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Unilateral Pulmonary Venous Obstruction with Uncorrected VSD

Imaging findings

Non-contrast CT demonstrates asymmetric lung findings: the left lung shows signs of severe pulmonary arterial hypertension with massively enlarged pulmonary arteries, while the right lung demonstrates diffuse septal thickening, bronchial cuffing, and a small right pulmonary artery. Contrast-enhanced CT reveals a large, uncorrected ventricular septal defect (VSD) and complete absence of pulmonary venous drainage from the right lung into the left atrium.

Key takeaways

Unilateral pulmonary venous obstruction or congenital absence of pulmonary veins is a rare condition that leads to localized pulmonary venous congestion, lymphatic dilatation, and interstitial edema on the affected side. This creates a striking asymmetry on chest imaging, mimicking unilateral interstitial lung disease, while the contralateral side may show signs of severe pulmonary hypertension, especially in the setting of a co-existing uncorrected left-to-right shunt.

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