CTICasesScimitar Syndrome with Coarctation of the Aorta
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Imaging findings
Chest radiographs demonstrate a small right hemithorax with ipsilateral mediastinal shift and curvilinear retrocardiac opacities. Chest computed tomography reveals the complete spectrum of Scimitar syndrome (hypogenetic lung syndrome), including a hypoplastic right lung with a single lobe, systemic arterial supply from the abdominal aorta (inferior phrenic artery), and a large anomalous pulmonary vein (the Scimitar vein) draining into the inferior vena cava. Additionally, there is severe narrowing/coarctation of the distal aortic arch (interrupted aortic arch) and extensive collateralization involving the internal mammary and epigastric arteries, as well as spontaneous intrahepatic veno-venous collaterals.
Key takeaways
Scimitar syndrome is a congenital anomaly characterized by a hypoplastic right lung and anomalous right pulmonary venous return to the systemic venous circulation (usually the IVC). It can be associated with other major cardiovascular anomalies, such as coarctation of the aorta or interrupted aortic arch. Collaterals from aortic coarctation can involve systemic arteries in the abdomen, and right-sided volume overload can lead to intrahepatic venous remodeling.
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