Outcomes of total cavopulmonary connection for single ventricle palliation.

Background: 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 intensive care unit (ICU) stay, and early and mid-term morbidity and mortality.
Results: The median age at surgery was 3.0 years. The main single ventricle diagnoses included 18 cases of a double-inlet single ventricle, 17 cases of heterotaxy, 16 cases of tricuspid atresia, 4 cases of mitral atresia, 12 cases of unbalanced complete atrioventricular canal (CAVC), 5 cases of double-outlet right ventricle (DORV) combined with ventricular septal defect (VSD) and pulmonary stenosis (PS), 4 cases of transposition of the great arteries (TGA) combined with VSD and PS, 4 cases of corrected transposition of great arteries (cTGA) combined VSD and PS, and 2 cases of criss-cross heart. Preoperative mean pulmonary artery pressure (mPAP) 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 mPAP >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 totally anomalous pulmonary venous connection (TAPVC) had significantly worse mid-term survival.
Conclusions: Staged TCPC improved the early and mid-term survival of patients with a single ventricle. mPAP >15 mmHg was independently associated with prolonged pleural effusions and a fenestration significantly associated with a lower odds of effusion.