Surgical Outcomes of Cor Triatriatum Sinister: A Single-Center Experience

BACKGROUND: We investigated surgical outcomes after the surgical repair of cor triatriatum sinister (CTS). METHODS: Thirty-two consecutive patients who underwent surgical repair of CTS from 1993 through 2020 were included in this study. The morphological characteristics, clinical features, and surgical outcomes were described and analyzed. RESULTS: The median age and body weight at operation were 9 months (interquartile range [IQR], 3–238 months) and 7.5 kg (IQR, 5.8–49.6 kg), respectively. There were 16 males (50%). According to the modified Lucas classification, type IA (classical CTS) was most common (n=20, 62.5%). Atrial septal defect was associated in 22 patients (68.8%) and anomalous pulmonary venous return in 8 patients (25%). Pulmonary hypertension was preoperatively suspected with a high probability in 18 patients (56.3%). There was 1 early death (3.1%) after emergent membrane excision and hybrid palliation in a high-risk hypoplastic left heart syndrome patient. There were no late deaths. The overall survival rate was 96.9% at 15 years post-repair. No early survivors required reoperation during follow-up. Most survivors (31 of 32 patients, 96.9%) were in New York Heart Association functional class I at a median follow-up of 74 months (IQR, 39–195 months). At the latest echocardiography performed at a median of 42 months (IQR, 6–112 months) after repair, no residual lesion was observed except in 1 patient who had moderate pulmonary hypertension (mean pulmonary arterial pressure of 36 mm Hg). CONCLUSION: Surgical repair of cor triatriatum could be performed safely and effectively with an extremely low risk of recurrence.