[Unroofed coronary sinus syndrome].

Unroofed coronary sinus syndrome or coronary sinus septal defect is a rare congenital cardiac anomaly. We performed corrective operations in 9 patients with such condition. Of them, seven patients had the completely unroofed coronary sinus and persistent left superior vena cava (PLSVC) directly draining into the left atrium (LA). One patient had PLSVC and the partially unroofed terminal portion of the coronary sinus (CS), or sinus ostium open into LA. Another patient had total anomalous pulmonary vein connection and the partially unroofed mid-portion of CS without PLSVC. The operative methods of the unroofed coronary sinus included: ligating PLSVC and repairing ostium primum or secundum atrial septal defects in 4 cases; creating an intra-atrial tunnel from PLSVC to the right atrium (RA) and repairing the atrial septal defect in 2 cases; reconstructing the intra-atrial septal with a patch as a baffle to guide PLSVC or sinus ostium respectively towards RA in 2 cases; enlarging the defect on the coronary sinus roof and repairing the atrial septal defect of the coronary sinus type in one case. No patient died in this group. Because unroofed coronary sinus syndrome has atypical clinical manifestation and usually complicates varied congenital anomalies, its preoperative diagnosis is very difficult in some times and may draw the reliable support from echocardiography and catheterization. The operative program and method must depend not only upon its type but also upon PLSVC presence of absence, and communication between PLSVC and RSVC.