Current insights regarding neurological and developmental abnormalities in children and young adults with complex congenital cardiac disease

Cardiology in the Young

Volumn 16, Issue SUPPL. 1, 2006, Pages 92-104

  Wernovsky, Gil ^a  

^a CHILDREN S HOSPITAL OF PHILADELPHIA   (United States)

Author keywords

Cardiac surgery; Congenital malformations; Quality of life

Indexed keywords

APOLIPOPROTEIN E; OXYGEN;

ARTIFICIAL VENTILATION; BRAIN FUNCTION; BRAIN OXYGEN CONSUMPTION; CARDIOPULMONARY BYPASS; CHILD DEVELOPMENT; CHROMOSOME 22Q; CHROMOSOME DELETION; COMORBIDITY; CONGENITAL HEART DISEASE; DEVELOPMENTAL DISORDER; DIGEORGE SYNDROME; DISEASE SEVERITY; DOWN SYNDROME; ENCEPHALOMALACIA; ENVIRONMENTAL FACTOR; EPILEPSY; EXTRACORPOREAL OXYGENATION; FONTAN PROCEDURE; HEART ARREST; HEART SINGLE VENTRICLE; HEMATOCRIT; HEREDITY; HUMAN; HYPOTHERMIA; INTELLIGENCE QUOTIENT; MICROCEPHALY; NEUROLOGIC DISEASE; OPEN HEART SURGERY; OUTCOME ASSESSMENT; PATHOLOGICAL ANATOMY; PATIENT MONITORING; PERIOPERATIVE PERIOD; PRENATAL DEVELOPMENT; PREOPERATIVE EVALUATION; QUALITY OF LIFE; REPERFUSION INJURY; REVIEW; SOCIAL STATUS; SOCIOECONOMICS; SYNDROME CHARGE; TREATMENT OUTCOME; TRISOMY 13; TRISOMY 18; TRISOMY 21; WILLIAMS BEUREN SYNDROME; ACADEMIC ACHIEVEMENT; ADULT; ARTICLE; ATTENTION; BRAIN MALFORMATION; CENTRAL NERVOUS SYSTEM DISEASE; CHILD; CHILD BEHAVIOR; CLINICAL STUDY; EXPERIMENTAL DESIGN; FETUS CIRCULATION; HANDWRITING; HOSPITAL PATIENT; INTENSIVE CARE; INTRAOPERATIVE PERIOD; LABORATORY TEST; LONGITUDINAL STUDY; MENTAL FUNCTION; OUTPATIENT CARE; POSTOPERATIVE PERIOD; PREOPERATIVE PERIOD; PREVALENCE; REOPERATION; RISK ASSESSMENT; SURGICAL RISK; CEREBROVASCULAR DISEASE; CONGENITAL HEART MALFORMATION; PROGNOSIS;

ADULT; CEREBROVASCULAR DISORDERS; CHILD; DEVELOPMENTAL DISABILITIES; HEART DEFECTS, CONGENITAL; HUMANS; PROGNOSIS; QUALITY OF LIFE;

EID: 33646192157     PISSN: 10479511     EISSN: 14671107     Source Type: Journal    
DOI: 10.1017/S1047951105002398     Document Type: Review

References (93)

