Discussion Author(s): Primary author: Jason Messinger
Contributing author: Eric Jones
Scimitar syndrome, also known as pulmonary venolobar syndrome, is a rare congenital cardiovascular defect that presents with pulmonary venous drainage into the inferior vena cava and is often associated with right hypoplastic lung and right hypolastic pulmonary artery. (Argueto-Morales et al., Frydrychoqicz et al., & Bell et al.).
Depending on the severity of the syndrome, patients may present with other congenital defects. The most severe form of the syndrome is that found in neonates where mortality is high (Argueta-Morales et al.). The incidence of Scimitar syndrome is 1-3 in 100,000 live births according to most recent statistics (Bell et al.).
The extreme rarity of the infantile scimitar syndrome has limited the ability for investigators to determine the best course of treatment. The most common course of treatment for infants is to stabilize stress on the heart until the infant has grown to an acceptable size. Surgery is the ultimate treatment to correct the scimitar vein blood flow.
However, reports of success are not abundant with mortality post-op being 45% (Argueta-Morales et al.).
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