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.

Background:We investigated the long-term outcomes of truncus arteriosus repair at a single institution with a 30-year study period. Methods: Patients who underwent repair of truncus arteriosus between 1993 and 2022 were reviewed retrospectively. Factors associated with early mortality, overall attrition, and reintervention were identified using appropriate statistical methods. Results: In total, 42 patients were enrolled in this study. The median age and weight at repair were 26 days and 3.5 kg, respectively. Thirty patients (71.4%) underwent 1-stage repair. There were 8 early deaths (19%). In the univariable analysis, undergoing surgery before 2011 was associated with early mortality (p=0.031). The overall survival rate at 10 years was 73.8%. In the multivariable analysis, significant truncal valve (TrV) dysfunction (p=0.010), longer cardiopulmonary bypass time (p=0.018), and the earlier era of surgery (p=0.004) were identified as risk factors for overall mortality. During follow-up, 47 reinterventions were required in 27 patients (64.3%). The freedom from all-cause reintervention rate at 10 years was 23.6%. In the multivariable analysis, associated arch obstruction (p<0.001) and significant TrV dysfunction (p=0.011) were identified as risk factors for all-cause reintervention. Arch obstruction (p=0.027) and a number of TrV cusps other than 3 (p=0.014) were identified as risk factors for right ventricle to pulmonary artery (RV-PA) reintervention, and significant TrV dysfunction was identified as a risk factor for TrV reintervention (p=0.002). Conclusion: Despite recent improvements in survival outcomes after repair of truncus arteriosus, RV-PA or TrV reinterventions were required in a significant number of patients during follow-up.