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Neurocognitive Consequences of Surgically Corrected Congenital Heart Defects: A Review

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Abstract

With advances in surgical procedures, neuropsychological assessment after congenital heart defects and pre, peri- and/or postoperative predictors of adverse outcome has become an important focus in research. We aim to summarize neuropsychological sequelae associated with different types of congenital heart defects, critically review the methodology used in more than 20 empirical studies that were retrieved from biomedical electronic search engines, and identify possible directions for future research. Despite the lack of adequate control groups and long-term studies, there seem to be some cognitive deficits. The largest group of children with isolated congenital heart defects present with normal intellectual capacities. However, they tend to show language deficits and motor dysfunction. Although performances on memory tasks are good, unambiguous conclusions concerning their attentional and executive functioning are still lacking. Serious behavioral problems are not an issue. In addition to a detailed description of the (neuro) psychological consequences of pediatric cardiac surgery, an overview of the predictors of the cognitive defects is provided.

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References

  • Aldèn, B., Gilljam, T., & Gillberg, C. (1998). Long term psychological outcome of children after surgery for transposition of the great arteries. Acta Paediatrica, 87, 405–410.

    Article  PubMed  Google Scholar 

  • Bellinger, D. C., Rappaport, L. A., Wypij, D., Wernovsky, G., & Newburger, J. W. (1997). Patterns of developmental dysfunction after surgery during infancy to correct transposition of the great arteries. Journal of Developmental and Behavioral Pediatrics, 18(2), 75–83.

    Article  PubMed  CAS  Google Scholar 

  • Bellinger, D. C., Wernovsky, G., Rappaport, L. A., Mayer, J. E., Castaneda, A. R., Farrell, D. M., Wessel, D. L., Lang, P., Hickey, P. R., Jonas, R. A., & Newburger, J. W. (1991). Cognitive development of children following early repair of transposition of the great arteries using deep hypothermic circulatory arrest. Pediatrics, 87(5), 701–707.

    PubMed  CAS  Google Scholar 

  • Bellinger, D. C., Wypij, D., du Plessis, A. J., Rappaport, L. A., Riviello, J., Jonas, R. J., & Newburger, J. W. (2001). Developmental and neurologic effects of alpha-stat versus pH-stat strategies for deep hypothermic cardiopulmonary bypass in infants. Journal of Thoracic and Cardiovascular Surgery, 121(2), 374–383.

    Google Scholar 

  • Bellinger, D. C., Wypij, D., Kuban, K. C. K., Rappaport, L. A., Hickey, P. R., Wernovsky, G., Jonas, R. A., & Newburger, J. W. (1999). Developmental and neurological status of children at 4 years of age after heart surgery with hypothermic circulatory arrest or low-flow cardiopulmonary bypass. Circulation, 100(5), 526–532.

    PubMed  CAS  Google Scholar 

  • Bruns, L. A., Chrisant, M. K., Lamour, J. M., Shaddy, R. E., Pahl, E., Blume, E. D., Hallowell, S., Addonizio, L. J., & Canter, C. E. (2001). Carvedilol as therapy in pediatric heart failure: An initial multicenter experience. Journal of Pediatrics, 138(4), 505–511.

    Article  PubMed  CAS  Google Scholar 

  • Colemann, K. (2002). Genetic counseling in congenital heart disease. Critical Care Nursing Quarterly, 25(3), 8–16.

    Google Scholar 

  • Connolly, D., Rutkowski, M., Auslender, M., & Artman, M. (2001). The New York University Pediatric Heart Failure Index: A new method of quantifying chronic heart failure severity in children. Journal of Pediatrics, 138(5), 644–648.

    Article  PubMed  CAS  Google Scholar 

  • Dickinson, D. F., & Sambrooks, J. E. (1979). Intellectual performance in children after circulatory arrest with profound hypothermia in infancy. Archives of Disease in Childhood, 54, 1–6.

    PubMed  CAS  Google Scholar 

  • Dittrich, H., Bührer, C., Grimmer, I., Dittrich, S., Abdul-Khaliq, H., & Lange, P. E. (2003). Neurodevelopment at 1 year of age in infants with congenital heart disease. Heart, 89(4), 436–441.

