Intellectual, neuropsychological, and behavioral functioning in children with tetralogy of Fallot


      Although it is known that pediatric cardiac surgery holds risks for later development, few studies investigated the long-term development in children with tetralogy of Fallot. The purpose of this study was to define their intellectual capacities, neuropsychological profile, and behavioral functioning 6 to 12 years postoperatively.


      Patients (n = 18; age, 8 years, 3 months ± 1 year, 6 months) were examined with a short-form intelligence scale (Wechsler Intelligence Scale for Children, 3rd edition, Dutch version) and a neuropsychological assessment battery (NEPSY). Their parents completed a behavioral questionnaire. The patient group was compared with an acyanotic congenital heart disease group and a healthy control group.


      No significant differences between the patient group and the acyanotic group emerged. Compared with the healthy control group, children with tetralogy of Fallot showed significantly lower scores on the estimated Full Scale IQ (P < .05) and on the NEPSY domains Language (P < .01) and Sensorimotor Functioning (P < .01). Also, the subtests Tower (P < .05), Memory for Names (P < .05), Narrative Memory (P < .05), and Design Copy (P < .05) elicited group differences. Parental reports revealed significantly higher scores on attention problems (P < .05) and the total problem scale (P < .05), as well as significantly lower school performances than those of healthy peers (P < .01).


      In children with tetralogy of Fallot, we identified a lower estimated full-scale intelligence than in healthy peers and a neuropsychological profile characterized by primarily mild motor deficits and difficulties with language tasks. Parents of the children with tetralogy of Fallot indicated attention problems and rated the child’s school competencies to be lower than in healthy control subjects.

      CTSNet classification

      Abbreviations and Acronyms:

      CBCL (Child Behavior Checklist), CHD (congenital heart disease), NEPSY (developmental neuropsychological assessment), SD (standard deviation), TGA (transposition of the great arteries), TOF (tetralogy of Fallot), VSD (ventricular septal defect)
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic and Personal
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to The Journal of Thoracic and Cardiovascular Surgery
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Wright M.
        • Nolan T.
        Impact of cyanotic heart disease on school performance.
        Arch Dis Child. 1994; 71: 64-70
        • Wray J.
        • Sensky T.
        How does the intervention of cardiac surgery affect the self-perception of children with congenital heart disease?.
        Child Care Health Dev. 1998; 24: 57-72
        • Wray J.
        • Sensky T.
        Congenital heart disease and cardiac surgery in childhood: effects on cognitive function and academic ability.
        Heart. 2001; 85: 687-691
        • Oates R.
        • Simpson J.
        • Cartmill T.
        • Turnbull J.
        Intellectual function and age of repair in cyanotic congenital heart disease.
        Arch Dis Child. 1995; 72: 298-301
        • Haneda K.
        • Itoh T.
        • Togo T.
        • Ohmi M.
        • Mohri H.
        Effects of cardiac surgery on intellectual function in infants and children.
        Cardiovasc Surg. 1996; 4: 303-307
        • Hövels-Gürich H.
        • Konrad K.
        • Skorzenski D.
        • Nacken C.
        • Minkenberg R.
        • Phys D.
        • et al.
        Long-term neurodevelopmental outcome and exercise capacity after corrective surgery for tetralogy of Fallot or ventricular septal defect in infancy.
        Ann Thorac Surg. 2006; 81: 958-967
        • Daliento L.
        • Mapelli D.
        • Russo G.
        • Scarso P.
        • Limongi F.
        • Iannizzi P.
        • et al.
        Health related quality of life in adults with repaired tetralogy of Fallot: psychosocial and cognitive outcomes.
        Heart. 2005; 91: 213-218
        • Utens E.
        • Verhulst F.C.
        • Meijboom F.J.
        • Duivenvoorden H.J.
        • Erdman R.A.
        • Bos E.
        • et al.
        Behavioural and emotional problems in children and adolescents with congenital heart disease.
        Psychol Med. 1993; 23: 415-424
        • Oates R.K.
        • Turnbull J.A.
        • Simpson J.M.
        • Cartmill T.B.
        Parent and teacher perceptions of child behaviour following cardiac surgery.
        Acta Paediatr. 1994; 83: 1303-1307
        • Bellinger D.C.
        • Rappaport L.A.
        • Wypij D.
        • Wernovsky G.
        • Newburger J.W.
        Patterns of developmental dysfunction after surgery during infancy to correct transposition of the great arteries.
        J Dev Behav Pediatr. 1997; 18: 75-83
        • World Medical Association Declaration of Helsinki
        Recommendations guiding physicians in biomedical research involving human subjects.
        JAMA. 1997; 277: 925-926
        • Grégoire J.
        Comparison of three short forms of the Wechsler Intelligence Scale for children–third edition (WISC-III).
        Eur Rev Appl Psychol. 2000; 50: 437-441
        • Sattler J.
        Assessment of children. 3rd ed. Author, San Diego, Calif1992
        • Korkman M.
        • Kirk U.
        • Kemp S.
        NEPSY. The Psychological Corporation, San Antonio, Texas1998 (A Harcourt Assessment Company)
        • Achenbach T.M.
        Manual for the child behaviour checklist/4-18 and 1991 profile. University of Vermont, Burlington, Vt1991
        • Verhulst F.C.
        • van der Ende J.
        • Koot H.M.
        Handleiding voor de CBCL/4-18 [Manual of the CBCL/4-18]. Universiteit Rotterdam, Rotterdam, The Netherlands1996
        • Hövels-Gürich H.H.
        • Seghaye M.C.
        • Schnitker R.
        • Wiesner M.
        • Huber W.
        • Minkenberg R.
        • et al.
        Long-term neurodevelopmental outcomes in school-aged children after neonatal arterial switch operation.
        J Thorac Cardiovasc Surg. 2002; 124: 448-458
        • Hemphill L.
        • Uccelli P.
        • Winner K.
        • Chang C.J.
        • Bellinger D.
        Narrative discourse in young children with histories of early corrective heart surgery.
        J Speech Lang Hear Res. 2002; 45: 318-331
        • Bellinger D.
        • Bernstein J.
        • Kirkwood M.
        • Rappaport L.
        • Newburger J.
        Visual-spatial skills in children after open-heart surgery.
        J Dev Behav Pediatr. 2003; 24: 169-179
        • O’Dougherty M.
        • Berntson G.
        • Boysen S.
        • Wright F.
        • Teske D.
        Psycho-physiological predictors of attentional dysfunction in children with congenital heart defects.
        Psychophysiology. 1988; 25: 305-315
        • Ternestedt B.M.
        • Wall K.
        • Oddsson H.
        • Riesenfeld T.
        • Groth I.
        • Schollin J.
        Quality of life 20 and 30 years after surgery in patients operated on for Tetralogy of Fallot and for atrial septal defect.
        Pediatr Cardiol. 2001; 22: 128-132
        • Nolan T.
        • Pless B.
        Emotional correlates and consequences of birth defects.
        J Pediatr. 1986; 109: 201-216
        • Kramer H.H.
        • Trampisch H.J.
        • Rammos S.
        • Giese A.
        Birthweight of children with congenital heart disease.
        Eur J Paediatr. 1990; 149: 752-757
        • Böhm B.
        • Katz-Salamon M.
        • Smedler A.C.
        • Lagercrantz H.
        • Forssberg H.
        Developmental risks and protective factors influencing cognitive outcome at 5½ years of age in very-low-birthweight children.
        Dev Med Child Neurol. 2002; 44: 508-516