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Pulmonary Veno-Occlusive Disease

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Imaging findings

Small effusions, cardiomegaly, and prominent pulmonary arteries on chest radiograph. CT reveals significant pulmonary artery enlargement, right ventricular and right atrial enlargement and hypertrophy, extensive ground-glass opacities, and discrete nodules. No shunt or signs of chronic thromboembolic pulmonary hypertension. Worsening symptoms after vasodilator initiation.

Key takeaways

Pulmonary Veno-Occlusive Disease (PVOD) is a rare cause of severe pulmonary hypertension, often suspected when patients worsen on vasodilators, and can be challenging to differentiate from pulmonary capillary hemangiomatosis due to overlapping imaging findings like ground-glass opacities and nodules. Early transplant evaluation is crucial due to the aggressive nature and poor response to conventional pulmonary hypertension therapies.

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