| Abstract: | We present 10 symptomatic patients with scimitar syndrome. Their age was below 1 year and four were newborn babies. In 5 patients there was secundum atrial septal defect and in one there was a patent ductus arteriosus. Sixty per cent of the patients had pulmonary sequestration. The right lung was moderately or severely hypoplasic in 8 cases and seven had frequent respiratory infections. The anomalous venous drainage produced a left-to right shunt at atrium level which in our series had a value of 2.87 +/- 0.5. Five patients had pulmonary hypertension either moderate or severe. The severity of this disease in early infancy is related to the pulmonary hypertension (40%), requiring a prompt surgical correction. Eight patients were operated upon, the drainage being connected to the left atrium. The six surviving patients are asymptomatic at 12 years of follow-up. Our series is the biggest published in our country and the second in the world literature in infancy. |
