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Duplicated Superior Vena Cava with Anomalous Pulmonary Venous Drainage Causing Paradoxical Embolism

Imaging findings

A patient presenting with neurologic symptoms had a bubble study that was normal with left upper extremity injection but produced a transient ischemic attack when injected from the right upper extremity. CT and cardiac MRA showed a duplicated superior vena cava: the left SVC drained conventionally via a large intercostal vein into the great cardiac vein, coronary sinus, and right atrium, but the bridging innominate vein was absent, and the right SVC received an anomalous superior pulmonary vein plus an additional anterior venous branch that drained directly into the left atrium rather than the right atrium, without an associated sinus venosus atrial septal defect.

Key takeaways

Anomalous pulmonary venous drainage into a right superior vena cava that in turn connects to the left atrium, rather than the right atrium, creates a right-to-left shunt that can cause paradoxical embolism and neurologic symptoms, and can occur even without the sinus venosus atrial septal defect with which this anomaly is classically associated. A bubble study that is positive only with right-arm injection, in the setting of a duplicated SVC, should prompt a careful search for this anomalous venoatrial connection.

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