An unroofed coronary sinus is a rare variant of atrial septal defect (ASD). The atrial wall between the coronary sinus and left atrium is either partially or completely absent, resulting in a left-to-right shunt.
Clinical presentation is variable and depends on the degree of left-to-right shunting. When there are associated symptoms it is then often termed unroofed coronary sinus syndrome (URCS) 4.
Unroofed coronary sinus has been classified into 4 groups:
- type I: completely unroofed with persistent LSVC
- type II: completely unroofed without persistent LSVC
- type III: partially unroofed mid portion
- type IV: partially unroofed terminal portion
The diagnosis is often made on transthoracic echocardiography, but because of the deep position of the anomaly within the chest, transesophageal echocardiography, cardiac CT, or cardiac MRI may also be useful.
- 1. Thangaroopan M, Truong QA, Kalra MK et-al. Images in cardiovascular medicine. Rare case of an unroofed coronary sinus: diagnosis by multidetector computed tomography. Circulation. 2009;119 (16): e518-20. doi:10.1161/CIRCULATIONAHA.107.707018 - Free text at pubmed - Pubmed citation
- 2. Ootaki Y, Yamaguchi M, Yoshimura N et-al. Unroofed coronary sinus syndrome: diagnosis, classification, and surgical treatment. J. Thorac. Cardiovasc. Surg. 2004;126 (5): 1655-6. Pubmed citation
- 3. https://www.ncbi.nlm.nih.gov/pubmed/23450671 - to be linked
- 4. https://heart.bmj.com/content/97/Suppl_3/A208.1 - to be linked