Pulmonary hypertension complicating fibrosing mediastinitis

Abstract

Fibrosing mediastinitis is caused by a proliferation of fibrous tissue in the mediastinum with encasement of mediastinal viscera and compression of mediastinal bronchovascular structures. Pulmonary hypertension (PH) is a severe complication of fibrosing mediastinitis caused by extrinsic compression of the pulmonary arteries and/or veins.We have conducted a retrospective observational study reviewing clinical, functional, hemodynamic, radiological characteristics, and outcome of 27 consecutive cases of PH associated with fibrosing mediastinitis diagnosed between 2003 and 2014 at the French Referral Centre for PH.Fourteen men and 13 women with a median age of 60 years (range 18-84) had PH confirmed on right heart catheterization. The causes of fibrosing mediastinitis were sarcoidosis (n ¼ 13), tuberculosis-infection confirmed or suspected (n ¼ 9), mediastinal irradiation (n ¼ 2), and idiopathic (n ¼ 3). Sixteen patients (59%) were in NYHA functional class III and IV. Right heart catheterization confirmed moderate to severe PH with a median mean pulmonary artery pressure of 42 mm Hg (range 27-90) and a median cardiac index of 2.8 L/min/m 2 (range 1.6-4.3). Precapillary PH was found in 22 patients, postcapillary PH in 2, and combined postcapillary and precapillary PH in 3. Severe extrinsic compression of pulmonary arteries (>60% reduction in diameter) was evidenced in 2, 8, and 12 patients at the main, lobar, or segmental levels, respectively. Fourteen patients had at least one severe pulmonary venous compression with associated pleural effusion in 6 of them. PAH therapy was initiated in 7 patients and corticosteroid therapy (0.5-1 mg/kg/day) was initiated in 3 patients with sarcoidosis, with 9 other being already on low-dose corticosteroids. At 1-year follow-up, 3 patients had died and among the 21 patients evaluated, 3 deteriorated, 14 were stable, and only 4 patients with sarcoidosis improved (4 receiving corticosteroids and 1 receiving corticosteroids and PAH therapy). 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 fibrosing mediastinitis due to sarcoidosis. Although never performed for this indication, lung transplantation may be proposed in eligible patients with severe PH and fibrosing mediastinitis. (Medicine 94(44):e1800)Abbreviations: 6MWD = 6-minute walking distance, CO = cardiac output, CTEPH = chronic thromboembolic pulmonary hypertension, DLCO = diffusion capacity for carbon monoxide, ERA = endothelin receptor antagonist, FEV-1 = forced expiratory volume in 1 second, FVC = forced vital capacity, HRCT = highresolution computer tomography, mPAP = mean pulmonary arterial pressure, NYHA = New York Heart Association, PaCO 2 = partial pressure of arterial carbon dioxide, PAH = pulmonary arterial hypertension, PaO 2 = partial pressure of arterial oxygen, PAwP = pulmonary artery wedge pressure, PDE5-I = phosphodiester...

Pulmonary Artery Occlusion and Mediastinal Fibrosis in a Patient on Dopamine Agonist Treatment for Hyperprolactinemia

“…The most common pattern of mediastinal fibrosis on CT is large, localized soft tissue masses with or without calcifications, while diffuse homogeneous soft tissue processes are less common ( Sherrick et al, 1994 ; Worrell et al, 2007 ). Hence, the lack of clear radiological evidence of mediastinal masses, lymphadenopathy and/or extrinsic compression and encasement of the pulmonary arteries by neighboring tissues ( Seferian et al, 2015 ) and the nonspecific lung parenchymal affection in this patient led to the incorrect diagnosis of CTEPH. Due to the variable etiologies and clinical presentations, there is no standard therapy for mediastinal fibrosis.…”

“…Pulmonary hypertension complicating mediastinal fibrosis shares many similarities with proximal CTEPH including perfusion defects on CTPA and V/Q scintigraphy ( Seferian et al, 2015 ), both of which have a high sensitivity for the diagnosis of CTEPH ( Galie et al, 2016 ). The most common pattern of mediastinal fibrosis on CT is large, localized soft tissue masses with or without calcifications, while diffuse homogeneous soft tissue processes are less common ( Sherrick et al, 1994 ; Worrell et al, 2007 ).…”

“…Pulmonary hypertension related to mediastinal fibrosis is associated with a poor prognosis. Invasive interventions include endovascular stenting and bypassing or reconstructing an obstructed pulmonary artery ( Brown et al, 2009 ; Seferian et al, 2015 ). In the current patient case, as pharmacological treatment was unsuccessful and direct manipulation of the right pulmonary artery was associated with a high risk of artery rupture due to the extensive fibrotic changes, lung transplantation was considered the only option.…”

“…Other unusual forms of pulmonary hypertension include pulmonary hypertension related to mediastinal fibrosis ( Seferian et al, 2015 ) and the use of serotonergic drugs ( Seferian et al, 2013 ). Mediastinal fibrosis (fibrosing mediastinitis) is a rare condition characterized by proliferation of fibrous tissues in the mediastinum often associated with granulomatous diseases, such as histoplasmosis, tuberculosis and sarcoidosis and other fibro-inflammatory and autoimmune diseases ( Rossi et al, 2016 ).…”

Mimickers of chronic thromboembolic pulmonary hypertension on imaging tests: a review

“…31 In a study of 27 cases of fibrosing mediastinits with associated PH, severe extrinsic compression of the pulmonary arteries was seen in 22 patients and severe pulmonary venous compression was seen in 14 cases. 35 FM is also reportedly the most common benign cause of superior vena cava obstruction in the United States. 30 One of the ways to differentiate FM from CTEPH (besides the mixed bronchovascular involvement) is that the arteries tend to be narrowed and stenosed centrally, specifically in areas of soft tissue infiltration.…”