Pulmonary vascular stenosis scoring in fibrosing mediastinitis

Abstract Aims This study aimed to develop a scoring system for evaluating the degree of pulmonary vascular stenosis in fibrosing mediastinitis (FM). Methods and Results A retrospective single-center study was conducted on 56 patients with FM in China between April 2014 and August 2021. The involvement of pulmonary vessels in patients with FM was assessed using dual phase-computed tomography pulmonary angiography, and we found that 85.7% of the patients had both pulmonary artery (PA) and vein (PV) involvement. PA involvement was mainly located proximal to both the upper PA and bilateral basal trunk levels in the lower lungs. The involvement of the superior PV was more common than that of the inferior PV, and the right inferior PV was the least involved. Most of these lesions exhibited moderate or severe stenosis. Additionally, a scoring system for the degree of pulmonary vascular stenosis was developed. Correlation analysis revealed a negative correlation between the final pulmonary vascular score and pulmonary arterial pressure, pulmonary vascular resistance, and maximum tricuspid regurgitation velocity. The calculated score of 17.1 was the best cut-off value for the diagnosis of mild and severe pulmonary hypertension. Conclusion We successfully developed a scoring system for pulmonary vascular stenosis that can be used to evaluate the severity of pulmonary vessel involvement and pulmonary hypertension. This scoring system may be relevant in the future development of target-based strategies for percutaneous interventions.