CTICasesLeft Superior Vena Cava Draining to Left Atrium with Unroofed Coronary Sinus (Raghib Syndrome variant)
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Imaging findings
The patient has median sternotomy wires from a previous secundum atrial septal defect (ASD) repair. Imaging reveals bilateral superior vena cavae connected by a rudimentary bridging innominate vein. The left superior vena cava (SVC) drains directly into the left atrium, evident from high-density contrast pooling from a left upper extremity injection. There is also a remnant coronary sinus communicating between the atria, indicative of an unroofed coronary sinus or a sinus venosus type ASD. Bolus timing images confirm a significant right-to-left shunt, with early contrast opacification of the aorta and delayed opacification of the pulmonary veins.
Key takeaways
A persistent left SVC draining directly into the left atrium, often in conjunction with an unroofed coronary sinus and an ASD (a complex sometimes referred to as Raghib syndrome), is a cause of right-to-left shunting and hypoxemia. This complex congenital anomaly can be incompletely addressed by initial ASD repair if not fully evaluated preoperatively. Comprehensive imaging, including bolus timing, is critical to accurately diagnose and plan for surgical or interventional correction, such as SVC occlusion or additional repair. The findings suggest the patient had only a partial correction of her congenital heart disease.
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