Use of 64 slice CT in scimitar syndrome

Scimitar Syndrome is a relatively rare variety of partial anomalous pulmonary venous connection in which the right pulmonary veins form an anomalous confluence which drains into the infra-diaphragmatic inferior vena cava. The X-ray chest in these patients shows the typical Scimitar Sign.

We are presenting a patient who was diagnosed to have Scimitar Syndrome on the basis of X-ray chest and echocardiography. Confirmation of diagnosis and precise anatomical characterization was achieved by 64 slice CT angiocardiography. The patient underwent successful surgical correction. Adequacy of procedure was demonstrated by the same procedure.

scimitar syndrome is a type of partial anomalous venous connection. It consists of sinus venosus type of atrial septal defect, anomalous confluence of right upper and lower pulmonary veins draining into the infra-diaphragmatic inferior vena cava and right lung lower lobe hypoplasia. The X-ray chest shows the characteristic Scimitar Sign. Precise anatomical characterization in required for operative correction and cannot usually be achieved by echocardiography and requires invasive angiocardiography, multi-detector CT angiocardiography or cardiac MRI. We used CT angiocardiography for diagnosis and post-operative confirmation of adequacy of correction.