Background: This study aims to analyze the data regarding cases diagnosed with cor triatriatum sinistrum at a single pediatric center, highlighting symptoms on presentation of the anomaly, mode of diagnosis, age at the time of diagnosis, any other accompanying cardiac defects, and outcomes.

Methods: Records of 21 patients (13 females, 8 males; mean age 32.4 month; range 7 days to 57 years) diagnosed with cor triatriatum sinistrum between January 1997 and January 2012 at the pediatric cardiology clinic were analyzed retrospectively. Medical records including demographic data, clinical progress, diagnostic approach, and surgical interventions were reviewed.

Results: The majority of patients (81%) had accompanying cardiac defects, atrial septal defect being the most frequent (33%). Restrictive pattern was detected in five patients. Seven patients (33%) died after diagnosis. We resect the fibromuscular diaphragm in 11 of 21 patients. One patient died after operation. Most common presenting symptoms were lower respiratory tract infections, dyspnea, and early fatigue. Sixteen patients were diagnosed with echocardiography.

Conclusion: Despite the diversity in clinical findings at presentation and accompanying cardiac lesions, cor triatriatum sinistrum is a treatable defect with satisfying treatment outcomes.

Keywords : Cardiac surgery; congenital heart defect; echocardiography