Asymptomatic atresia of the anomalous pulmonary vein in a patient with scimitar syndrome presenting in childhood.
An asymptomatic 6-year-old boy with a history of right lung hypoplasia was referred for cardiology evaluation. Echocardiography demonstrated right pulmonary artery hypoplasia with flow reversal in that vessel. The right pulmonary veins were not visualised in the echocardiogram. Cardiac catheterisation confirmed the diagnosis of scimitar syndrome with a characteristic large vertical vein; however, the right pulmonary veins were found to be atretic with no connection to the heart with decompression through the azygos vein. In all, four systemic to pulmonary arterial collaterals were identified, supplying the right lung, which were occluded using embolization coils. This case demonstrates the potential for progressive stenosis and atresia of the so-called "scimitar vein" without previous surgical instrumentation, and that this can occur without haemodynamic embarrassment or development of pulmonary vascular disease.
["O'Byrne|Michael L|ML|", 'Cross|Russell R|RR|', 'Martin|Gerard R|GR|']
D000015:Abnormalities, Multiple D006328:Cardiac Catheterization D006348:Cardiac Surgical Procedures D002648:Child D000072226:Computed Tomography Angiography D006801:Humans D008297:Male D011651:Pulmonary Artery D011667:Pulmonary Veins D012587:Scimitar Syndrome
CHD CT transcatheter intervention