title: Clinical condition at mid-to-late follow-up after transatrial-transpulmonary repair of tetralogy of Fallot.
abstract: OBJECTIVE
  To assess the clinical condition at mid-to-late follow-up in tetralogy of Fallot corrected by a transatrial-transpulmonary approach at a young age and to identify risk factors associated with right ventricular dilation/dysfunction and with decreased exercise tolerance.
  
  
  METHODS
  Patients with tetralogy of Fallot underwent cardiac magnetic resonance imaging, maximal bicycle ergometry, electrocardiography, Holter monitoring, and spirometry. Multivariate linear regression analyses were used to determine independent predictors for selected clinical parameters.
  
  
  RESULTS
  Fifty-nine patients (mean +/- SD), age at repair 0.9 +/- 0.5 years, interval since repair 14 +/- 5 years, were included. The median pulmonary regurgitant fraction was 32% (0%-57%). Compared with published data on healthy controls, Fallot patients had significantly larger right ventricular end-diastolic and end-systolic volumes and smaller right ventricular and left ventricular ejection fractions. Maximum oxygen consumption was 97% +/- 17% and maximum workload 89% +/- 13% of predicted. Median QRS duration was 110 ms (82-161 ms). No important ventricular arrhythmias were found. Compared with patients without a transannular patch, patients with a patch had more pulmonary regurgitation, a larger right ventricle, worse right ventricular and left ventricular ejection fractions, but comparable exercise capacity. Multivariate regression analysis identified the following independent determinants for larger right ventricular volumes: longer interval since repair, longer QRS duration, and higher pulmonary regurgitation percentage. The following were independent determinants for smaller right ventricular ejection fraction: abnormal right ventricular outflow tract wall motion, longer interval since repair, and longer QRS duration. For smaller maximum oxygen consumption, the independent determinants were smaller right ventricular ejection fraction and longer QRS duration.
  
  
  CONCLUSIONS
  At mid-to-late follow-up, clinical condition in tetralogy of Fallot corrected according to contemporary surgical approaches appears well preserved. However, even these patients show right ventricular dilation and dysfunction associated with impaired functional capacity. Abnormalities relate to right ventricular outflow tract motion abnormalities, longer interval since repair, longer QRS duration, and more severe pulmonary regurgitation.
authors.affiliation: Department of Paediatric Cardiology, Erasmus MC, Rotterdam, The Netherlands.
authors.forename: Jochem
authors.initials: J
authors.lastname: van den Berg
authors.forename: Wim C
authors.initials: WC
authors.lastname: Hop
authors.forename: Jan L M
authors.initials: JL
authors.lastname: Strengers
authors.forename: Johan C
authors.initials: JC
authors.lastname: de Jongste
authors.forename: Lennie
authors.initials: L
authors.lastname: van Osch-Gevers
authors.forename: Folkert J
authors.initials: FJ
authors.lastname: Meijboom
authors.forename: Peter M T
authors.initials: PM
authors.lastname: Pattynama
authors.forename: Ad J J C
authors.initials: AJ
authors.lastname: Bogers
authors.forename: Willem A
authors.initials: WA
authors.lastname: Helbing
mesh_terms: D000293:Adolescent; D000328:Adult; D000704:Analysis of Variance; D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D004562:Electrocardiography / Q000379:methods; D015716:Electrocardiography, Ambulatory; D016552:Ergometry; D017079:Exercise Tolerance; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D016014:Linear Models; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D015999:Multivariate Analysis; D010101:Oxygen Consumption / Q000502:physiology*; D011336:Probability; D013147:Spirometry / Q000379:methods; D013318:Stroke Volume; D013771:Tetralogy of Fallot / Q000175:diagnosis* / Q000601:surgery*; D013997:Time Factors; D016896:Treatment Outcome