Case report
A 3-year-old girl presented with failure to thrive. An echocardiogram revealed a partial atrioventricular septal defect, a persistent left superior caval vein draining to the left atrium with an unroofed coronary sinus, and a small secundum atrial septal defect (Fig 1).
Figure 1. Transthoracic echocardiogram showing all three inter-atrial defects as labelled.
At surgery, three interatrial communications were identified – a large ostium primum defect, a small secundum defect, and a coronary sinus defect (Fig 2). The left caval vein drained into the left atrium.
Figure 2. Intra-operative photograph displaying the three inter-atrial defects.
The surgical procedure involved repair of the left atrioventricular valve, closure of the primum defect and coronary sinus defect with a single autologous pericardial patch, and direct closure of the secundum defect. The left caval vein was translocated to the right atrial appendage.
Discussion
Coronary sinus defects are rare interatrial communications that lie outside the confines of the atrial septum. They are formed as a result of defects in the embryonic left arteriovenous fold forming the common wall between coronary sinus and left atrium. They may or may not be associated with a persistent left caval vein. Primum and secundum atrial septal defects are the result of incomplete formation, resolution, and fusion of the septum primum and septum secundum. Reference Naqvi, McCarthy and Ho1
The concurrence of three types of interatrial communications in a single patient is a rarity and is therefore being presented. The surgical management is relatively straightforward.
Acknowledgements
None.
Financial support
This research received no specific grant from any funding agency, commercial or not-for-profit sectors.
Conflict of interest
None.
