Pulmonary Venous and Arterial Hypertension due to Chronic Fibrous Mediastinitis

Abstract

A patient with chronic fibrous mediastinitis presented with bronchial, superior vena caval, and pulmonary venous obstruction, pulmonary hypertension, and pulmonary fibrosis. Pulmonary venous obstruction was confirmed at surgery and explains the hemodynamic findings of elevated pulmonary artery and pulmonary wedge pressures despite low left atrial pressure. Pulmonary function studies showed marked alterations in ventilation-distribution, diffusion, work of breathing, and fixed airway resistance. It is concluded that the pulmonary arterial hypertension was due, in large part, to the pulmonary venous hypertension caused by pulmonary venous obstruction. Unsuccessful surgical intervention has been followed by 10 months of observation during which aldosterone inhibition together with use of chlorothiazide has prevented recurrence of heart failure and pleural effusion. The relationship between the physiological abnormalities and the patient's symptoms is discussed along with the possible relationship between pulmonary venous obstruction and pulmonary fibrosis.

Fibrosing Mediastinitis

“…Pulmonary function results depend on the type and extent of occlusion and provide an overall assessment of ventilatory impairment. 2 Magnetic resonance imaging of the chest and positron emission tomography (PET) in the assessment and management of FM may provide additional information about disease location and activity but needs more study.…”

“…1 The pathological findings typically include thick, "woody," hard fibrotic tissue encasing mediastinal structures, occasionally extending into the interlobular and interalveolar septa of the lung parenchyma and thickening the mediastinal pleura. 2,3 This fibrous tissue can compress and obstruct pulmonary vessels, the trachea and bronchi, and the esophagus. 2 The etiology of FM is diverse; it has been most commonly associated with fungal infections, especially with histoplasmosis in the United States.…”

“…2,3 This fibrous tissue can compress and obstruct pulmonary vessels, the trachea and bronchi, and the esophagus. 2 The etiology of FM is diverse; it has been most commonly associated with fungal infections, especially with histoplasmosis in the United States. Mycobacterial infections and non-infectious causes, such as rheumatologic or neoplastic diseases (e.g., lymphoma), have also been reported in these cases.…”

Fibrosing mediastinitis as a rare mechanism of pulmonary oedema in sarcoidosis

“…Superior vena cava obstruction is the most commonly reported and is easily diagnosed. Pulmonary artery obstruction has also been reported [3][4][5][6]. In sarcoidosis, two cases were reported by HAMILTON-CRAIG et al [7] who were successfully treated by stent placement in the pulmonary arteries.…”

“…Regarding sarcoidosis, DEVARAJ et al [2] reported two cases in a series of 12 patients, with CT images and histology of fibrosing mediastinitis (16.6% of patients). Vascular obstruction due to fibrosing mediastinitis is frequent [3][4][5][6][7][8][9][10][11][12][13], particularly during histoplasmosis. Superior vena cava obstruction is the most commonly reported and is easily diagnosed.…”