    Article  PubMed  CAS  Google Scholar 

  • Ekroth, R., Thompson, R. J., Lincoln, C., Scallan, M., Rossi, R., & Tsang, V. (1989). Elective deep hypothermia with total circulatory arrest: Changes in plasma creatine kinease BB, blood glucose, and clinical variables. Journal of Thoracic and Cardiovascular Surgery, 97, 30–35.

    PubMed  CAS  Google Scholar 

  • Feldt, R. H., Ewart, J. C., Stickler, G. B., & Weidman, W. H. (1969). Children with congenital heart disease. American Journal of Diseases in Children, 117, 281–287.

    CAS  Google Scholar 

  • Forbess, J. M., Visconti, K. J., Bellinger, D. C., Howe, R. J., & Jonas, R. A. (2002). Neurodevelopmental outcomes after biventricular repair of congenital heart defects. Journal of Thoracic and Cardiovascular Surgery, 123(4), 631–639.

    Article  PubMed  Google Scholar 

  • Forbess, J. M., Visconti, K. J., Hancock-Friesen, C., Howe, R. C., Bellinger, D. C., & Jonas, R. A. (2002). Neurodevelopmental outcome after congenital heart surgery: Results from an institutional registry. Circulation, 106(13), I95–I102.

    PubMed  Google Scholar 

  • Gaynor, J. W., Gerdes, M., Zackai, E. H., Bernbaum, J., Wernovsky, G., Clancy, R. R., Newman, M. F., Saunders, A. M., Heagerty, P. J., D’Agostino, J. A., McDonald-McGinn, D., Nicolson, S. C., Spray, T. L., & Jarvik, G. P. (2003). Apolipoprotein E genotype and neurodevelopmental sequelae of infant cardiac surgery. Journal of Thoracic and Cardiovascular Surgery, 126, 1736–1745.

    Article  PubMed  CAS  Google Scholar 

  • Hamrick, S. E. G., Gremmels, D. B., Keet, C. A., Leonard, C. H., Connell, J. K., Hawgood, S., & Piecuch, R. E. (2003). Neurodevelopmental outcome of infants supported with extracorporeal membrane oxygenation after cardiac surgery. Pediatrics, 111(6), E671–E675.

    Article  PubMed  Google Scholar 

  • Haneda, K., Itoh, T., Togo, T., Ohmi, M., & Mohri, H (1996). Effects of cardiac surgery on intellectual function in infants and children. Cardiovascular Surgery, 4(3), 303–307.

    Article  PubMed  CAS  Google Scholar 

  • Hoffman, J. I., & Kaplan, S. (2002). The incidence of congenital heart disease. Journal of American College of Cardiology, 39,1890–1900.

    Article  Google Scholar 

  • Hövels-Gürich, H. H., Seghaye, M. C., Däbritz, S., Messmer, B. J., & von Bernuth, G. (1997). Cognitive and motor development in preschool and school-aged children after neonatal arterial switch operation. Journal of Thoracic and Cardiovascular Surgery, 114(4), 578–585.

    Google Scholar 

  • Hövels-Gürich, H. H., Seghaye, M. C., Schnitker, R., Wiesner, M., Huber, W., Minkenberg, R., Kotlarek, F., Messmer, B. J., & von Bernuth, G. (2002). Long-term neurodevelopmental outcomes in school-aged children after neonatal arterial switch operation. Journal of Thoracic and Cardiovascular Surgery, 124(3), 448–458.

    Google Scholar 

  • Hövels-Gürich, H. H., Seghaye, M. C., Sigler, M., Kotlarek, F., Bartl, A., Neuser, J., Minkenberg, R., Messmer, B. J., & von Bernuth, G. (2001). Neurodevelopmental outcome related to cerebral risk factors in children after neonatal arterial switch operation. Annals of Thoracic Surgery, 71(3), 881–888.

    Article  PubMed  Google Scholar 

  • Jonas, R. A., Wypij, D., Roth, S. J., Bellinger, D. C., Visconti, K. J., du Plessis, A. J. Goodkin, H., Laussen, P. C., Farrell, D. M., Bartlett, J., McGrath, E., Rappaport, L. J., Bacha, E. A., Forbess, J. M., & del Nido, P. (2003). The influence of hemodilution on outcome after hypothermic cardiopulmonary bypass: results of a randomized trial in infants. Journal of Thoracic and Cardiovascular Surgery, 126, 1764–1774.

