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Partial Agenesis/Interruption of the Right Interlobar Pulmonary Artery

Imaging findings

CT showed a main pulmonary artery and upper lobe pulmonary artery, but the right interlobar pulmonary artery abruptly terminated, leading to absent perfusion in the right lower lobe. The right lung was small, with nodularity along the pleural surface, cysts in the lower lobe, and abnormal-looking lung parenchyma, suggesting developmental anomaly. Dilated intercostal arteries and transpleural collaterals were also noted.

Key takeaways

A partial interruption or atresia of a pulmonary artery, affecting only a lobe rather than the entire pulmonary artery, is a rare variant of pulmonary artery agenesis. It can lead to hypoplasia of the affected lung segment, cystic changes, and compensatory systemic arterial supply via collaterals. Fibrosing mediastinitis should be considered in the differential but is ruled out by the absence of a mass and patent pulmonary veins.

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