Idiopathic pulmonary hilar fibrosis: an unusual cause of pulmonary hypertension.
case_report · Level V
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Abstract
A 37-year-old man with progressive exertional dyspnea had pulmonary hypertension associated with pulmonary arterial and venous obstruction. An autopsy revealed that the cause of death was idiopathic pulmonary hilar fibrosis, a variant of mediastinal fibrosis. Pulmonary hilar fibrosis can mimic thromboembolic pulmonary hypertension, pulmonary veno-occlusive disease, and pulmonary venous hypertension.
Medical subject headings
- Hypertension, Pulmonary
- Pulmonary Embolism
- Pulmonary Fibrosis