Neurodevelopmental status at eight years in children with dextro-transposition of the great arteries: the Boston Circulatory Arrest Trial.

OBJECTIVES Our goal was to determine which of the two major methods of vital organ support used in infant cardiac surgery, total circulatory arrest and low-flow cardiopulmonary bypass, results in better neurodevelopmental outcomes at school age. METHODS In a single-center trial, infants with dextrotransposition of the great arteries underwent the arterial switch operation after random assignment to either total circulatory arrest or low-flow cardiopulmonary bypass. Developmental, neurologic, and speech outcomes were assessed at 8 years of age in 155 of 160 eligible children (97%). RESULTS Treatment groups did not differ in terms of most outcomes, including neurologic status, Full-Scale or Performance IQ score, academic achievement, memory, problem solving, and visual-motor integration. Children assigned to total circulatory arrest performed worse on tests of motor function including manual dexterity with the nondominant hand (P =.003), apraxia of speech (P =.01), visual-motor tracking (P =.01), and phonologic awareness (P =.003). Assignment to low-flow cardiopulmonary bypass was associated with a more impulsive response style on a continuous performance test of vigilance (P <.01) and worse behavior as rated by teachers (P =.05). Although mean scores on most outcomes were within normal limits, neurodevelopmental status in the cohort as a whole was below expectation in many respects, including academic achievement, fine motor function, visual-spatial skills, working memory, hypothesis generating and testing, sustained attention, and higher-order language skills. CONCLUSIONS Use of total circulatory arrest to support vital organs during heart surgery in infancy is generally associated with greater functional deficits than is use of low-flow cardiopulmonary bypass, although both strategies are associated with increased risk of neurodevelopmental vulnerabilities.

[1]  Relation of pH strategy and developmental outcome after hypothermic circulatory arrest. , 1993 .

[2]  T. Nolan,et al.  Impact of cyanotic heart disease on school performance. , 1994, Archives of disease in childhood.

[3]  D. Wypij,et al.  Developmental and neurological status of children at 4 years of age after heart surgery with hypothermic circulatory arrest or low-flow cardiopulmonary bypass. , 1999, Circulation.

[4]  C. Reynolds,et al.  Critical Measurement Issues in Learning Disabilities , 1984 .

[5]  Joanne F. Carlisle,et al.  A study of handwriting in written stories of normal and learning disabled children , 1996 .

[6]  B P Rourke,et al.  Principal Identifying Features of the Syndrome of Nonverbal Learning Disabilities in Children , 1994, Journal of learning disabilities.

[7]  T. Miura,et al.  Higher hematocrit improves cerebral outcome after deep hypothermic circulatory arrest. , 1996, The Journal of thoracic and cardiovascular surgery.

[8]  B. McKenzie,et al.  Information processing deficits associated with developmental coordination disorder: a meta-analysis of research findings. , 1998, Journal of child psychology and psychiatry, and allied disciplines.

[9]  M. Klein,et al.  Neuropsychological Abilities of Children With Cardiac Disease Treated With Extracorporeal Membrane Oxygenation , 1999 .

[10]  K. Devriendt,et al.  Neuropsychological, learning and psychosocial profile of primary school aged children with the velo-cardio-facial syndrome (22q11 deletion): evidence for a nonverbal learning disability? , 1999, Child neuropsychology : a journal on normal and abnormal development in childhood and adolescence.

[11]  H. Mohri,et al.  Effects of cardiac surgery on intellectual in and children , 1996 .

[12]  G. Holmes,et al.  Relation of seizures after cardiac surgery in early infancy to neurodevelopmental outcome. Boston Circulatory Arrest Study Group. , 1998, Circulation.

[13]  D. Francis,et al.  Persistence of dyslexia: the Connecticut Longitudinal Study at adolescence. , 1999, Pediatrics.

[14]  K. Heilman,et al.  Developmental dyslexia: A motor‐articulatory feedback hypothesis , 1996, Annals of neurology.

[15]  K. Wolstencroft The Wide Range Assessment of Memory and Learning, W. Adams & D. Sheslow (1990). Wilmington , 1996, Australian Journal of Guidance and Counselling.

[16]  A. Aronson,et al.  Motor Speech Disorders , 2014 .

[17]  J. Fisher,et al.  Neuropsychological Assessment, 2nd Ed , 1985 .

[18]  B. Rosenblatt,et al.  Neurodevelopmental status of newborns and infants with congenital heart defects before and after open heart surgery. , 2000, The Journal of pediatrics.

[19]  J. Brenner,et al.  Neurodevelopmental Outcomes in Children Surviving d‐Transposition of the Great Arteries , 1998, Journal of developmental and behavioral pediatrics : JDBP.

[20]  J. Windsor The role of phonological opacity in reading achievement. , 2000, Journal of speech, language, and hearing research : JSLHR.

[21]  H. Duivenvoorden,et al.  Prediction of behavioural and emotional problems in children and adolescents with operated congenital heart disease. , 1998, European heart journal.

[22]  J. Watson,et al.  Neuropsychological and neuropathological sequelae of cerebral anoxia: A critical review , 2000, Journal of the International Neuropsychological Society.

