Abdominal aortogram shows accessory pulmonary blood flow via aorto-pulmonary collateral arteries. The pulmonary venous return (at the end of the angiogram) returns to IVC-RA junction constituting partial anomalous pulmonary venous return.
This is scimitar syndrome.
Scimitar syndrome is characterized by (i) Lung sequestration (usually, right lower lobe), (ii) Accessory blood flow to the sequestered segment from abdominal aorta and (iii) Partial anomalous pulmonary venous return - drainage to IVC).
Pulmonary hypertension may occur in newborn and infants. Exact pathogenesis of pulmonary hypertension in Scimitar syndrome is unknown. Neonatal or infantile presentation with symptoms and presence of pulmonary hypertension is a bad prognostic signs.
Recurrent pneumonia in the sequestered lung segment is an indication for removal of the sequestered lung segment.
Transcatheter intervention: Coil occlusion of acc. pulmonary blood flow helps to reduce the vascularity. When the surgeon goes to remove the sequestered lung segment, prior coil occlusion of accessory blood flow to the sequestered segment saves the surgeon having to deal with ligating the blood vessels in the abdomen.
Journal article on Scimitar syndrome - 20 year experience from Toronto.
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