Unroofed coronary sinus

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.

It is associated with persistent left-sided SVC and heterotaxy syndromes.

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.

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Article information

rID: 37047
System: Cardiac
Synonyms or Alternate Spellings:
  • Coronary sinus ASD
  • Coronary sinus atrial septal defect
  • Un-roofed coronary sinus

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