Idiopathic pulmonary hilar fibrosis: an unusual cause of pulmonary hypertension.
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.
Espinosa
R E
RE
Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic Rochester, Minnesota 55905.
Edwards
W D
WD
Rosenow
E C
EC
Schaff
H V
HV
D000328:Adult; D001344:Autopsy; D003937:Diagnosis, Differential; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D008297:Male; D011655:Pulmonary Embolism / Q000175:diagnosis*; D011658:Pulmonary Fibrosis / Q000175:diagnosis* / Q000473:pathology