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Tetralogy of Fallot with Left Lung Hypoplasia

Imaging findings

CT chest in a patient with repaired Tetralogy of Fallot shows splaying and narrowing of the trachea (tracheomalacia). The left pulmonary artery is hypoplastic, with a patent stent in place, and the left lung is significantly smaller than the right (lung hypoplasia).

Key takeaways

Tetralogy of Fallot can be associated with branch pulmonary artery stenosis. Severe, congenital branch stenosis leads to hypoperfusion of the ipsilateral lung during development, resulting in congenital lung hypoplasia. Repetitive surgical interventions and mediastinal vessels can also lead to acquired tracheomalacia.

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