Coronary Sinus Atresia With Persistent Left Superior Vena Cava: Unusual Clinical Presentation and Endovascular Management

Abstract

Atresia of the coronary sinus (ACS) is a rare congenital anomaly. When associated with persistent left superior vena cava (PLSVC), this defect could have no significant hemodynamic effect, and the patient might remain asymptomatic. However, vascular interventions might induce changes or complications that could show the anomaly. Appropriate management requires a good understanding of this condition. We present the first reported case of ACS and PLSVC occurring after thrombosis of the innominate vein (IV) after central venous catheter placement. The patient presented with atypical subacute chest pain and recurrent extrasystoles. Diagnosis and characterization of vascular anomalies was made by computed tomography phlebography, and the patient was successfully managed by endovascular recanalization of the IV.

A curious case of impossible coronary sinus cannulation

“…Inadvertent ligation of the left-sided superior vena cava during Fontan or bi-directional Glenn in a patient with coronary sinus atresia will result in the venous congestion and myocardial ischemia due to the elevation of the trans-capillary pressure with reduced myocardial perfusion pressure. Rarely, catheter induced thrombosis of left superior vena cava can present as acute coronary syndrome [5].…”

Persistent Left Superior Vena Cava in Hematological Malignancy Requiring Central Venous Catheter Insertion for Intensive Chemotherapy

“…Some researchers have called attention to the risk of atresia of the coronary sinus among the anomalies associated with PLSVC [ 4 , 6 ], but there are no recommendations regarding anticoagulation therapy for patients with PLSVC. Some anecdotal case reports describe PLSVC-associated arrhythmia such as atrial fibrillation [ 7 , 8 ].…”