Scimitar Syndrome: Experience with 6 Patients

Scimitar syndrome is a rare congenital anomaly characterized by anomalous pulmonary venous drainage to the inferior vena cava, causing a left-to-right shunt. Six patients with scimitar syndrome were diagnosed in our hospital between 2002 and 2008. There were 4 girls and 2 boys; 4 < 5 kg in weight, 2 < 8 kg in weight. Scimitar syndrome was suspected in 5 cases because of dextroversion, and diagnosed by color Doppler echocardiography in all 6 when a scimitar vein was detected entering the inferior vena cava. Computed tomography confirmed the diagnosis in all patients. Two patients had horseshoe lung, 2 had a unilobar right lung, 1 had a hypoplastic right lung, and 1 had a hypoplastic right lower lobe. Three patients had severe pulmonary arterial hypertension, 2 had moderate pulmonary arterial hypertension, and one had normal pulmonary arterial pressure. All patients had lower respiratory tract infections, volume loss of the right lung, a normal or hyperinflated left lung, dextroversion of the heart, and scimitar arteries from the descending aorta. Pneumonectomy was performed in 3 patients, lobectomy in 1, ligation of anomalous vessels in 1, and 1 died before surgery.