title: Unnatural history of tetralogy of Fallot: prospective follow-up of 40 years after surgical correction.
abstract: BACKGROUND
  Prospective data on long-term survival and clinical outcome beyond 30 years after surgical correction of tetralogy of Fallot are nonexistent.
  
  
  RESULTS
  This longitudinal cohort study consists of the 144 patients with tetralogy of Fallot who underwent surgical repair at <15 years of age between 1968 and 1980 in our center. They are investigated every 10 years. Cumulative survival (data available for 136 patients) was 72% after 40 years. Late mortality was due to heart failure and ventricular fibrillation. Seventy-two of 80 eligible survivors (90%) participated in the third in-hospital investigation, consisting of ECG, Holter, echocardiography, cardiopulmonary exercise testing, N-terminal pro-brain natriuretic peptide measurement, cardiac magnetic resonance (including dobutamine stress testing), and the Short Form-36 questionnaire. Median follow-up was 36 years (range, 31-43 years). Cumulative event-free survival was 25% after 40 years. Subjective health status was comparable to that in the normal Dutch population. Although systolic right and left ventricular function declined, peak exercise capacity remained stable. There was no progression of aortic root dilation. A previous shunt operation, low temperature during surgery, and early postoperative arrhythmias were found to predict late mortality (hazard ratio, 2.9, 1.1, and 2.5, respectively). An increase in QRS duration and a deterioration of exercise tolerance and ventricular dysfunction did not predict mortality. Insertion of a transannular patch was a predictor for late arrhythmias (hazard ratio, 4.0; 95% confidence interval, 1.2-13.4).
  
  
  CONCLUSIONS
  Although many patients needed a reoperation or developed arrhythmias, late mortality was low, and the clinical condition and subjective health status of most patients remained good. Previous shunt, low temperature during surgery, and early postoperative arrhythmias were found to predict late mortality.
authors.affiliation: From the Department of Cardiology (J.A.A.E.C., M.E.M., E.E.M.K., P.O., M.W., A.E.v.D.B., R.T.v.D., E.B., J.W.R.-H.), Department of Radiology (W.A.H., M.O.), Department of Biostatistics (D.R.), and Department of Cardiothoracic Surgery (A.J.J.C.B.), Erasmus Medical Center, Rotterdam, The Netherlands; Department of Child and Adolescent Psychiatry and Psychology (E.M.W.J.U.) and Department of Pediatrics (Division of Cardiology) (W.A.H.), Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands; and Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands (F.J.M.). j.cuypers@erasmusmc.nl.
authors.forename: Judith A A E
authors.initials: JA
authors.lastname: Cuypers
authors.affiliation: From the Department of Cardiology (J.A.A.E.C., M.E.M., E.E.M.K., P.O., M.W., A.E.v.D.B., R.T.v.D., E.B., J.W.R.-H.), Department of Radiology (W.A.H., M.O.), Department of Biostatistics (D.R.), and Department of Cardiothoracic Surgery (A.J.J.C.B.), Erasmus Medical Center, Rotterdam, The Netherlands; Department of Child and Adolescent Psychiatry and Psychology (E.M.W.J.U.) and Department of Pediatrics (Division of Cardiology) (W.A.H.), Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands; and Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands (F.J.M.).
authors.forename: Myrthe E
authors.initials: ME
authors.lastname: Menting
authors.affiliation: From the Department of Cardiology (J.A.A.E.C., M.E.M., E.E.M.K., P.O., M.W., A.E.v.D.B., R.T.v.D., E.B., J.W.R.-H.), Department of Radiology (W.A.H., M.O.), Department of Biostatistics (D.R.), and Department of Cardiothoracic Surgery (A.J.J.C.B.), Erasmus Medical Center, Rotterdam, The Netherlands; Department of Child and Adolescent Psychiatry and Psychology (E.M.W.J.U.) and Department of Pediatrics (Division of Cardiology) (W.A.H.), Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands; and Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands (F.J.M.).
