Abstract
Isolated right superior vena cava (RSVC) drainage into the left atrium (LA) is a rare congenital anomaly, presenting diagnostic and management challenges. This study presents two cases of isolated RSVC drainage into the LA alongside a comprehensive literature review to improve understanding and delineate optimal surgical approaches. The study describes two cases of isolated RSVC drainage into the LA and their surgical management. Additionally, it includes a narrative literature review summarizing existing knowledge and highlighting key clinical insights regarding this rare anomaly. Both cases exhibited diverse clinical manifestations, such as cerebral abscess and dyspnea, leading to detailed evaluation revealing RSVC drainage into the LA. Surgical correction involved intracardiac baffle surgery in one case and extracardiac re-implantation in the other, resulting in successful symptom resolution. The literature review uncovered challenges in diagnosis, embryological hypotheses, and various surgical techniques employed for correction. Isolated RSVC drainage into the LA poses a diagnostic and therapeutic challenge due to its rarity. Recognizing salient clinical features and diverse presentations underscores the importance of early identification and appropriate surgical intervention. These cases contribute to understanding this anomaly and advocate for tailored surgical approaches based on individual patient characteristics.