    Google Scholar 

  • Kern, J. H., Hinton, V. J., Nereo, N. E., Hayes, C. J., & Gersony, W. M. (1998) Early developmental outcome after the Norwood procedure for hypoplastic left heart syndrome. Pediatrics, 102(5), 1148–1152.

    Google Scholar 

  • Kirkham, F. J. (1998). Recognition and prevention of neurological complications in pediatric cardiac surgery. Pediatric Cardiology, 19, 331–345.

    Article  PubMed  CAS  Google Scholar 

  • Lewis, F. J., & Taufic, M. (1953). Closure of atrial septal defects with the aid of hypothermia: Experimental accomplishments and the report of one successful case. Surgery, 32, 52–59.

    Google Scholar 

  • Limperopoulos, C., Majnemer, A., Shevell, M. I., Rohlicek, C., Rosenblatt, B., Tchervenkov, C., & Darwish, H. Z. (2002). Predictors of developmental disabilities after open-heart surgery in young children with congenital heart defects. Journal of Pediatrics, 141(5), 51–58.

    PubMed  Google Scholar 

  • Limperopoulos, C., Majnemer, A., Shevell, M. I., Rosenblatt, B., Rohlicek, C., & Tchervenkov, C. (1999). Neurologic status of newborns with congenital heart defects before open-heart surgery. Pediatrics, 103(2), 402–408.

    Article  PubMed  CAS  Google Scholar 

  • Linde, L. M., Rasof, B., & Dunn, O. (1967). Mental development in congenital heart disease. Journal of Pediatrics, 71, 198–203.

    Article  PubMed  CAS  Google Scholar 

  • Mahle, W. T., Clancy, R. R., Moss, E. M., Gerdes, M., Jobes, D. R., & Wernovsky, G. (2000). Neurodevelopmental outcome and lifestyle assessment in school-aged and adolescent children with hypoplastic left heart syndrome. Pediatrics, 105(5), 1082–1089.

    Article  PubMed  CAS  Google Scholar 

  • Mahle, W. T., Spray, T. L., Wernovsky, G., Gaynor, J. W., & Clark, B. J. (2000). Survival after reconstructive surgery for hypoplastic left heart syndrome: a 15-year experience from a single institution. Circulation, 102, III136—III141.

    Google Scholar 

  • Mahle, W. T., Tavani, F., Zimmerman, R. A., Nicolson, S. C., Galli, K. K., Gaynor, W., Clancy, R. R., Montenegro, L. M., Spray, T. L., Chiavacci, R. M., Wernovsky, G., & Kurth, C. D. (2002). An MRI study of neurological injury before and after congenital heart surgery. Circulation, 106(suppl I), I109–I114.

    Google Scholar 

  • May, L. E. (2001). Pediatric Heart Surgery: A ready reference for professionals. 2nd edn. Milwaukee, Wis: Maxishare.

    Google Scholar 

  • McConnell, J. R., Fleming, W. H., Chu, W. K., Hahn, F. J., Sarafan, L. B., Hofschire, P. J., & Kugler, J. D. (1990). Magnetic resonance imaging of the brain in infants and children before and after cardiac surgery. A prospective study. American Journal of Diseases in Children, 144(3), 374–378.

    Google Scholar 

  • Miller, G., & Vogel, H. (1999). Structural evidence of injury or malformations in the brains of children with congenital heart disease. Seminars in Pediatric Neurology, 6, 20–26.

    Article  PubMed  CAS  Google Scholar 

  • Miller, G., Tesman, J. R., Ramer, J. C., Baylen, B. G., & Myers, J. L. (1996). Outcome after open-heart surgery in infants and children. Journal of Child Neurology, 11, 49–53.

    PubMed  CAS  Google Scholar 

  • Newburger, J. W., Jonas, R. A., Wernovsky, G., Wypij, D., Hickey, P. R., Kuban, K. C. K., Farrell, D. M., Holmes, G. L., Helmers, S. L., Constantinou, J., Carrazana, E., Barlow, J. K., Walsh, A. Z., Lucius, K. C., Share, J. C., Wessel, D. L., Hanley, F. L., Mayer, J. E., Castaneda, A. R., & Ware, J. H. (1993). A comparison of the perioperative neurologic effects of hypothermic circulatory arrest versus low-flow cardiopulmonary bypass in infant heart-surgery. New England Journal of Medicine, 329(15), 1057–1064.

