CTICasesCongenital Unilateral Pulmonary Vein Atresia
[...]. Congenital Unilateral Pulmonary Vein Atresia. CTICases. Published January 13, 2017. https://str-case-explorer.pages.dev/c/m3jwj
Imaging findings
Chest radiograph shows a hypoplastic right lung, ipsilateral mediastinal shift, an elevated right hemidiaphragm, and prominent septal lines. Chest CT confirms the complete absence of right-sided pulmonary veins, a severely hypoplastic right pulmonary artery, retrograde flow, and extensive systemic-to-pulmonary collateralization via hypertrophied bronchial arteries.
Key takeaways
Congenital unilateral pulmonary vein atresia is a rare developmental anomaly characterized by the complete absence of pulmonary veins on one side. The affected lung is hypoplastic, and drainage occurs via retrograde flow into systemic veins or pleural and esophageal collaterals, which can lead to symptoms such as hemoptysis and pulmonary hypertension in the contralateral lung due to hyperperfusion.
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