title: Long-term outcomes of transatrial-transpulmonary repair of tetralogy of Fallot.
abstract: OBJECTIVE
  The surgical approach to repair of tetralogy of Fallot (ToF) has shifted over the years. We aimed to report the long-term follow-up after ToF repair with the transatrial-transpulmonary approach and to determine predictors of long-term outcomes.
  
  
  METHODS
  Retrospective analysis of patients operated on in two tertiary referral centres. Primary outcome measures were: death, pulmonary valve replacement (PVR), reintervention for other reasons, internal cardiodefibrillator and/or pacemaker placement. Kaplan-Meier assessment of overall and event-free survival as well as uni- and multivariate analyses of risk factors for outcomes were performed.
  
  
  RESULTS
  Four hundred and fifty-three patients were included. Median age at operation was: 0.6 years (range 0-19.6) and median age at the last follow-up was 14.3 years (range 0.1-42.1). Median age at repair decreased from 1.2 years (range 0.6-5.8) (1970-80) to 0.3 years (range 0-4.7) (2000-12). A transannular patch (TP) was used in 65% of all patients. The use of a TP showed a decline from 89% in the initial years of the cohort to 64% in 2000-12. Early mortality was 1.1% (5 patients) for the entire cohort and late mortality 2.4% (11 patients). Overall survival for the entire cohort was 97.3% (95% CI 95.7-98.8) and 91.8% (95% CI 85.9-97.7) at 10 and 25 years, respectively. For patients with a TP (n = 294) vs non-TP (n = 159), this was 97.2% (95% CI 95.2-99.2) vs 97.5% (95% CI 95.1-99.9) at 10-year and 91.0% (95% CI 83.9-98.1) vs 96.3% (95% CI 93.0-99.6) at 25-year follow-up (P = 0.958). Fifty-two patients underwent PVR, and in 5 a pacemaker was inserted. Event-free survival for TP versus non-TP patients was 80.2% (95 CI% 75.5-84.9) vs 81.7% (95% CI 75.2-88.2) at 10-year and 27.9% (95% CI 17.7-38.1) vs 78.5% (95% CI 71.4-85.6) at 25-year follow-up (P = 0.016). In multivariate analysis, both the use of a TP (HR 1.705, 95% CI 1.023-2.842) and the year of surgical repair of tetralogy of Fallot (HR 1.039, 95% CI 1.006-1.073) were associated with a higher probability of an event.
  
  
  CONCLUSIONS
  ToF patients corrected with the transatrial-transpulmonary approach have good long-term survival. PVR is a frequent event at longer follow-up, and other events are limited. The use of a TP is a predictor for poorer event-free outcomes, increasing the risk of the composite endpoint 1.7 times.
authors.affiliation: Department of Paediatrics (Cardiology), Erasmus University Medical Center-Sophia Children's Hospital, Rotterdam, the Netherlands Department of Paediatrics (Cardiology), University Medical Center St Radboud, Nijmegen, the Netherlands.
authors.forename: Linda W G
authors.initials: LW
authors.lastname: Luijten
authors.affiliation: Department of Paediatrics (Cardiology), Erasmus University Medical Center-Sophia Children's Hospital, Rotterdam, the Netherlands Department of Paediatrics (Cardiology), University Medical Center St Radboud, Nijmegen, the Netherlands.
authors.forename: Eva
authors.initials: E
authors.lastname: van den Bosch
authors.affiliation: Department of Paediatrics (Cardiology), Erasmus University Medical Center-Sophia Children's Hospital, Rotterdam, the Netherlands Department of Paediatrics (Cardiology), University Medical Center St Radboud, Nijmegen, the Netherlands Department of Radiology, Erasmus University Medical Center, Rotterdam, the Netherlands.
authors.forename: Nienke
authors.initials: N
authors.lastname: Duppen
authors.affiliation: Department of Paediatrics (Cardiology), University Medical Center St Radboud, Nijmegen, the Netherlands.
authors.forename: Ronald
authors.initials: R
authors.lastname: Tanke
authors.affiliation: Department of Cardiology, Erasmus University Medical Center, Rotterdam, the Netherlands.
authors.forename: J
authors.initials: J
authors.lastname: Roos-Hesselink
authors.affiliation: Department of Cardiothoracic Surgery, University Medical Center St Radboud, Nijmegen, the Netherlands.
authors.forename: Aagje
authors.initials: A
authors.lastname: Nijveld
authors.affiliation: Department of Cardiology, University Medical Center St Radboud, Nijmegen, the Netherlands.
authors.forename: Arie
authors.initials: A
authors.lastname: van Dijk
authors.affiliation: Department of Cardiothoracic Surgery, Erasmus University Medical Center, Rotterdam, the Netherlands.
authors.forename: Ad J J C
authors.initials: AJ
authors.lastname: Bogers
authors.affiliation: Department of Cardiothoracic Surgery, Erasmus University Medical Center, Rotterdam, the Netherlands.
authors.forename: Ron
authors.initials: R
authors.lastname: van Domburg
authors.affiliation: Department of Paediatrics (Cardiology), Erasmus University Medical Center-Sophia Children's Hospital, Rotterdam, the Netherlands Department of Radiology, Erasmus University Medical Center, Rotterdam, the Netherlands w.a.helbing@erasmusmc.nl.
authors.forename: Willem A
authors.initials: WA
authors.lastname: Helbing
mesh_terms: D000293:Adolescent; D000328:Adult; D006348:Cardiac Surgical Procedures / Q000379:methods; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D011664:Pulmonary Valve / Q000601:surgery*; D012189:Retrospective Studies; D013771:Tetralogy of Fallot / Q000601:surgery*; D016896:Treatment Outcome; D055815:Young Adult
keywords: Outcome; Pulmonary valve replacement; Tetralogy of Fallot; Transannular patch