Proximal Interruption of the Right Pulmonary Artery
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Imaging findings
A V/Q scan demonstrates homogeneous ventilation but severely reduced perfusion to the right lung. CT shows a normal left pulmonary artery (PA) but an absence of the right PA, with large collateral bronchial arteries present. The right lung is hypoplastic, and its vessels appear irregular and extend to the periphery, consistent with transpleural collaterals.
Key takeaways
This is a classic presentation of proximal interruption of a pulmonary artery, typically occurring contralateral to the aortic arch (e.g., right PA interruption with a left aortic arch). Collateral bronchial arteries supply the affected lung, and transpleural collaterals can explain minimal radiotracer uptake. While often contralateral, the association with the aortic arch is not invariable.
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Related Congenital cases
- Double Aortic Arch (Asymptomatic)
- Left Upper Lobe Anomalous Pulmonary Venous Stricture (Incidental)
- Anomalous Left Coronary Artery from Pulmonary Artery (ALCAPA) with Proximal Interruption of the Right Pulmonary Artery
- Hypertrophic Cardiomyopathy (HCM)
- Unilateral Acute Respiratory Distress Syndrome (ARDS) due to Left Pulmonary Artery Agenesis
- Aberrant Right Subclavian Artery