Scimitar Syndrome

Excerpt

Scimitar syndrome; also known as congenital venolobar syndrome, Halasz syndrome, mirror-image lung syndrome, hypogenetic lung syndrome, and vena cava bronchovascular syndrome, is a rare congenital heart defect. It is a variant of a partial anomalous pulmonary venous return that results in a left-to-right shunt, with a characteristic anatomic feature that resembles a backsword or a saber with a curved blade known as the Middle Eastern or Turkish sword, "scimitar."

The distinct characteristics that constitute scimitar syndrome are:

1) Partial or entire anomalous curved venous drainage of the right lung to the inferior vena cava.

2) Association with variable right lung and pulmonary artery hypoplasia.

3) Dextraposition of the heart.

4) An anomalous systemic blood supply to the ipsilateral lung.

Other associated findings include atrial septal defects and aortopulmonary collaterals. Scimitar syndrome was first described by George Cooper in 1836 while conducting an autopsy of a 10-month-old infant. The first imaging diagnosis was made with cardiac catheterization and described by Dotter et al. in 1949. First surgical intervention performed in 1950 by Drake and Lynch, which involved resection the right lower lung. First corrective surgery in 1956 by Kirklin et al. Schramel et al. with recognition two variations of scimitar veins; a simple classic vein that goes from the middle portion of the right lung running to the cardiophrenic angle and a second type in which a double-arched vein in the lower and upper lung zones drain into the left atrium and inferior vena cava.