验、Mann-Whitney U秩和检验及Fisher精确检验比较两组的一般资料、临床症状、实验室检查结果、心功能指标及CT肺动脉造影（CTPA）表现。 结果:FM-PH组与FM组相比，FM-PH组患者多存在外周水肿、PaO 2下降，肺动脉内径增宽，右心室内径、右心室/左心室横径增大，三尖瓣反流速率及估测肺动脉收缩压升高，差异均有统计学意义（均 P<0.05）；BNP与三尖瓣环收缩期位移差异无统计学意义（ P>0.05）。6例FM-PH患者中，5例为毛细血管前PH，1例为混合性PH，除FM-PH组患者肺血管阻力明显高于FM组（ P<0.05）外，心排血量、混合静脉血氧饱和度及肺毛细血管楔压在两组间差异均无统计学意义。患者CT肺血管造影的表现为肺动、静脉狭窄，FM-PH组更多表现为肺动脉及肺静脉重度狭窄（ P<0.05）以及肺静脉多支受累（ P<0.05）。 结论:FM合并PH的临床表现与肺动脉、肺静脉、气道的受累程度不同相关，需结合临床表现、心脏超声、右心导管与影像学等多参数进行疑诊与评估。

abstractsObjective:To investigate the clinical features and CT findings of pulmonary hypertension (PH) in patients with fibrosing mediastinitis (FM).Methods:Thirteen patients with FM diagnosed between September 2015 and June 2022 were studied retrospectively, including patients with PH (FM-PH group) and patients without PH (FM group) confirmed on right heart catheterization. The t test of two independent samples, Mann‐Whitney U rank sum and Fisher′s test were used to compare the general information, symptoms, laboratory examination, right ventricular and pulmonary artery measurement data and pulmonary artery CT findings between the two groups, respectively. Results:Compared with the 7 FM patients aged 28-79 (60.00±17.69) years, the 6 patients in the FM-PH group, aged from 60 to 82 (68.83±8.35) years, had more peripheral edema, lower percentage of PaO 2, wider inner diameters of pulmonary artery and right ventricle, a higher ratio of right ventricle and left ventricular transverse diameter, faster tricuspid regurgitation velocity and higher estimated systolic pulmonary artery pressure ( P<0.05). There were no differences in BNP levels and tricuspid annular plane systolic excursion between groups ( P>0.05). Of the 6 patients with PH, 5 had precapillary PH and 1 had mixed PH. Except that the pulmonary vascular resistance in patients of the FM-PH group was significantly higher than that in the FM group ( P<0.05), there were no significant differences in cardiac output, mixed venous oxygen saturation and pulmonary capillary wedge pressure between the two groups. CT pulmonary angiography (CTPA) showed pulmonary artery and vein stenosis. Patients in the FM-PH group had more severe stenosis and occlusion of pulmonary artery and pulmonary vein ( P<0.05), and more involvement of multiple pulmonary veins ( P<0.05). Conclusions:The clinical manifestation of FM complicated with PH is related to the degree of involvement of pulmonary artery, vein and airway. It is recommended that the disease be evaluated in combination with multiple parameters such as clinical manifestations, cardiac ultrasound, right cardiac catheter and CTPA.