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[纵隔纤维化相关性肺动脉高压的临床特征及CT表现]

[Clinical features and CT findings of fibrosing mediastinitis associated pulmonary hypertension].

作者信息

Li F, Wan J, Zhai Z G, Xie W M, Gao Y, Liu D T, Dou R Y, Guo W, Xiao Y, Zhu G F

机构信息

Department of Pulmonary and Critical Care Medicine, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China.

Department of Pulmonary and Critical Care Medicine, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital; Institute of Respiratory Medicine, Chinese Academy of Medical Sciences, National Clinical Research Center for Respiratory Disease, Beijing 100029, China.

出版信息

Zhonghua Jie He He Hu Xi Za Zhi. 2023 May 12;46(5):460-465. doi: 10.3760/cma.j.cn112147-20220912-00754.

DOI:10.3760/cma.j.cn112147-20220912-00754
PMID:37147807
Abstract

To investigate the clinical features and CT findings of pulmonary hypertension (PH) in patients with fibrosing mediastinitis (FM). 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 test of two independent samples, Mann-Whitney rank sum and 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. 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, 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 (<0.05). There were no differences in BNP levels and tricuspid annular plane systolic excursion between groups (>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 (<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 (<0.05), and more involvement of multiple pulmonary veins (<0.05). 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.

摘要

探讨纤维性纵隔炎(FM)患者肺动脉高压(PH)的临床特征及CT表现。回顾性研究2015年9月至2022年6月期间确诊的13例FM患者,包括经右心导管检查确诊的PH患者(FM-PH组)和无PH患者(FM组)。采用两独立样本的Mann-Whitney秩和检验分别比较两组患者的一般资料、症状、实验室检查、右心室及肺动脉测量数据和肺动脉CT表现。与7例年龄2879(60.00±17.69)岁的FM患者相比,FM-PH组的6例患者年龄6082(68.83±8.35)岁,外周水肿更多,PaO₂百分比更低,肺动脉和右心室内径更宽,右心室与左心室横径比值更高,三尖瓣反流速度更快,估计肺动脉收缩压更高(P<0.05)。两组间BNP水平和三尖瓣环平面收缩期位移无差异(P>0.05)。6例PH患者中,5例为毛细血管前性PH,1例为混合性PH。除FM-PH组患者的肺血管阻力显著高于FM组(P<0.05)外,两组间心输出量、混合静脉血氧饱和度和肺毛细血管楔压无显著差异。CT肺动脉造影(CTPA)显示肺动脉和静脉狭窄。FM-PH组患者的肺动脉和肺静脉狭窄及闭塞更严重(P<0.05),多支肺静脉受累更多(P<0.05)。FM合并PH的临床表现与肺动脉、静脉及气道的受累程度有关。建议结合临床表现、心脏超声、右心导管及CTPA等多参数对该病进行评估。

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