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Unroofed Coronary Sinus Atrial Septal Defect: Diagnosis by Multimodality Cardiac Imaging

Unroofed coronary sinus atrial septal defect (ASD), a rare congenital cardiac anomaly, is essentially a direct communication between the coronary sinus and the left atrium. The associated interatrial shunt can lead to right-sided volume overload and paradoxical emobolization. It is commonly associated with a persistent left superior vena cava (PLSVC). An unroofed coronary ASD can be further classified into 4 subtypes: type I, completely unroofed with PLSVC; type II, completely unroofed without PLSVC; type III, partially unroofed midportion; type IV, partially unroofed terminal portion. Although the diagnosis can be suspected by two-dimensional (2D) echocardiography (Philips, Andover, MA, USA), the precise subtype can be difficult to delineate. 3D echocardiography and multidetector computed tomographic angiography (MDCT) can provide incremental evaluation of this condition.