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Fibrosing Mediastinitis from Histoplasmosis with Cor Pulmonale

Imaging findings

CT in a 35-year-old demonstrates dense soft tissue encasing the right hilum and mediastinum with associated calcifications, consistent with fibrosing mediastinitis from prior histoplasmosis. The fibrous tissue narrows the right pulmonary artery and its branches, occludes the right pulmonary veins and superior vein, and narrows the left pulmonary artery and airway. The left atrium is markedly small from pulmonary venous obstruction. Mosaic attenuation is extensive on the left with asymmetric distribution. The right heart is dilated and hypertrophied from severe pulmonary hypertension with a bowing interventricular septum. Splenic punctate calcifications support prior histoplasma exposure.

Key takeaways

Fibrosing mediastinitis from histoplasmosis can produce progressive occlusion of pulmonary vasculature (arteries and veins) and airways, leading to severe secondary pulmonary hypertension and cor pulmonale. The order of vessel involvement is typically pulmonary veins first, then arteries, then airways. Once all central vessels are occluded there is no surgical or interventional option, and the disease is uniformly fatal. Calcifications in the mediastinal fibrous tissue are a helpful clue to histoplasmosis as the underlying cause. Patients from endemic regions of the Ohio and Mississippi River Valleys are at greatest risk.

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