Cor triatriatum: clinical presentation and surgical results in 12 patients.

Twelve patients with cor triatriatum have been seen at our institution since 1979. The clinical presentation, diagnostic evaluation, and surgical results are outlined in this retrospective review. Operation is the treatment of choice for this rare congenital cardiac defect. One patient died 1 day before scheduled operation, and 2 patients died postoperatively, yielding a surgical mortality rate of 17% and an overall mortality rate of 25%. Resection of the obstructing atrial membrane was performed using hypothermic cardiopulmonary bypass in all cases. Left atriotomy was performed in 6 patients, and right atriotomy was performed in 7. The two postoperative deaths occurred in patients who had serious associated cardiac defects. Associated anomalies include atrial septal defect, persistent left superior vena cava, and partial anomalous pulmonary venous return. The postoperative course has been excellent in all 9 surviving patients; all remain asymptomatic. Cor triatriatum is amenable to surgical repair with excellent results when diagnosed early and when not complicated by other severe cardiac anomalies.