Abstract

A sixty eight year-old woman with a long-standing history of hypertension, dizziness and a history of congenital heart disease presented with speech difficulties and disorientation. She was diagnosed with a brain abscess, confirmed by a stereotactic biopsy. Transthoracic echocardiographic evaluation revealed a persistent left superior vena cava (PLSVC) with an unroofed coronary sinus (URCS) along with a small secundum atrial septal defect. Her heart catheterization showed a partially unroofed coronary sinus along with a bidirectional shunt. She was referred for surgical closure of her unroofed coronary sinus and the secundum atrial septal defect. Her brain abscess responded well to antibiotic treatment. While waiting for open-heart surgery, she suffered from an acute myocardial infarction and underwent emergent percutaneous coronary intervention to the right coronary artery. Subsequently, she underwent elective surgical repair of the unroofed coronary sinus, along with closure of the atrial septal defect. When she was seen in follow-up she reported a complete resolution of her dizziness and felt more energetic. Unroofed coronary sinus syndrome (URCS) is a rare congenital cardiac anomaly in which there is a communication between the coronary sinus and the left atrium. While non-invasive imaging with echocardiography, MRI or CT is helpful in making the diagnosis, cardiac catheterization remains integral in the evaluation and management planning. Management is guided by the presence of clinical symptoms with consideration of repair when patients become symptomatic. Prognosis after surgery is excellent, recently transcatheter based treatment therapies are becoming more frequent. We present a rare case of URCS with PLSVC presenting as a cerebral abscess in late adulthood. She had bidirectional shunting manifesting as a cerebral abscess. She responded well to the corrective surgery and was doing well on follow up.

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