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.
Baskar Karthekeyan
Ranjith
R
Department of Cardiac Anesthesiology, Sri Ramachandra Medical College and Research Institute, No. 1 Ramachandra Nagar, Porur, Chennai - 116, India. ranjithb73@gmail.com
Saldanha
Richard
R
Sahadevan
M Ranjith
MR
Rao
Suresh K G
SK
Vakamudi
Mahesh
M
Rajagopal
Balakrishnan K
BK
D002675:Child, Preschool; D018618:Echocardiography, Doppler, Color; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology; D007223:Infant; D007231:Infant, Newborn; D008026:Ligation; D008168:Lung / Q000002:abnormalities / Q000000981:diagnostic imaging; D008176:Lung Volume Measurements; D008297:Male; D011013:Pneumonectomy; D011651:Pulmonary Artery / Q000002:abnormalities / Q000000981:diagnostic imaging; D011667:Pulmonary Veins / Q000002:abnormalities / Q000000981:diagnostic imaging; D012141:Respiratory Tract Infections / Q000209:etiology; D012587:Scimitar Syndrome / Q000175:diagnosis* / Q000601:surgery*; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed