CTICasesUnilateral Pulmonary Vein Atresia
Imaging findings
Chest radiograph reveals a severely hypoplastic right lung with ipsilateral mediastinal shift and coarse reticular opacities at the base. Contrast-enhanced CT demonstrates a small but patent right pulmonary artery with unexpectedly bright contrast enhancement compared to the main pulmonary trunk, as well as multiple prominent systemic collaterals and extensive septal lines. Crucially, there is a complete absence of the right pulmonary veins entering the left atrium.
Key takeaways
Unilateral pulmonary vein atresia is a rare congenital vascular anomaly characterized by the absence of pulmonary veins draining a single lung. The affected lung is hypoplastic, and the absence of normal venous drainage leads to severe pulmonary venous congestion, resulting in marked interstitial thickening, septal lines, and systemic arterial collateralization. The bright contrast enhancement seen in the hypoplastic pulmonary artery on CT is due to retrograde flow of systemic collateral blood into the pulmonary arterial bed, a feature that helps differentiate this condition from proximal interruption of the pulmonary artery.
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