    Article  PubMed  CAS  Google Scholar 

  • Newburger, J. W., Wypij, D., Bellinger, D. C., du Plessis, A. J., Kuban, K. C. K., Rappaport, L. A., Almirall, D., Wessel, D. L., Jonas, R. A., & Wernovsky, G. (2003). Length of stay after infant heart surgery is related to cognitive outcome at age 8 years. Journal of Pediatrics, 143, 67–73.

    Article  PubMed  Google Scholar 

  • Nuutinen, M., Koivu, M., & Rantakallio, P. (1989). Long-term outcome for children with congenital heart defects. Arctic Medical Research, 48, 175–184.

    PubMed  CAS  Google Scholar 

  • O’Dougherty, M., Wright, F. S., Garmezy, N., Lowenson, R. B., & Torres, F. (1983). Later competence and adaptation in infants who survive severe heart defects. Child Development, 54, 1129–1142.

    Article  PubMed  CAS  Google Scholar 

  • O’Dougherty, M., Wright, F. S., Loewenson, R. B., & Torres, F. (1985). Cerebral dysfunction after chronic hypoxia in children. Neurology, 35, 42–46.

    PubMed  CAS  Google Scholar 

  • Oates, R. K., Simpson, J. M., Cartmill, T. B., & Turnbull, J. A. B. (1995a). Intellectual function and age of repair in cyanotic congenital heart disease. Archives of Disease in Childhood, 72(4), 298–301.

    PubMed  CAS  Google Scholar 

  • Oates, R. K., Simpson, J. M., Turnbull, J. A. B., & Cartmill, T. B. (1995 b) The relationship between intelligence and duration of circulatory arrest with deep hypothermia. Journal of Thoracic and Cardiovascular Surgery, 110(3), 786–792.

    Article  PubMed  CAS  Google Scholar 

  • Rappaport, L. A., Wypij, D., Bellinger, D. C., Helmers, S. L., Holmes, G. L., Barnes, P. D., Wernovsky, G., Kuban, K. C. K., Jonas, R. A. & Newburger, J. W. (1998). Relation of seizures after cardiac surgery in early infancy to neurodevelopmental outcome. Circulation, 97, 773–779.

    PubMed  CAS  Google Scholar 

  • Rasof, B., Linde, L. M., & Dunn, O. (1967). Intellectual development in children with congenital heart disease. Child Development, 38, 1043–1053.

    Article  PubMed  CAS  Google Scholar 

  • Rogers, B. T., Msall, M. E., Buck, G. M., Lyon, N. R., Norris, M. K., Roland, J. M. A., Gingell, R. L., Cleveland, D. C., & Pieroni, D. R. (1995). Neurodevelopmental outcome of infants with hypoplastic left heart syndrome. Journal of Pediatrics, 126(3), 496–498.

    Article  PubMed  CAS  Google Scholar 

  • Rosenthal, A. (1967). Visual simple reaction time in cyanotic heart disease. American Journal of Diseases in Children, 114, 139–143.

    CAS  Google Scholar 

  • Rossi, R., Ekroth, R., Lincoln, C., Jackson, A. P., Thompson, R. J., Scallan, M., & Tsang, V. (1986). Detection of cerebral injury after total circulatory arrest and profound hypothermia by estimation of specific creatine kinase isoenzyme levels using monoclonal antibody techniques. American Journal of Cardiology, 58(13), 1236–1241.

    Article  PubMed  CAS  Google Scholar 

  • Rossi, R., vander Linden, J., Ekroth, R., Scallan, M., Thompson, R. J., & Lincoln, C. (1989). No flow or flow: a study of ischemic marker creatine kinase BB after deep hypothermic procedures. Journal of Thoracic and Cardiovascular Surgery, 98, 193–199.

    PubMed  CAS  Google Scholar 

  • Scallan, M. J. H. (2003). Brain injury in children with congenital heart disease. Paediatric Anesthesia, 13, 284–293.

