Severe Asymmetric Perfusion Deficit in Cystic Fibrosis
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Imaging findings
Chest CT demonstrates severe asymmetric lung destruction, with a completely collapsed and bronchiectatic left upper lobe and diffuse gas trapping, hyperlucency, and marked attenuation of pulmonary vessels in the remaining left lung, contrasting with a hyperperfused right lung displaying significantly larger pulmonary arteries.
Key takeaways
Severe chronic regional airway obstruction and alveolar hypoxia in cystic fibrosis can induce profound local hypoxic pulmonary vasoconstriction and vascular remodeling. This physiologic response can divert nearly all cardiac output to the contralateral, less diseased lung, resulting in a dramatic asymmetric perfusion deficit on nuclear medicine scans that can mimic acute pulmonary embolism but is purely a reflex of the underlying parenchymal destruction.
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