CTICasesRepaired Tetralogy of Fallot with Left Isomerism, Azygos Continuation of IVC, and Aortic Stenosis
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Imaging findings
Chest radiograph shows sternotomy and a large azygos vein. CT reveals a history of repaired Tetralogy of Fallot with residual overriding tissue at the aortic root/LVOT causing functional outlet obstruction. There is a large left atrium, dilated azygos vein, and an aortic aneurysm. The azygos vein continues the IVC, and there are multiple heterogeneously enhancing masses in the left upper abdomen consistent with polysplenia. Lung windows show bilateral morphologic left lungs, indicating left isomerism.
Key takeaways
This case demonstrates a complex congenital heart disease with associated visceral abnormalities. The residual hypertrophied septum at the aortic root/LVOT post-Tetralogy of Fallot repair can lead to significant aortic stenosis and functional outlet obstruction. Left isomerism (polysplenia syndrome) is characterized by specific organ malpositions, including azygos continuation of the IVC and bilateral left lungs, commonly associated with congenital heart disease.
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