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Health and well-being of children with congenital cardiac malformations, and their families, following open-heart surgery

  • Annette Majnemer (a1), Catherine Limperopoulos (a1), Michael Shevell (a1), Charles Rohlicek (a1), Bernard Rosenblatt (a1) and Christo Tchervenkov (a1)...

Infants who survive open-heart surgery are at risk for developmental disability, which may impact on the well-being not only of the child, but also the family. The objective of our prospective study, therefore, was to determine the long-term health-related quality of life of children with congenital cardiac malformations following open-heart surgery, and to describe the persisting level of stress in their families. To this end, 49 parents completed the Child Health Questionnaire, the Parenting Stress Index, and the Child Behaviour Checklist as part of a developmental follow-up protocol when their child was 5 years of age. Mean scores on the Child Health Questionnaire were in the normal range, with physical well-being equal to 53.5, psychosocial well-being 50.9, with only 6.4 percent and 8.5 percent of subjects, respectively, falling within the suboptimal range of less than 40. The distribution of scores on the Parenting Stress Index, however, were more variable, with over one-quarter of parents indicating a high level of stress, with almost one-fifth having low levels of stress, and just over half scoring in the normal range, with the group mean being 52.6 plus or minus 32.3. An abnormal neurologic examination before surgery was associated with lower physical health (β equal to −5.5, p equal to 0.02, r2 equal to 0.18), whereas lower arterial saturations of oxygen, less than 85 percent preoperatively, was associated with lower psychosocial health (β equal to −6.6, p equal to 0.01, and r2 equal to 0.14). The internalizing and externalizing behaviours of the child were significantly correlated with psychosocial well being, with r ranging from −0.32 to −0.52, and p less than 0.05. Parental stress also correlated with psychosocial health (r equal to −0.48 and p equal to 0.0009). Overall, the perception by the parents of the health-related quality of life of their child is favourable 5 years following open-heart surgery during infancy. Many parents, nonetheless, continue to feel either stressed or defensive about their child, particularly if their child exhibits behavioural difficulties. Our findings suggest that strategies need to be considered to enhance family well-being in the planning and delivery of health services to this population at high risk.

