Partially Unroofed Coronary Sinus

Abstract

A 52-year-old man presented with moderate-effort dyspnea for 2 years, cardiomegaly on the chest radiograph, and atrial fibrillation on the ECG. The transthoracic echocardiography image was unclear because of obesity. Transesophageal echocardiography showed both atrial and right ventricular enlargement (Figure 1) and normal pulmonary venous return. An intact interatrial septum was found on the 4-chamber view of the transverse plane. A defect of the partial coronary sinus was found on the near longitudinal plane, and a significant left-to-right shunt was demonstrated by color Doppler flow imaging (Figure 1). With further rotation of the transducer, an intact interatrial septum and a defect beyond the realm of the atrial septum were obtained simultaneously in the same view (Figure 2). Diameter of the coronary sinus defect was 2.3 cm. The left superior vena cava was not confirmed by injection of agitated salt into the left median cubital vein. A coronary sinus defect may also be suggested by the catheter passing the coronary sinus through the left atrium and pulmonary vein. Selection angiography of the coronary sinus confirmed the communication between the coronary sinus and left atrium. Pulmonary artery pressure was 60/18 (mean 34) mm Hg. Pulmonary-to-systemic blood flow ratio was 1.68. The pulmonary vascular resistance was 3.2 Wood units. The patient was referred for surgical repair of the lesion. DisclosuresNone.

A Case of Complete Unroofed Coronary Sinus Syndrome Combined With Coronary Sinus Stenosis Leading to Asymptomatic Presentation

“…The unroofed CS syndrome is a rare congenital anomaly that is often associated with a persistent left SVC ( 1 - 5 ). The classification of the unroofed CS syndrome varies in the literature ( 1 - 5 ). It can be classified simply complete or partial depending on the size of the defect between the CS and the left atrium.…”

“…the degree of left-to-right shunting) and associated anomalies such as a persistent left SVC (i.e. brain abscess or infarction caused by a right-to-left shunt) ( 1 - 5 ). The present case had no related symptoms in spite of a large defect between the CS and the left atrium.…”

“…The unroofed coronary sinus (CS) syndrome is a rare entity ( 1 - 5 ). Approximately, 75% of cases with the unroofed CS syndrome are associated with a persistent left superior vena cava (SVC) ( 1 ).…”

Partial Unroofed Coronary Sinus Associated With Upper Septal Ventricular Tachycardia and Atrioventricular Nodal Reentrant Tachycardia

“…Isolated partial unroofing to the coronary sinus with a left to right shunt is a rare anomaly. [1., 2., 3.] Unroofed coronary sinus or coronary sinus diverticulums have been associated with accessory pathway and related tachycardia. [4,5]…”

Partially unroofed coronary sinus with persistent left superior vena cava: the utility of two and three-dimensional transesophageal echocardiography: a case report

“…TEE is more accurate in visualizing these posterior cardiac structures and provides better delineation of anatomy and size of the defect. On TEE, URCS may be seen in the midesophageal two-chamber or long-axis views [8,9]. When URCS is present, the CS wall closest to the LA appears to be missing, suggesting communication between the posterior aspect of the LA and the CS [8].…”