    Article  Google Scholar 

  • Sharma, R., Choudhary, S. K., Mohan, M. R., Padma, M. V., Jain, S., Bhardwaj, M., Bhan, A., Kiran, U., Saxena, N., & Venugopal, P. (2000). Neurological evaluation and intelligence testing in the child with operated congenital heart disease. Annals of Thoracic Surgery, 70, 575–581.

    Article  PubMed  CAS  Google Scholar 

  • Silbert, A., Wolff, P. H., Mayer, A., Rosenthal, A., & Nada, A. S. (1969). Cyanotic heart disease and psychological development. Pediatrics, 43(2), 192–200.

    PubMed  CAS  Google Scholar 

  • Treasure, T., Naftel, D. C., Conger, K. A., Garcia, J. H., Kirklin, J. W., & Blackstone, E. H. (1983). The effect of hypothermic circulatory arrest time on cerebral function, morphology and biochemistry. Journal of Thoracic and Cardiovascular Surgery, 86, 761–770.

    PubMed  CAS  Google Scholar 

  • Uzark, K., Lincoln, A., Lamberti, J. J., Mainwaring, R. D., Spicer, R. L., & Moore, J. W. (1998). Neurodevelopmental outcomes in children with Fontan repair of functional single ventricle. Pediatrics, 101(4), 630–633.

    Article  PubMed  CAS  Google Scholar 

  • Van Hoecke, E., & Dhont, M. (2004). Aangeboren hartaandoening: verwerking door het kind en zijn ouders [Congenital heart disease: coping mechanisms of the child and its parents]. Tijdschrift voor Geneeskunde, 60(21),1548–1554.

    Article  Google Scholar 

  • Visconti, K. J., Bichell, D. P., Jonas, R. A., Newburger, J. W., & Bellinger, D. C. (1999) Developmental outcome after surgical versus interventional closure of secundum atrial septal defect in children. Circulation, 100(19), 145–150.

    Google Scholar 

  • Wells, F. C., Coghill, S., Caplan, H. L., Lincoln, C., & Kirklin, J. W. (1983). Duration of cardiopulmonary arrest does influence the psychological development of children after cardiac operation in early life. Journal of Thoracic and Cardiovascular Surgery, 86, 823–831.

    PubMed  CAS  Google Scholar 

  • Wernovsky, G., Stiles, K. M., Gauvreau, K., Gentles, T. L., duPlessis, A. J., Bellinger, D. C., Walsh, A. Z., Burnett, J., Jonas, R. A., Mayer, J. E., & Newburger, J. W. (2000) Cognitive development after the Fontan operation. Circulation, 102(8), 883–889.

    PubMed  CAS  Google Scholar 

  • Wray, J., & Sensky, T. (1999). Controlled study of preschool development after surgery for congenital heart disease. Archives of Disease in Childhood, 80, 511–516.

    PubMed  CAS  Google Scholar 

  • Wray, J., & Sensky, T. (2001). Congenital heart disease and cardiac surgery in childhood: effects on cognitive function and academic ability. Heart, 85(6), 687–691.

    Article  PubMed  CAS  Google Scholar 

  • Wright, M., & Nolan, T. (1994). Impact of cyanotic heart disease on school performance. Archives of Disease in Childhood, 71, 64–70.

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Laboratory for Neuropsychology, Department of Internal Medicine, Ghent University, De Pintelaan 185, 4 K 3, B-9000, Ghent, Belgium

    M. Miatton & G. Vingerhoets

  2. Pediatric Cardiology, Department of Pediatrics, Ghent University, Ghent, Belgium

    D. De Wolf

  3. Pediatric Cardiac Surgery, Department of Pediatrics, Ghent University, Ghent, Belgium

    K. François

  4. Reference Center for Refractory Epilepsy, Ghent University, Ghent, Belgium

    E. Thiery

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  1. M. Miatton
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  2. D. De Wolf
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  3. K. François
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  4. E. Thiery
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Correspondence to M. Miatton.

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Miatton, M., De Wolf, D., François, K. et al. Neurocognitive Consequences of Surgically Corrected Congenital Heart Defects: A Review. Neuropsychol Rev 16, 65–85 (2006). https://doi.org/10.1007/s11065-006-9005-7

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