Evaluation of Unroofed Coronary Sinus Syndrome Using Cardiovascular CT Angiography: An Observational Study

The purpose of this study was to determine the prevalence of unroofed coronary sinus (CS) syndrome at a tertiary hospital and analyze the clinical information, cardiovascular CT angiography (CCTA) imaging findings, associated anomalies, and surgical treatment of the identified cases.

We retrospectively searched the database of a tertiary hospital for cases of unroofed CS syndrome among patients who underwent CCTA for known or suspected congenital heart disease. After the prevalence of unroofed CS syndrome was determined, CCTA findings, associated cardiovascular abnormalities, presence or absence of airway compression, clinical information, and surgical outcome were recorded.

A total of 23 patients with unroofed CS syndrome were identified, with the syndrome therefore having a prevalence of 0.36% among patients with congenital heart disease who underwent CCTA. The diagnostic accuracy of CCTA for unroofed CS syndrome was 100%, whereas that of echocardiography was 69%. Type I unroofed CS syndrome was the most commonly noted type (52% of patients). All 23 patients had associated cardiovascular anomalies, including persistent left superior vena cava (65% of patients) and atrial septal defect (65%). Surgery was performed for 70% of patients because of cardiovascular anomalies. Seven patients (30%) had associated secondary airway compression but did not require surgical correction.

At our institution, the prevalence of unroofed CS syndrome was 0.36% among patients with congenital heart disease who underwent CCTA. CCTA has excellent diagnostic performance, delineating different subtypes of unroofed CS syndrome and associated cardiovascular planning for treatment of unroofed CS syndrome abnormalities, improving clinical decision making, and permitting preoperative planning for treatment of unroofed CS syndrome.