authors.forename: Elisabeth E M
authors.initials: EE
authors.lastname: Konings
authors.affiliation: From the Department of Cardiology (J.A.A.E.C., M.E.M., E.E.M.K., P.O., M.W., A.E.v.D.B., R.T.v.D., E.B., J.W.R.-H.), Department of Radiology (W.A.H., M.O.), Department of Biostatistics (D.R.), and Department of Cardiothoracic Surgery (A.J.J.C.B.), Erasmus Medical Center, Rotterdam, The Netherlands; Department of Child and Adolescent Psychiatry and Psychology (E.M.W.J.U.) and Department of Pediatrics (Division of Cardiology) (W.A.H.), Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands; and Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands (F.J.M.).
authors.forename: Petra
authors.initials: P
authors.lastname: Opić
authors.affiliation: From the Department of Cardiology (J.A.A.E.C., M.E.M., E.E.M.K., P.O., M.W., A.E.v.D.B., R.T.v.D., E.B., J.W.R.-H.), Department of Radiology (W.A.H., M.O.), Department of Biostatistics (D.R.), and Department of Cardiothoracic Surgery (A.J.J.C.B.), Erasmus Medical Center, Rotterdam, The Netherlands; Department of Child and Adolescent Psychiatry and Psychology (E.M.W.J.U.) and Department of Pediatrics (Division of Cardiology) (W.A.H.), Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands; and Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands (F.J.M.).
authors.forename: Elisabeth M W J
authors.initials: EM
authors.lastname: Utens
authors.affiliation: From the Department of Cardiology (J.A.A.E.C., M.E.M., E.E.M.K., P.O., M.W., A.E.v.D.B., R.T.v.D., E.B., J.W.R.-H.), Department of Radiology (W.A.H., M.O.), Department of Biostatistics (D.R.), and Department of Cardiothoracic Surgery (A.J.J.C.B.), Erasmus Medical Center, Rotterdam, The Netherlands; Department of Child and Adolescent Psychiatry and Psychology (E.M.W.J.U.) and Department of Pediatrics (Division of Cardiology) (W.A.H.), Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands; and Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands (F.J.M.).
authors.forename: Willem A
authors.initials: WA
authors.lastname: Helbing
authors.affiliation: From the Department of Cardiology (J.A.A.E.C., M.E.M., E.E.M.K., P.O., M.W., A.E.v.D.B., R.T.v.D., E.B., J.W.R.-H.), Department of Radiology (W.A.H., M.O.), Department of Biostatistics (D.R.), and Department of Cardiothoracic Surgery (A.J.J.C.B.), Erasmus Medical Center, Rotterdam, The Netherlands; Department of Child and Adolescent Psychiatry and Psychology (E.M.W.J.U.) and Department of Pediatrics (Division of Cardiology) (W.A.H.), Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands; and Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands (F.J.M.).
authors.forename: Maarten
authors.initials: M
authors.lastname: Witsenburg
authors.affiliation: From the Department of Cardiology (J.A.A.E.C., M.E.M., E.E.M.K., P.O., M.W., A.E.v.D.B., R.T.v.D., E.B., J.W.R.-H.), Department of Radiology (W.A.H., M.O.), Department of Biostatistics (D.R.), and Department of Cardiothoracic Surgery (A.J.J.C.B.), Erasmus Medical Center, Rotterdam, The Netherlands; Department of Child and Adolescent Psychiatry and Psychology (E.M.W.J.U.) and Department of Pediatrics (Division of Cardiology) (W.A.H.), Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands; and Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands (F.J.M.).
authors.forename: Annemien E
authors.initials: AE
authors.lastname: van den Bosch
authors.affiliation: From the Department of Cardiology (J.A.A.E.C., M.E.M., E.E.M.K., P.O., M.W., A.E.v.D.B., R.T.v.D., E.B., J.W.R.-H.), Department of Radiology (W.A.H., M.O.), Department of Biostatistics (D.R.), and Department of Cardiothoracic Surgery (A.J.J.C.B.), Erasmus Medical Center, Rotterdam, The Netherlands; Department of Child and Adolescent Psychiatry and Psychology (E.M.W.J.U.) and Department of Pediatrics (Division of Cardiology) (W.A.H.), Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands; and Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands (F.J.M.).
authors.forename: Mohamed
authors.initials: M
authors.lastname: Ouhlous
authors.affiliation: From the Department of Cardiology (J.A.A.E.C., M.E.M., E.E.M.K., P.O., M.W., A.E.v.D.B., R.T.v.D., E.B., J.W.R.-H.), Department of Radiology (W.A.H., M.O.), Department of Biostatistics (D.R.), and Department of Cardiothoracic Surgery (A.J.J.C.B.), Erasmus Medical Center, Rotterdam, The Netherlands; Department of Child and Adolescent Psychiatry and Psychology (E.M.W.J.U.) and Department of Pediatrics (Division of Cardiology) (W.A.H.), Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands; and Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands (F.J.M.).
