title: Clinical outcome following total cavopulmonary connection: a 20-year single-centre experience.
abstract: OBJECTIVE
  This study aims to evaluate the clinical outcome following total cavopulmonary connection (TCPC) and to identify factors affecting early and late outcome.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Cardiovascular Surgery, German Heart Center Munich at the Technische Universität München, Munich, Germany ono@dhm.mhn.de.
authors.forename: Masamichi
authors.initials: M
authors.lastname: Ono
authors.affiliation: Department of Cardiovascular Surgery, German Heart Center Munich at the Technische Universität München, Munich, Germany.
authors.forename: Jelena
authors.initials: J
authors.lastname: Kasnar-Samprec
authors.affiliation: Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Center Munich at the Technische Universität München, Munich, Germany.
authors.forename: Alfred
authors.initials: A
authors.lastname: Hager
authors.affiliation: Department of Cardiovascular Surgery, German Heart Center Munich at the Technische Universität München, Munich, Germany.
authors.forename: Julie
authors.initials: J
authors.lastname: Cleuziou
authors.affiliation: Department of Cardiovascular Surgery, German Heart Center Munich at the Technische Universität München, Munich, Germany.
authors.forename: Melchior
authors.initials: M
authors.lastname: Burri
authors.affiliation: Department of Cardiovascular Surgery, German Heart Center Munich at the Technische Universität München, Munich, Germany.
authors.forename: Constantin
authors.initials: C
authors.lastname: Langenbach
authors.affiliation: Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Center Munich at the Technische Universität München, Munich, Germany.
authors.forename: Alessia
authors.initials: A
authors.lastname: Callegari
authors.affiliation: Department of Cardiovascular Surgery, German Heart Center Munich at the Technische Universität München, Munich, Germany.
authors.forename: Martina
authors.initials: M
authors.lastname: Strbad
authors.affiliation: Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Center Munich at the Technische Universität München, Munich, Germany.
authors.forename: Manfred
authors.initials: M
authors.lastname: Vogt
authors.affiliation: Department of Congenital Heart Disease, Marie Lannelongue Hospital, Les Plessis-Robinson, France.
authors.forename: Jürgen
authors.initials: J
authors.lastname: Hörer
authors.affiliation: Department of Cardiovascular Surgery, German Heart Center Munich at the Technische Universität München, Munich, Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Schreiber
authors.affiliation: Department of Cardiovascular Surgery, German Heart Center Munich at the Technische Universität München, Munich, Germany.
authors.forename: Rüdiger
authors.initials: R
authors.lastname: Lange
mesh_terms: D002675:Child, Preschool; D005080:Exercise Test; D005260:Female; D018729:Fontan Procedure / Q000379:methods / Q000401:mortality / Q000706:statistics & numerical data; D006228:Hand Deformities, Congenital / Q000401:mortality / Q000601:surgery; D006801:Humans; D007223:Infant; D007677:Kidney Function Tests; D007902:Length of Stay; D008111:Liver Function Tests; D008297:Male; D012086:Reoperation / Q000706:statistics & numerical data; D012189:Retrospective Studies; D012307:Risk Factors; D016896:Treatment Outcome
keywords: Exercise capacity; Extracardiac conduit; Fontan circulation; Functional single ventricle; Tachyarrhythmia; Total cavopulmonary connection