Congenital pulmonary vein stenosis: a radiographic study

Seven patients with congenital pulmonary venous stenosis were retrospectively evaluated. Chest radiographs and pulmonary angiograms were interpreted without knowledge of anatomic findings, and results were correlated with autopsy data. Bilateral foci of stenosis of varying severity were found in all patients but occurred with greater frequency and severity in the left-sided pulmonary veins. Chest radiographic findings revealed asymmetric pulmonary vascularity in all patients. Shunt vascularity was seen in three right upper zones and three right lower zones but was not observed in any of the left lung zones. In seven patients angiograms revealed asymmetry in the size and number of pulmonary arterial branches. Arterial vascularity was increased in two right upper zones and four right lower zones but was not increased in any of the left lung zones. Pulmonary veins from 22 zones were visualized. Pulmonary venous opacification was delayed, asymmetric, and asynchronous in the majority. Venoatrial stenosis was seen in veins from 18 zones. Congenital pulmonary vein stenosis can be suspected based on chest radiographic findings of asymmetry of pulmonary vascularity between both lungs as well as regional discrepancies in the vascularity within each lung. Pulmonary angiography can support the diagnosis.