Scimitar syndrome: five cases examined with two-dimensional and Doppler echocardiography

Five patients with scimitar syndrome (three boys, two girls) with a mean age of 3.4 years (range 6 months to 11 years) were studied with two-dimensional and Doppler echocardiography. Four-chamber apical and parasternal short- and long-axis views were obtained, in addition to subcostal views. The anomalous drainage was seen entering the inferior vena cava below the diaphragm in three patients, and in two cases the inferior vena cava was considered normal (both patients had supradiaphragmatic drainage). The anomalous flow velocity pattern was monophasic without reverse flow at atrial contraction. The peak velocity ranged from 0.6 to 1.0 m/s (mean 0.8 m/s). In one case an ostium secundum atrial septal defect was detected. It is concluded that two-dimensional and Doppler echocardiography permit demonstration of the anomalous drainage, analyze the anomalous flow velocity pattern, and detect other associated cardiac abnormalities.