OBJECTIVETo analyze symptoms, associated anomalies, diagnostic approach, and surgical procedures in patients with unroofed coronary sinus syndrome and atrioventricular canal defect.

METHODSThe clinical data of 20 patients with unroofed coronary sinus syndrome from September 1999 to October 2007 were reviewed retrospectively. There were 10 male and 10 female patients. The age ranged from 6 months to 38 years old, with a mean of (11.4 +/- 11.0) years old. The body weight ranged from 6.7 to 73.0 kg, with a mean of (28.4 +/- 21.3) kg. There were 18 cases of partial atrioventricular canal defect, 2 cases of complete atrioventricular canal defect, and 12 cases of common atrium. The initial diagnosis of unroofed coronary sinus syndrome was made by the surgeon at repair of a partial or complete atrioventricular canal defect in 20 patients. Complex unroofed coronary sinus with left superior vena cava (LSVC) directly draining into the left atrium was found in 11 cases, 1 case of LSVC was ligated, 10 cases were reconstructed the intraatrial baffle or the intracardiac tunnel to drain LSVC to right atrium. The other 9 patients with simple unroofed coronary sinus were repaired with other procedures. The associated cardiac lesions were corrected concomitantly.

RESULTSDeath occurred in 1 patient with complex congenital cardiac disease due to pulmonary infection. In the 14 early survivors, who had been followed up from 4 months to 3 years, there was no death and severe complications.

CONCLUSIONSWhen associated with a partial or complete atrioventricular canal defect, LSVC and a common atrium, unroofed coronary sinus syndrome should be considered as a possible additional finding. Repair according to the type of unroofed coronary sinus syndrome is effective.