Data regarding long-term outcome after percutaneous closure of left superior caval vein draining into the left atrium are lacking. The aim of the present study was to report the long-term follow-up by using contrast-enhanced CT.

In all, three patients underwent percutaneous closure of left superior caval vein draining into the left atrium between 2005 and 2015. All of them were evaluated clinically and underwent contrast-enhanced CT.

In one patient, the Amplatzer® Septal Occluder was used. In two patients, the Amplatzer® Vascular Plug type-1 was preferred: the device size/LSVC diameter ratio was 1.7 in the child and 1.2 in the adult. There were no early-onset or long-term onset complications. CT was performed 1, 2, and 10 years after the procedure, respectively. Complete occlusion of the vessel was documented in all. After 10 years since the procedure, CT revealed a persistent trivial residual shunt through the accessory hemiazygos vein in one patient, in whom the device was implanted above its drainage into the left superior caval vein. When an Amplatzer® Vascular Plug type-1 is oversized compared with the venous vessel diameter, it immediately assumes a dog-bone shape that disappears early to regain its shape memory and nominal size.

Percutaneous occlusion of left superior caval vein draining into the left atrium has excellent early and long-term outcomes. The optimal implantation of the device is below the drainage of the accessory hemiazygos vein, when present. The device might be oversized compared with the left superior caval vein diameter according to the age of the patient.

The pathophysiological entity of a persisting left-sided superior caval vein draining into the roof of the left atrium represents an extreme form of coronary sinus de-roofing. This is an uncommon, but well-documented condition associated with systemic desaturation due to a right-to-left shunt. Depending on the size of the coronary ostium, the defect may also present with right-sided volume loading. We describe two patients, both of whom presented with desaturation, and highlight the important anatomical features underscoring management.

Both patients were managed interventionally with previous assessment of the size of the coronary sinus ostium through cross-sectional imaging. This revealed a restrictive interatrial communication at the right atrial mouth of the coronary sinus in both patients, which permitted an interventional approach, as the residual left-to-right shunt subsequent to closure of the aberrant vessel would be negligible. At intervention, test occlusion of the left superior caval vein allowed assessment of decompressing vessels before successful occlusion using an Amplatzer Vascular Plug.

Persistence of a left superior caval vein draining to the left atrium may be associated with an interatrial communication at the mouth of the unroofed coronary sinus. The ostium of the de-roofed coronary sinus can be atretic, restrictive, normally sized, or enlarged. Careful assessment of the size of this defect is required before treatment. In view of its importance, which has received little attention in the literature to date, we suggest an additional consideration to the classification of unroofed coronary sinus.