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Unilateral Pulmonary Artery Agenesis

Imaging findings

Chest CT shows complete absence of the right pulmonary artery (unilateral right pulmonary artery agenesis/atresia) with hypoplastic right pulmonary veins and a small, hypolucent right lung. Hypertrophied systemic collateral vessels from the subclavian and axillary arteries supply the right lung. VQ scan shows 100% of perfusion going to the left lung.

Key takeaways

Unilateral pulmonary artery agenesis is a rare congenital vascular anomaly. It can present with hemoptysis due to extensive systemic arterial collateralization from the chest wall and mediastinum, and the affected lung is typically small and hyperlucent.

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