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.


TERMINOLOGY


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)


Definitions



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


IMAGING FINDINGS


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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