[23]  J. Newburger,et al.  Brain Injury and Pediatric Cardiac Surgery , 1995 .

[24]  Mark F. Newman,et al.  Longitudinal assessment of neurocognitive function after coronary-artery bypass surgery. , 2001 .

[25]  D. Bellinger,et al.  Narrative discourse in young children with histories of early corrective heart surgery. , 2002, Journal of speech, language, and hearing research : JSLHR.

[26]  D. R. Smith,et al.  CHAPTER 6 – Wechsler Individual Achievement Test , 2001 .

[27]  R. Reitan,et al.  Clinical neuropsychology: Current status and applications. , 1974 .

[28]  J. Tesman,et al.  Outcome After Open-Heart Surgery in Infants and Children , 1996, Journal of child neurology.

[29]  L M Greenberg,et al.  Developmental normative data on the test of variables of attention (T.O.V.A.). , 1993, Journal of child psychology and psychiatry, and allied disciplines.

[30]  A. Majnemer,et al.  Developmental progress of children with congenital heart defects requiring open heart surgery. , 1999, Seminars in pediatric neurology.

[31]  Rohe N. Eshbaugh Illinois Test of Psycholinguistic Abilities. , 1971 .

[32]  G. von Bernuth,et al.  Cognitive and motor development in preschool and school-aged children after neonatal arterial switch operation. , 1997, The Journal of thoracic and cardiovascular surgery.

[33]  Gregory L. Holmes,et al.  Developmental and neurologic status of children after heart surgery with hypothermic circulatory arrest or low-flow cardiopulmonary bypass. , 1995, The New England journal of medicine.

[34]  P. Fallon,et al.  Incidence of neurological complications of surgery for congenital heart disease. , 1995, Archives of disease in childhood.

[35]  Later competence and adaptation in infants who survive severe heart defects. , 1983 .

[36]  T. Sensky,et al.  Controlled study of preschool development after surgery for congenital heart disease , 1999, Archives of disease in childhood.

[37]  Robert K. Heaton,et al.  Wisconsin Card Sorting Test Manual – Revised and Expanded , 1993 .

[38]  B. Rosenblatt,et al.  Neurologic status of newborns with congenital heart defects before open heart surgery. , 1999, Pediatrics.

[39]  S. Artan,et al.  22q11.2 deletions in a series of patients with non-selective congenital heart defects: incidence, type of defects and parental origin , 1998 .

[40]  T. Achenbach,et al.  Manual for the Teacher's Report Form and 1991 Profile , 1991 .

[41]  D. Wypij,et al.  Patterns of Developmental Dysfunction After Surgery During Infancy to Correct Transposition of the Great Arteries , 1997, Journal of developmental and behavioral pediatrics : JDBP.

[42]  D. Bellinger,et al.  Just pretend: Participation in symbolic talk by children with histories of early corrective heart surgery , 2000, Applied Psycholinguistics.

[43]  O. Spreen,et al.  A Compendium of Neuropsychological Tests: Administration, Norms, and Commentary , 1991 .

[44]  T. Klee,et al.  Clinical assessment of oropharyngeal motor development in young children. , 1987, The Journal of speech and hearing disorders.

[45]  A. Rothman,et al.  High Incidence of Cranial Ultrasound Abnormalities in Full-Term Infants with Congenital Heart Disease , 1996, American journal of perinatology.

[46]  J. Mayer,et al.  Cognitive development of children following early repair of transposition of the great arteries using deep hypothermic circulatory arrest. , 1991, Pediatrics.

[47]  J. Simpson,et al.  The relationship between intelligence and duration of circulatory arrest with deep hypothermia. , 1995, The Journal of thoracic and cardiovascular surgery.

[48]  T. Achenbach Manual for the child behavior checklist/4-18 and 1991 profile , 1991 .

[49]  P. D. del Nido,et al.  Perioperative effects of alpha-stat versus pH-stat strategies for deep hypothermic cardiopulmonary bypass in infants. , 1997, The Journal of thoracic and cardiovascular surgery.

[50]  E. Zackai,et al.  Psychoeducational profile of the 22q11.2 microdeletion: A complex pattern. , 1999, The Journal of pediatrics.

[51]  F. Kirkham,et al.  Pathophysiology of the cerebral circulation during cardiac surgery , 2000, Critical Care.

[52]  J. Ware,et al.  A comparison of the perioperative neurologic effects of hypothermic circulatory arrest versus low-flow cardiopulmonary bypass in infant heart surgery. , 1993, The New England journal of medicine.

[53]  J. Mayer,et al.  Postoperative course and hemodynamic profile after the arterial switch operation in neonates and infants. A comparison of low-flow cardiopulmonary bypass and circulatory arrest. , 1995, Circulation.

[54]  D. Wypij,et al.  Developmental and neurologic effects of alpha-stat versus pH-stat strategies for deep hypothermic cardiopulmonary bypass in infants. , 2001, The Journal of thoracic and cardiovascular surgery.

[55]  R. Heaton Wisconsin Card Sorting Test manual , 1993 .