[A "late" scimitar syndrome. Diagnostic contribution of cardiac computed tomography]

A 51-year-old female hospitalized with a non-specific colitis, presented a crescent-like shadow in the right lower lung accompanied by a reduced right lung volume on a routine chest x-ray. There was no family history of congenital heart disease. The initially performed, noninvasive, contrast enhanced cardiac CT (electron-beam tomography [EBT]) proved the suspected diagnosis of a partial, anomalous pulmonary, transdiaphragmatic vein drainage (APVD) in combination with a hypoplastic right lower lobe and dextrocardia. These findings are in accordance with scimitar syndrome. Regarding to the clinical situation with symptoms like slowly progressive dyspnea on exertion and low exercise tolerance for the last 2 years and an invasively documented left-to-right shunt ratio >50% (Qp:Qs = 2.6 : 1) surgical repair was recommended. The anomalous vein was connected to the left atrium creating a "neo-septum". On a postoperative checkup after 9 months the patient is without any medication, symptoms during moderate activity are relieved, exercise tolerance was substantially better and noninvasive imaging visualized the corrected drainage of the anomalous vein to the left atrium.