Sreeram N,Pretel E, Pillekamp F, Bennink G. Scimitar syndrome in infancy. Images Paediatr Cardiol 2008;37


(808Kb)	Sreeram N, Pretel E, Pillekamp F, Bennink G. Scimitar syndrome in infancy. Images Paediatr Cardiol 2008;37:1-4
	University Hospital of Cologne, Germany.

MeSH

Heart Catheterization	Scimitar Syndrome	Heart defects, congenital
Infant

Scimitar syndrome, if presenting in infancy, is associated with signs of heart failure and pulmonary hypertension.¹ The typical pathological features are sequestration of a segment of the lung, usually the right lower lobe, with arterial supply arising from the abdominal aorta, and partial anomalous pulmonary venous connection, with the sequestered segment draining to the inferior caval vein. Additional features include an atrial septal defect and hypoplasia of the right pulmonary artery and lung. Standard therapy consists of occlusion of the anomalous arterial supply to abolish the left to right shunt, and surgical repair, if required, of associated cardiac defects.²

We present the typical angiographic features of an infant with Scimitar syndrome. In addition to occlusion of the aortopulmonary collateral vessel, the infant underwent successful surgical closure of an atrial septal defect with rerouting of the anomalous pulmonary venous connection.

Fig. 1: Abdominal aortogram demonstrating anomalous arterial supply to the right lower lobe of the lung. fig01

Fig. 2: Follow through demonstrating the extent of the sequestered segment. fig02

Fig. 3: Aortogram following single coil occlusion of the aortopulmonary collateral vessel. fig03

Fig. 4: Pulmonary artery angiogram demonstrating hypoplasia of the right pulmonary artery. fig04

Fig. 5: Follow-through showing a small right lung supplied by the native pulmonary artery. fig05

Fig. 6: Venous phase showing venous drainage to the inferior vena cava. fig06

References

Gao YA, Burrows PE, Benson LN, Rabinovitch M, Freedom RM. Scimitar syndrome in infancy. J Am Coll Cardiol 1993;22:873-82.
Najm HK, Williams WG, Coles JG, Rebeyka IM, Freedom RM. Scimitar syndrome: twenty years‘ experience and results of repair. J Thorac Cardiovasc Surg 1996;112:1161-8.

Contact information

Contact information N. Sreeram, Department of Paediatric Cardiology, University Hospital of Cologne, Kerpenerstrasse 62, 50937 Cologne, Germany. Phone: +49 221 478 32517 Fax: +49 221 478 32515 N.Sreeram@uni-koeln.de