title: Results of extracardiac conduit total cavopulmonary connection in 500 patients.
abstract: OBJECTIVE
  This single-institution study aimed to evaluate the early to mid-term outcomes of extracardiac conduit total cavopulmonary connection (EC-TCPC).
  
  
  METHODS
  Between March 1994 and March 2014, 500 patients (median age, 3.4 years) underwent EC-TCPC at our hospital. One hundred and twenty-three patients (24.6%) showed heterotaxy, and fenestration was created in 6 patients (1.2%). The standard institutional treatment policy included postoperative anticoagulation and individualized cardiovascular medication. The mortality and morbidity rates, haemodynamic status, cardiopulmonary exercise capacity and liver examination results during the follow-up period (median, 6.7 years) were retrospectively reviewed.
  
  
  RESULTS
  There were 2 early and 17 late deaths. The Kaplan-Meier estimated survival rate was 96.2% at 10 years and 92.8% at 15 years. Bradyarrhythmia and tachyarrhythmia occurred in 19 and 13 patients, respectively. Other late-occurring morbidities included protein-losing enteropathy in 8, thromboembolism in 5, bleeding complications in 6 and liver cirrhosis in 1 patient. The rate of freedom from late-occurring morbidities was 82.1% at 15 years. In the multivariate analysis, heterotaxy was found to be a predictor for mortality (P = 0.02), whereas age at operation was a predictor for new-onset arrhythmias (P = 0.048). In the cardiopulmonary exercise test (n = 312), the peak VO2 was 84.9 ± 17.3% of the predicted value, which tended to decrease with age (R(2) = 0.32) and elapsed time since operation (R(2) = 0.21). Postoperative cardiac catheterization (n = 468; time from surgery, 3.6 ± 4.3 years) showed central venous pressure of 9.9 ± 2.4 mmHg, ventricular end-diastolic pressure of 5.2 ± 3.3 mmHg, cardiac index of 3.4 ± 0.8 l/min/m(2) and arterial oxygen saturation of 94.2 ± 4.8%. In 101 patients who were followed up for ≥10 years, amino-terminal type III procollagen peptide and collagen type IV levels exceeded the normal ranges in 52.9 and 75.2% of patients, respectively, and liver ultrasonography revealed hyper-echoic spots in 43.3% of patients.
  
  
  CONCLUSIONS
  The early to mid-term outcomes of post-EC-TCPC patients managed with individualized pharmacotherapy were excellent, with low mortality and morbidity rates; however, development of late-occurring morbidities specific to Fontan physiology, including exercise intolerance and liver disease, must be carefully monitored during the long-term follow-up.
authors.affiliation: Department of Cardiovascular Surgery, Fukuoka Children's Hospital, Fukuoka, Japan nakano.to@fcho.jp.
authors.forename: Toshihide
authors.initials: T
authors.lastname: Nakano
authors.affiliation: Department of Cardiovascular Surgery, Fukuoka Children's Hospital, Fukuoka, Japan.
authors.forename: Hideaki
authors.initials: H
authors.lastname: Kado
authors.affiliation: Department of Cardiovascular Surgery, Fukuoka Children's Hospital, Fukuoka, Japan.
authors.forename: Hideki
authors.initials: H
authors.lastname: Tatewaki
authors.affiliation: Department of Cardiovascular Surgery, Fukuoka Children's Hospital, Fukuoka, Japan.
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Hinokiyama
authors.affiliation: Department of Cardiovascular Surgery, Fukuoka Children's Hospital, Fukuoka, Japan.
authors.forename: Shinichiro
authors.initials: S
authors.lastname: Oda
authors.affiliation: Department of Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan.
authors.forename: Hiroya
authors.initials: H
authors.lastname: Ushinohama
authors.affiliation: Department of Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan.
authors.forename: Koichi
authors.initials: K
authors.lastname: Sagawa
authors.affiliation: Department of Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan.
authors.forename: Makoto
authors.initials: M
authors.lastname: Nakamura
authors.affiliation: Department of Neonatal Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan.
authors.forename: Naoki
authors.initials: N
authors.lastname: Fusazaki
authors.affiliation: Department of Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan.
authors.forename: Shiro
authors.initials: S
authors.lastname: Ishikawa
mesh_terms: D000367:Age Factors; D002675:Child, Preschool; D015444:Exercise; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects / Q000379:methods* / Q000401:mortality; D059446:Heterotaxy Syndrome / Q000401:mortality / Q000601:surgery; D006801:Humans; D008107:Liver Diseases / Q000209:etiology; D008297:Male; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome
keywords: Extracardiac; Fontan; Late complication; Outcome; Total cavopulmonary connection