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Persistent Left SVC with Unroofed Coronary Sinus

Imaging findings

Gated cardiac CT shows a persistent left superior vena cava (SVC) draining directly into the left atrium. The coronary sinus is unroofed (absent wall), communicating directly with the left atrium. There is a small innominate vein and aortic coarctation.

Key takeaways

A persistent left SVC associated with an unroofed coronary sinus (Raghib syndrome) is a congenital anomaly that creates a left-to-right shunt. It often co-exists with other congenital cardiovascular anomalies such as coarctation of the aorta.

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