Pulmonary Venoocclusive Disease

Pulmonary Venoocclusive Disease

Jud W. Gurney, MD, FACR

Axial CECT shows smooth interlobular septal thickening image and centrilobular nodules image in a patient with pulmonary venoocclusive disease.

Axial HRCT shows centrilobular ground-glass nodules image in a patient with pulmonary artery hypertension image from pulmonary venoocclusive disease.


Abbreviations and Synonyms

  • Pulmonary venoocclusive disease (PVOD), pulmonary vasoocclusive disease

  • Isolated pulmonary venous sclerosis, obstructive disease of pulmonary veins, venous form of primary pulmonary hypertension, pulmonary artery hypertension (PAH)


  • Rare cause of pulmonary hypertension due primarily to occlusion of post-capillary venous radicles


General Features

  • Best diagnostic clue: Pulmonary arterial hypertension + smooth interlobular septal lines

  • Patient position/location: Centrilobular ground-glass opacities and septal thickening have random distribution within lung

CT Findings

  • Lung

    • Ground-glass opacities common (100%) but nonspecific

      • Centrilobular (65%), most specific pattern

      • Geographic (50%)

      • Diffuse

      • Mosaic

      • Perihilar

      • Patchy

    • Thickened interlobular septa (80%), smooth

      • Range from few to numerous

    • Peribronchovascular bundle thickening

    • Distribution of septal thickening and ground-glass opacities: Random

  • Lymph nodes

    • Mild lymphadenopathy, mean 15 ± 5 mm (50%)

  • Pulmonary vasculature

    • Enlarged main and central pulmonary arteries

    • Pulmonary vein caliber normal

  • Cardiac

    • Dilated right atrium and right ventricle

    • Thickened right ventricular myocardium

    • Left ventricle and atrium have normal dimensions

  • Pleura-pericardium

    • Pleural effusions (20-40%) may be bilateral, usually small

    • Pericardial effusion (10%), small

  • Accuracy

    • Presence of 2 or more findings (lymphadenopathy, septal thickening, centrilobular ground-glass opacities)

      • Sensitivity 75% and specificity of 85% for PVOD

      • Presence of only 1 finding cannot rule out PVOD

  • Life-threatening pulmonary edema

    • May follow vasodilator therapy for primary pulmonary hypertension (PPH)

    • Vasodilators include continuous intravenous infusion prostacyclin (epoprostenol) and calcium channel blockers

    • Patients with centrilobular ground-glass opacities and septal lines at highest risk

Sep 20, 2016 | Posted by in RESPIRATORY IMAGING | Comments Off on Pulmonary Venoocclusive Disease

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