Clinical outcome following total cavopulmonary connection: a 20-year single-centre experience

This study aims to evaluate the clinical outcome following total cavopulmonary connection (TCPC) and to identify factors affecting early and late outcome.

Between May 1994 and March 2015, 434 patients underwent TCPC with 50 lateral tunnels and 374 extracardiac conduits. The clinical outcome, exercise capacity and liver examination results were retrospectively reviewed.

Thirty-day survival was 98.2%, and the estimated survival rate at 15 years was 92.3%. Freedom from tachyarrhythmia at 15 years was 91.0%. Other late morbidities included bradyarrhythmia in 17, protein-losing enteropathy (PLE) in 15, thromboembolism in 3 and plastic bronchitis in 3 patients. At last follow-up, normal systemic ventricular function (ejection fraction >50%) was observed in 88.2%. Atrioventricular valve (AVV) regurgitation was mild or less in 90% of patients with systemic left ventricle, in 63% of those with systemic right ventricle and 58% of the patients with unbalanced atrioventricular septal defect or common inlet ventricles. Cardiopulmonary exercise capacity showed impaired peak oxygen uptake (71% of normal) in a sub-group of 120 patients at a mean of 9 years postoperatively. Biochemistry of 338 patients at last follow-up revealed a gamma-glutamyl transferase value beyond normal in 90 patients (26%), with a positive correlation between the level and the time after the initial operation (P < 0.01). Pre-TCPC high transpulmonary gradient emerged as a predictor for delayed hospital recovery (P = 0.002), late mortality (P = 0.016) and reoperation (P = 0.015) in multivariable analysis.

Contemporary TCPC can be performed with low risk and provides excellent survival in the long-term. Classic morbidities of the original Fontan procedure, such as Fontan pathway revision, tachyarrhythmia and thromboembolism seem mitigated. However, exercise limitations, PLE and liver dysfunction remain an issue. AVV insufficiency and ventricular dysfunction are still a concern.