Scimitar syndrome is a rare congenital anomaly characterized by anomalous right pulmonary venous drainage into the inferior vena cava, resulting in a left-to-right shunt. Surgical correction may be achieved by direct reimplantation of the scimitar vein or by intra-atrial baffling, which often requires deep hypothermic circulatory arrest. We report the case of a 47-year-old man with symptomatic scimitar syndrome and an infradiaphragmatic scimitar vein who underwent successful intra-atrial baffling without circulatory arrest. Preoperative imaging demonstrated a significant shunt with dilation of the right atrium and ventricle. Repair was performed using cardiopulmonary bypass with femoral venous cannulation and vacuum-assisted lower body venous drainage, allowing adequate visualization despite the challenging infradiaphragmatic localization of the anomalous vein without interruption of systemic perfusion. Postoperative echocardiography confirmed unobstructed pulmonary venous return with minimal gradient. The patient had an uneventful recovery and reported symptomatic improvement at 1-year follow-up. This case demonstrates that intra-atrial baffling of an infradiaphragmatic scimitar vein can be safely performed without circulatory arrest using vacuum-assisted venous drainage.
Székely et al. (Thu,) studied this question.