Case report of a left superior vena cava to left atrial connection treated with percutaneous covered stent placement

Persistent left superior vena cava (LSVC) with connection to the left atrium (LA) is a rare anomaly with serious clinical implications. Depending on the direction of flow through the intracardiac shunt, clinical presentation varies from cyanosis or paradoxical embolism to overt right heart failure.

A 26-year-old man with repaired ventricular septal defect (VSD) during infancy presented with symptoms of progressive exercise intolerance. Cardiac imaging revealed a large defect at the posterior wall of the LSVC above its entry to the coronary sinus (CS), connecting the LSVC with the LA and resulting in diversion of pulmonary venous return to the CS. All pulmonary veins connect normally to the LA. The large left-to-right intracardiac shunt led to significant right ventricular dilation and tricuspid regurgitation. He underwent successful anatomical repair with transcatheter implantation of covered stent from LSVC to the CS, redirecting pulmonary venous return to the LA. At 1 year follow up, his exercise capacity had improved, and cardiac imaging showed complete seal of the LSVC defect without obstruction to pulmonary venous return.

Our case is the first to our knowledge to report this unusual anatomic variant of LSVC to LA connection, and complete repair by transcatheter treatment. Previous case reports of other forms of LSVC to LA connection were treated with surgery or device occlusion without reconnection of LSVC. This case highlights the efficacy and safety of innovative percutaneous techniques in the management of complex congenital heart lesions. Meticulous procedural planning through 3D modelling and simulation is vital to mitigate the risks of these innovative procedures.