Comparison of Clinical, Hemodynamic and Imaging Profiles Between Isolated Pulmonary Vasculitis and Chronic Thromboembolic Pulmonary Hypertension.

Isolated pulmonary vasculitis (IPV) and chronic thromboembolic pulmonary hypertension (CTEPH) are important causes of pulmonary artery stenosis-related pulmonary hypertension. Differentiating between them remains challenging. Therefore, this study aims to compare the clinical, hemodynamic and imaging profiles of patients diagnosed with IPV and CTEPH by pathological diagnosis, so as to improve the accuracy of clinical diagnosis. Patients with IPV and CTEPH confirmed by pathological diagnosis were retrospectively enrolled. The general information, clinical manifestations, laboratory indicators, imaging features, hemodynamics, and echocardiographic data before and after pulmonary endarterectomy (PEA) were collected. There were no significant differences in general information, comorbidities, and symptoms. Compared with the IPV group, the CTEPH group had worse oxygenation status, hemodynamic conditions and right ventricular function. And those in the IPV group had higher levels of ESR. The imaging manifestations showed that IPV is mainly characterized by thickening of the vessel walls, while the filling defects of the lumen account for the majority of CTEPH. Moreover, echocardiography showed improvement in right ventricular function and hemodynamics after PEA in both groups, remarkably in the CTEPH group. Comprehensive evaluation of laboratory indicators, imaging features, and hemodynamic parameters help improve the accuracy of differential diagnosis between IPV and CTEPH.