Corresponding author
Correspondence to: Dr Annette Majnemer, Montreal Children's Hospital, Division of Pediatric Neurology, 2300 Tupper Street, Room A-509, Montreal, Quebec H3H 1P3, Canada. Tel: +1 514 412 4400, ext. 22902; Fax: +1 514 412 4373; E-mail:
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Olley PM. Congenital heart disease; Six decades of progress. Semin Pediatr Neurol 1999; 6: 311.
Majnemer A, Limperopoulos C. Developmental progress of children with congenital heart defects. Semin Pediatr Neurol 1999; 6: 1219.
Moons P, De Bleser L, Budts W, et al. Health status, functional abilities, and quality of life after the Mustard or Senning operation. Ann Thorac Surg 2004; 77: 13591365.
Ternestedt BM, 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: 128132.
Lane DA, Lip GYH, Millane TA. Quality of life in adults with congenital heart disease. Heart 2002; 88: 7175.
Simko LC, McGinnis KA. Quality of life experienced by adults with congenital heart disease. AACN Clinical Issues 2003; 14: 4253.
McMurray R, Kendall L, Parsons JM, et al. A life less ordinary: Growing up and coping with congenital heart disease. Coronary Health Care 2001; 5: 5157.
Tong EM, Sparacino PSA, Messias DKH, Foote D, Chesla CA, Gilliss CL. Growing up with congenital heart disease: The dilemmas of adolescents and young adults. Cardiol Young, 1998; 8: 303309.
Dunbar-Masterson C, Wypij D, Bellinger DC, et al. General health status of children with D-Transposition of the Great Arteries after the arterial switch operation. Circulation 2001; 104: I-138I-142.
Moyen Laane K, Meberg A, Otterstad JE, et al. Quality of life in children with congenital heart defects. Acta Paediatr 1997; 86: 975980.
Wiegner S, Donders J. Predictors of parental distress after congenital disabilities. J Dev Behav Pediatr 2000; 21: 271277.
De Maso DR, Campis LK, Wypij D, Bertram S, Lipshitz M, Freed M. The impact of maternal perceptions and medical severity on the adjustment of children with congenital heart disease. J Pediatr Psychol 1991; 16: 137149.
Goldberg S, Janus M, Washington J, Simmons RJ, MacLusky I, Fowler RS. Prediction of preschool behavioral problems in healthy and pediatric samples. J Dev Behav Pediatr 1997; 18: 304313.
Limperopoulos C, Majnemer A, Shevell MI, Rosenblatt B, Rohlicek C, Tchervenkov C. Neurologic status of newborns with congenital heart defects prior to open heart surgery. Pediatrics 1999; 103: 403409.
Limperopoulos C, Majnemer A, Shevell MI, Rosenblatt B, Rohlicek C, Tchervenkov C. Neurodevelopmental status of newborns and infants with congenital heart defects before and after open heart surgery. J Pediatr 2000; 137: 638645.
Limperopoulos C, Majnemer A, Shevell M, et al. Functional limitations in young children with congenital heart defects following cardiac surgery. Pediatrics 2001; 108: 13251331.
Limperopoulos C, Majnemer A, Shevell M, et al. Predictors of developmental disabilities after open heart surgery in young children with congenital heart defects. J Pediatr 2002; 141: 5158.
Majnemer A, Limperopoulos C, Shevell M, Rosenblatt B, Rohlicek C, Tchervenkov C. Long-term neuromotor outcome at school entry of infants with congenital heart defects requiring open heart surgery. J Pediatr 2006; 148: 7277.
Landgraf J, Abetz L, Wsre Jr JE. Child Health Questionnaire (CHQ): A user's manual. 1st edition. The Health Institute, New England Medical Center, Boston, MA, 1996.
Abidin RR. Parenting Stress Index (PSI). 3rd edition. Psychological Assessment Resources, Inc., Odessa, Florida, 1990.
Ostberg M, Hagekull B, Wettergren S. A measure of parental stress in others with small children: Dimensionality, stability and validity. Scand J Psychol 1997; 38: 199208.
Eiser C, Morse R. A review of measures of quality of life for children with chronic illness. Arch Dis Child 2001; 84: 205211.
Hlatky M. Should we measure health-related quality of life? Am Heart J 2000; 140: 359360.
Majnemer A, Limperopoulos C. The importance of outcome determination in pediatric rehabilitation (annotation). Dev Med Child Neurol 2002; 44: 773777.
Uzark K, Jones K, Burwinkle TM, Varni JW. The Pediatric Quality of Life Inventory in children with heart disease. Progress in Pediatric Cardiology 2003; 18: 141148.
Albrecht GL, Devlieger PJ. The disability paradox: high quality of life against all odds. Soc Sci Med 1999; 48: 977988.
Fuhrer MJ. Subjectifying quality of life as a medical rehabilitation outcome: Review. Disabil Rehabil 2000; 22: 481489.
Mattera JA, Mendes de Leon C, Wackers FJT, Williams CS, Wang Y, Krumholz HM. Association of patients' perception of health status and exercise electrocardiogram, myocardial perfusion imaging, and ventricular function measures. Am Heart J 2000; 140: 409418.
Lawoko S, Soares JJF. Quality of life among parents of children with congenital heart disease, parents of children with other diseases and parents of healthy children. Qual Life Res 2003; 12: 655666.
Rosenbaum PL, Saigal S. Measuring health-related quality of life in pediatric populations. Conceptual issues. In: Spilker B (ed.), Quality of Life and Pharmacoeconomics in Clinical trials, 2nd edition, Lippincott-Raven Publishers, Philadelphia, 1996, pp 785791.
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Cardiology in the Young
  • ISSN: 1047-9511
  • EISSN: 1467-1107
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