Troublesome triad: fibrosing mediastinitis, severe pulmonary hypertension, and pulmonary vein stenosis

Gregory W Wigger, Jean Elwing
2017 Vascular Diseases and Therapeutics  
Fibrosing mediastinitis is a disorder of invasive and proliferative fibrous tissue growth in the mediastinum. Its occurrence is rare and cause unknown, though it has been associated with several infections. The fibrosis of mediastinal structures, particularly vasculature, results in common symptoms of cough, dyspnea and hemoptysis due to pulmonary hypertension, pulmonary edema, and parenchyma fibrosis. Progression of disease carries a high mortality rate and causes of death are typically due to
more » ... cor pulmonale,pulmonary infections and respiratory failure. Several medical treatments and surgical procedures have been unsuccessful; however the use of endovascular stenting is showing promising results. Early recognition and diagnosis are essential to provide patients with the best chance of survival and management options.We review the case of a 43 year-old female diagnosed with fibrosing mediastinitis and sequelae of pulmonary vein stenosis and pulmonary hypertension. Unfortunately, she was a poor candidate for pulmonic vein stenting due to her unstable condition. Without treatment of the stenosis, her clinical status and pulmonary hypertension worsened. Pulmonary vasodilators resulted in pulmonary edema and diuresis resulted in hypotension. Ultimately her condition was untreatable. This case illustrates the devastating effects of advanced fibrosing mediastinitis.
doi:10.15761/vdt.1000126 fatcat:frmx4qygdbbylifrn5o75fpt5y