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Systemic Arterial Supply to the Left Lower Lobe with Atrial Septal Defect

Imaging findings

A chest radiograph raised concern for pulmonary arterial dilation, and subsequent CT showed an anomalous systemic artery arising from the descending aorta supplying an area of the left lower lobe, with an increased number of small vessels in that segment but a normal complement of airways (excluding sequestration) and no arteriovenous shunt or anomalous venous drainage (excluding an arteriovenous malformation); the pulmonary veins drained normally to the left atrium. A separately noted large secundum atrial septal defect with right ventricular enlargement and shunt vascularity was also identified.

Key takeaways

Systemic arterial supply to otherwise normal lung parenchyma with preserved airway connections and normal venous drainage is a distinct congenital entity from bronchopulmonary sequestration (which requires airway isolation) and from an arteriovenous malformation (which requires an abnormal arteriovenous shunt). This case featured two separate congenital anomalies occurring together: anomalous systemic arterial supply to the lung and a secundum atrial septal defect.

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