SCIMITAR SYNDROME
Findings:
Right pulmonary veins are not seen arising from left atrium and appears to arise from the intrahepatic IVC crossing the diaphragm.
Suprahepatic IVC is grossly dilated.
Right lung appears hypoplastic with shift of mediastinum toward right.
Above features suggest possibility of scimitar syndrome.
Discussion:
Scimitar syndrome is a rare congenital heart defect, variant of a partial anomalous pulmonary venous return that results in a left-to-right shunt.
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.
There is an estimated incidence of 1 to 3 per 100000 live births.
