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Absent pulmonary valve syndrome (APVS) is a rare cardiac malformation with massive pulmonary insufficiency that presents with short-term and long-term respiratory problems secondary to severe bronchial compression from enlarged central and hilar pulmonary arteries. Association with chromosome 22q11 deletions and DiGeorge syndrome is common. This historical review illustrates the airway disease with emphasis on assessment of the bronchial compression in patients with persistent respiratory difficulties after valvular repair. Cases that had MRI for cardiac assessment are used to illustrate the pattern of airway disease. Keywords Absent pulmonary valve syndrome * Airway abnormalities * MRI