Objectives The aim of this study was to review the early and mid-term outcomes of the total cavopulmonary connection (TCPC) procedure and evaluate risk factors for prolonged pleural effusions.Methods The clinical records of 82 consecutive patients,who underwent a TCPC operation between January 2008 and December 2013,were reviewed for incidence of prolonged pleural effusions,duration of ventilation time and pleural drainage,length of ICU stay,and early and mid-term morbidity and mortality.Results The median age at surgery was 3.0 years.Associated anatomic subtypes were categorized as heterotaxy (n=17,20.7%),common atrioventricular valve (n=16,19.5%),obstructed totally anomalous pulmonary venous connection (n=12,14.6%),or anomalous systemic venous return (n =18,22.0%).Preoperative mean pulmonary artery pressure was 13.66 ± 2.21 mmHg with 23.2% (n=19) higher than 15 mmHg.A total of 61 (74.4%) patients underwent a fenestration.The perioperative mortality was 4.9%.The median duration of pleural effusion was 10 days (range,3-80 days),and prolonged pleural effusions occurred in 16 (19.5%) patients.Multivariable analysis revealed that mean pulmonary artery pressure > 15 mmHg was independently associated with prolonged pleural effusions (OR,8.33;95% CI,2.33-29.74;p=0.001),and creation of a fenestration was associated with decreased odds of effusion (OR,0.21;95% CI,0.06-0.74;p=0.015).Five-year estimated Kaplan-Meier survival of two-stage TCPC was significantly higher than that of one-stage group(96.7% vs.79.7,p=0.023).Patients with heterotaxy or obstructed TAPVC had significantly worse mid-term survival.Conclusions Staged TCPC improved the early and mid-term survival of patients with a single ventricle.Mean pulmonary artery pressure > 15 mmHg was independently associated with prolonged pleural effusions and a fenestration significantly associated with a lower odds of effusion.