Surgical experience with unroofed coronary sinus.

Between January, 1967, and October, 1977, we performed intracardiac repair in 24 patients with unroofed coronary sinus. Eight patients had the so-called pure form of the syndrome, with the concomitant findings of total absence of the partition between the coronary sinus and left atrium, connection of the left superior vena cava (LSVC) to the upper left corner of the left atrium, and a coronary sinus type of atrial septal defect. In 2 additional patients this combination was repaired, along with repair of the common atrium in 1 and tetralogy of Fallot in the other. In 4 patients with either a partial or complete atrioventricular canal defect, only the distal or downstream portion of the sinus was unroofed so that the coronary sinus ostium was considerably to the left of its usual location. In 6 patients, 5 of whom had situs ambiguous, the unroofed coronary sinus was associated with complex congenital heart disease, and in 5 there was a L(contralateral) SVC. Three of these 6 patients died. The basic repair consists of roofing the coronary sinus from within the left atrium, so that the LSVC drains through the newly created coronary sinus and its ostium into the right atrium. In the absence of a LSVC, the basic repair is simply closure of the coronary sinus atrial septal defect. This leaves the coronary veins draining into the left atrium.