ABSTRACT
Introduction Individual pulmonary vein stenosis, hypoplasia, or atresia is a rare congenital cardiac lesion, which may cause pulmonary hypertension. Both stenosis and atresia can occur in the same patient, and one or more veins can be involved. The obstruction may be localized to the veno-atrial junction, or may extend into the lung parenchyma for some distance. It may occur in isolation or in combination with other cardiac lesions (e.g., scimitar syndrome) (Figure 50.1) or due to compression from the left atrium and descending aorta, or from an extracardiac Fontan conduit (right-sided venous compression).1,2 It may evolve with time and affect additional pulmonary veins.
