Evaluation of Outcomes Following Pulmonary Artery Stenting in Fibrosing Mediastinitis

Purpose: Fibrosing mediastinitis is a rare disease characterized by fibrosis of mediastinal structures with subsequent constriction of the bronchi and pulmonary vessels leading to potential respiratory compromise and death. Presently, there is no effective curative treatment with available treatments focused on reducing symptomology, including placement of pulmonary artery stents. Limited studies examine the use of stents in fibrosing mediastinitis. Given this knowledge gap, we assessed stent patency, hemodynamics, complications, and secondary outcomes of clinical improvement of pulmonary artery stenting for fibrosing mediastinitis. Materials and Methods: Nine patients with fibrosing mediastinitis and pulmonary artery stents were retrospectively identified for inclusion (six females, three males; mean age 44.17 years, range 13–68; total 13 primary stents) from 2005 to 2018. Eight patients had history of PH. All patients had dyspnea on presentation. Seven patients had ventilation/perfusion studies demonstrating impairment. Results from computed tomography and echocardiography studies were collected to assess patency and physiologic response. Results: All patients received initial angioplasty and stenting of the right pulmonary artery (10 stents). Two patients underwent additional left-sided intervention (3 stents). Stenting significantly increased lesion luminal patency (54–79%; P < 0.005) and reduced systolic pressure gradients across stenoses (mean −9.38 mmHg; P < 0.005). Primary patency at one year was 90%. Two stents received reintervention at 276 and 497 days. 89% reported improvement in dyspnea in the initial post-stenting period. There were no mortalities or major complications. Conclusion: Pulmonary artery stenting improves vascular patency and provides symptomatic relief in patients with fibrosing mediastinitis.