authors.forename: Ron T
authors.initials: RT
authors.lastname: van Domburg
authors.affiliation: From the Department of Cardiology (J.A.A.E.C., M.E.M., E.E.M.K., P.O., M.W., A.E.v.D.B., R.T.v.D., E.B., J.W.R.-H.), Department of Radiology (W.A.H., M.O.), Department of Biostatistics (D.R.), and Department of Cardiothoracic Surgery (A.J.J.C.B.), Erasmus Medical Center, Rotterdam, The Netherlands; Department of Child and Adolescent Psychiatry and Psychology (E.M.W.J.U.) and Department of Pediatrics (Division of Cardiology) (W.A.H.), Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands; and Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands (F.J.M.).
authors.forename: Dimitris
authors.initials: D
authors.lastname: Rizopoulos
authors.affiliation: From the Department of Cardiology (J.A.A.E.C., M.E.M., E.E.M.K., P.O., M.W., A.E.v.D.B., R.T.v.D., E.B., J.W.R.-H.), Department of Radiology (W.A.H., M.O.), Department of Biostatistics (D.R.), and Department of Cardiothoracic Surgery (A.J.J.C.B.), Erasmus Medical Center, Rotterdam, The Netherlands; Department of Child and Adolescent Psychiatry and Psychology (E.M.W.J.U.) and Department of Pediatrics (Division of Cardiology) (W.A.H.), Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands; and Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands (F.J.M.).
authors.forename: Folkert J
authors.initials: FJ
authors.lastname: Meijboom
authors.affiliation: From the Department of Cardiology (J.A.A.E.C., M.E.M., E.E.M.K., P.O., M.W., A.E.v.D.B., R.T.v.D., E.B., J.W.R.-H.), Department of Radiology (W.A.H., M.O.), Department of Biostatistics (D.R.), and Department of Cardiothoracic Surgery (A.J.J.C.B.), Erasmus Medical Center, Rotterdam, The Netherlands; Department of Child and Adolescent Psychiatry and Psychology (E.M.W.J.U.) and Department of Pediatrics (Division of Cardiology) (W.A.H.), Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands; and Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands (F.J.M.).
authors.forename: Eric
authors.initials: E
authors.lastname: Boersma
authors.affiliation: From the Department of Cardiology (J.A.A.E.C., M.E.M., E.E.M.K., P.O., M.W., A.E.v.D.B., R.T.v.D., E.B., J.W.R.-H.), Department of Radiology (W.A.H., M.O.), Department of Biostatistics (D.R.), and Department of Cardiothoracic Surgery (A.J.J.C.B.), Erasmus Medical Center, Rotterdam, The Netherlands; Department of Child and Adolescent Psychiatry and Psychology (E.M.W.J.U.) and Department of Pediatrics (Division of Cardiology) (W.A.H.), Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands; and Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands (F.J.M.).
authors.forename: Ad J J C
authors.initials: AJ
authors.lastname: Bogers
authors.affiliation: From the Department of Cardiology (J.A.A.E.C., M.E.M., E.E.M.K., P.O., M.W., A.E.v.D.B., R.T.v.D., E.B., J.W.R.-H.), Department of Radiology (W.A.H., M.O.), Department of Biostatistics (D.R.), and Department of Cardiothoracic Surgery (A.J.J.C.B.), Erasmus Medical Center, Rotterdam, The Netherlands; Department of Child and Adolescent Psychiatry and Psychology (E.M.W.J.U.) and Department of Pediatrics (Division of Cardiology) (W.A.H.), Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands; and Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands (F.J.M.).
authors.forename: Jolien W
authors.initials: JW
authors.lastname: Roos-Hesselink
mesh_terms: D000293:Adolescent; D000328:Adult; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D013771:Tetralogy of Fallot / Q000175:diagnosis* / Q000503:physiopathology / Q000601:surgery*; D013997:Time Factors; D055815:Young Adult
keywords: arrhythmias, cardiac; follow-up studies; morbidity; survival; tetralogy of Fallot