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

Abstract

Background 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. Case Summary 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 left atrium and resulting in diversion of pulmonary venous return to the CS. All pulmonary veins connect normally to the left atrium. 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 10 month follow up, his exercise capacity had improved, and cardiac imaging showed complete seal of the LSVC defect without obstruction to pulmonary venous return. Discussion 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 modeling and simulation is vital to mitigate the risks of these innovative procedures.

Left atrial appendage occlusion using hydrogel coils in a child

“…3,5 In unique scenarios, transcatheter treatment can be an option. 4,6,7 LSVC baffle leak presentation and management are not well described in the literature. 3 However, in patients with D transposition of the great arteries who underwent arterial switch, baffle leak is a well-recognized cause of desaturation, which can be closed using occluder devices or covered stents.…”

“…The alternative is ligating the LSVC above its drainage into the LA, usually preferred when the bridging vein is well‐developed 3,5 . In unique scenarios, transcatheter treatment can be an option 4,6,7 . LSVC baffle leak presentation and management are not well described in the literature 3 .…”