CTICasesPulmonary Veno-Occlusive Disease / Pulmonary Capillary Hemangiomatosis
Imaging findings
A woman around 40 referred for pulmonary hypertension has high right heart pressures with enlarged pulmonary arteries but no compelling evidence of chronic thromboembolic disease. There are extensive peripheral reticulations predominantly in the lower lobes, thickened interlobular septa in the upper lobes, some faint tree-in-bud-like and centrilobular ground glass, and a small amount of stable mediastinal and hilar lymph node tissue. The pulmonary veins are normal and a prior left heart catheterization was normal. Possible small subpleural nodules raise the question of transpleural systemic collaterals.
Key takeaways
Pulmonary hypertension with peripheral reticulation, thickened septa, and centrilobular ground glass but normal pulmonary veins and a normal left heart catheterization suggests pulmonary veno-occlusive disease or pulmonary capillary hemangiomatosis. In severe pulmonary hypertension with sufficient obstructive small-vessel disease, systemic collateral vessels can traverse the pleural surface to anastomose with pulmonary artery segments, a subtle clue seen as small subpleural nodular or tubular structures.
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