Abstract
Fibrosing mediastinitis (FM) is caused by proliferation of fibrous tissue in the mediastinum with encasement of mediastinal viscera and compression of mediastinal structures. Pulmonary hypertension (PH) is caused by extrinsic compression of the pulmonary arteries and/or veins.
We reviewed clinical, functional, hemodynamic, radiological characteristics and outcome of 27 consecutive cases of PH associated with FM diagnosed at the French Referral Centre for PH (2003-2014).
Right heart catheterization (RHC) confirmed PH in 14 men and 13 women. The causes of FM were sarcoidosis (n=13), tuberculosis-infection (n=9), mediastinal irradiation (n=2) and idiopathic (n=3). Sixteen patients were in NYHA functional class III and IV. RHC found moderate PH: median mean pulmonary artery pressure 42mmHg (27-90) and median cardiac index 2.8L/min/m2 (1.6-4.3). Severe extrinsic compression of pulmonary arteries was found in 2, 8 and 12 patients at the main, lobar or segmental level, respectively. 14 patients had at least one severe pulmonary venous compression. PAH therapy was initiated in 7 patients and corticosteroid therapy (0.5-1mg/kg/day) was initiated in 3 patients with sarcoidosis, 9 other being already on low dose corticosteroids. At 1 year follow-up, 3 were dead and among the 21 evaluated, 3 deteriorated, 14 were stable and 4 patients with sarcoidosis improved. Survival was 88%, 73% and 56% at 1, 3 and 5 years, respectively.
We found no clear clinical improvement with the use of specific PAH therapy. Corticosteroid therapy may be associated with clinical improvement, in some patients with FM due to sarcoidosis. Although never performed for this indication, lung transplantation may be proposed in eligible patients with severe PH and FM.
- Copyright ©the authors 2016
