Fact-sheet: Pulmonary venoocclusive disease
Updated on 03/26/2020 at 2:03 PM
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Definition
Pulmonary hypertension of venous origin.
Histologically characterized by occlusion of the pulmonary venules by fibrosis, intimal thickening of the venules and small veins of the lobular septa, and arterialization of the media.
Dilation of the pulmonary capillaries and lymphatic network is also observed.
Clinical features
Exertional dyspnea.
Cyanosis, chest pain, and syncope may be found at an advanced stage.
CT
Septal thickening, ill-defined centrilobular nodules, lymphadenopathy, and pleural effusions.
Pulmonary fibrosis predominating at the bases and periphery.
Ground-glass opacities with mosaic perfusion pattern.
Differential diagnosis
Pulmonary arterial hypertension