CTICasesPulmonary Veno-Occlusive Disease
Imaging findings
CT chest in a patient with limited scleroderma shows mild septal thickening, pleural effusion, a dilated main pulmonary artery, and a normal-sized left atrium, with severe pulmonary hypertension confirmed on right heart catheterization in the setting of a normal pulmonary capillary wedge pressure.
Key takeaways
Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension characterized by progressive fibrous occlusion of the pulmonary venules. It is strongly associated with systemic sclerosis (scleroderma) and has an extremely poor prognosis. It must be suspected when signs of post-capillary pulmonary hypertension (like septal lines and effusions) are present with a normal wedge pressure.
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