title: What to expect after repair of total anomalous pulmonary venous connection: data from 193 patients and 2902 patient years.
abstract: OBJECTIVE
  Total anomalous pulmonary venous connection (TAPVC) occurs as isolated cases, in combination with single ventricle physiology, and may be complicated by pulmonary venous obstruction. We sought to identify potential risk factors for long-term mortality and reoperations.
  
  
  METHODS
  Data from 193 consecutive patients who had undergone repair of TAPVC between 1974 and 2011 were analysed using multivariate Cox regression. Mean follow-up time was 15.0 ± 11.0 years, 95% complete.
  
  
  RESULTS
  Survival was 82.7 ± 2.9% at 20 years. Single ventricle physiology (5.9% of the patients, P < 0.001) emerged as the only significant risk factor for mortality in multivariate analyses. Freedom from cardiac reoperation was 82.2 ± 3.3% at 20 years. Single ventricle physiology (P < 0.001) was the only risk factor for cardiac reoperations in multivariate analyses. Freedom from reoperations for pulmonary venous obstruction was 90.4 ± 2.5% at 20 years. An age at operation of ≤30 days (52.8% of the patients, P = 0.007) was the only risk factor for reoperations for pulmonary venous obstruction in univariate analyses. In patients with isolated TAPVC (n = 177), preoperative pulmonary venous obstruction (53.7% of the patients, P = 0.030) and deep hypothermic circulatory arrest (78.5% of the patients, P = 0.017) emerged as risk factors for mortality in univariate analyses. An age at operation of ≤30 days (53.7% of the patients, P = 0.022) was the only risk factor for reoperations for pulmonary venous obstruction in univariate analyses.
  
  
  CONCLUSIONS
  Survival into the third decade without reoperations is excellent in patients with isolated TAPVC without preoperative pulmonary venous obstruction, irrespective of the type of anomalous connection. In contrast, survival of patients with TAPVC and single ventricle physiology is among the poorest of all congenital heart defects. Reoperations for pulmonary venous obstruction are rare and are predominantly required in patients who were operated on as neonates. Survival may be improved by using a strategy of low-flow cardiopulmonary bypass.
authors.affiliation: Department of Cardiovascular Surgery, German Heart Center Munich at the Technical University, Munich, Germany.
authors.forename: Jürgen
authors.initials: J
authors.lastname: Hörer
authors.forename: Caroline
authors.initials: C
authors.lastname: Neuray
authors.forename: Manfred
authors.initials: M
authors.lastname: Vogt
authors.forename: Julie
authors.initials: J
authors.lastname: Cleuziou
authors.forename: Jelena
authors.initials: J
authors.lastname: Kasnar-Samprec
authors.forename: Rüdiger
authors.initials: R
authors.lastname: Lange
authors.forename: Christian
authors.initials: C
authors.lastname: Schreiber
mesh_terms: D000704:Analysis of Variance; D006348:Cardiac Surgical Procedures / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D012086:Reoperation; D012189:Retrospective Studies; D012307:Risk Factors; D012587:Scimitar Syndrome / Q000601:surgery*
keywords: Anomalous pulmonary venous return; Congenital; Long-term results; Surgery; Total anomalous pulmonary venous connection