1. Wernovsky G, Jonas RA, Hickey PR, et al. Clinical neurologic and developmental studies after cardiac surgery utilizing hypothermic circulatory arrest and cardiopulmonary bypass. Cardiol Young 1993; 3: 308-316.
2. Gaynor JW, Bridges ND, Cohen MI, et al. Predictors of outcome after the Fontan operation: Is hypoplastic left heart syndrome still a risk factor? J Thorac Cardiovasc Surg 2002; 123: 237-245.
3. Mayer Jr JE, Bridges ND, Lock JE, et al. Factors associated with marked reduction in mortality for Fontan operations in patients with single ventricle. J Thorac Cardiovasc Surg 1992; 103: 444-452.
4. Wernovsky G, Mayer Jr JE, Jonas RA, et al. Factors influencing early and late outcome of the arterial switch operation for transposition of the great arteries. J Thorac Cardiovasc Surg 1995; 109: 289-302.
5. Tweddell JS, Hoffman GM, Mussatto KA, et al. Improved survival of patients undergoing palliation of hypoplastic left heart syndrome: lessons learned from 115 consecutive patients. Circulation 2002; 106: I-82-I-89.
6. Wernovsky G, Stiles KM, Gauvreau K, et al. Cognitive development after the Fontan operation. Circulation 2000; 102: 883-889.
7. Forbess JM, Visconti KJ, Hancock-Friesen C, et al. Neurodevelopmental outcome after congenital heart surgery: Results from an institutional registry. Circulation 2002; 106: I95-I102.
8. 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: I138-I142.
9. Bellinger DC, Wypij D, duPlessis AJ, et al. Neurodevelopmental status at eight years in children with dextro-transposition of the great arteries: the Boston Circulatory Arrest Trial. J Thorac Cardiovasc Surg 2003; 126: 1385-1396.
10. Gaynor JW, Gerdes M, Zackai EH, et al. Apolipoprotein E genotype and neurodevelopmental sequelae of infant cardiac surgery. J Thorac Cardiovasc Surg 2003; 126: 1736-1745.
11. Limperopoulos C, Majnemer A, Shevell MI, et al. Neurodevelopmental status of newborns and infants with congenital heart defects before and after open heart surgery. J Pediatr 2000; 137: 638-645.
12. Limperopoulos C, Majnemer A, Shevell M, et al. Long-term neuromotor sequelae in young infants with congenital heart defects after cardiac surgery. Ann Neurol 2001; 50: S94.
13. Limperopoulos C, Majnemer A, Shevell MI, et al. Functional limitations in young children with congenital heart defects after cardiac surgery. Pediatrics 2001; 108: 1325-1331.
14. Limperopoulos C, Majnemer A, Shevell MI, et al. Predictors of developmental disabilities after open heart surgery in young children with congenital heart defects. J Pediatr 2002; 141: 51-58.
15. Oates RK, Simpson JM, Turnbull JA, et al. The relationship between intelligence and duration of circulatory arrest with deep hypothermia. J Thorac Cardiovasc Surg 1995; 110: 786-792.
16. Mahle WT, Clancy RR, Moss EM, et al. Neurodevelopmental-outcome and lifestyle assessment in school-aged and adolescent children with hypoplastic left heart syndrome. Pediatrics 2000; 105: 1082-1089.
17. Hemphill L, Uccelli P, Winner K, et al. Narrative discourse in young children with histories of early corrective heart surgery. J Speech Lang Hear Res 2002; 45: 318-331.
18. Bellinger DC, Bernstein JH, Kirkwood MW, et al. Visual-spatial skills in children after open-heart surgery. J Dev Behav Pediatr 2003; 24: 169-179.
19. Goldberg CS, Schwartz EM, Brunberg JA, et al. Neurodevelopmental outcome of patients after the Fontan operation: A comparison between children with hypoplastic left heart syndrome and other functional single ventricle lesions. J Pediatr 2000; 137: 646-652.
20. Kirshbom PM, Flynn TB, Clancy RR, et al. Late neurodevelopmental outcome after repair of total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg 2005; 129: 1091-1097.
21. Wray J, Sensky T. Congenital heart disease and cardiac surgery in childhood: Effects on cognitive function and academic ability. Heart 2001; 85: 687-691.
22. Shillingford AJ, Glanzman MM, Ittenbach RF, et al. Inattention and hyperactivity are common 6-11 years after neonatal cardiac surgery [Abstract]. Pediatr Cardiol 2005; 25: 575A-576A.
23. Hoffman TM, Wernovsky G, Atz AM, et al. Efficacy and safety of milrinone in preventing low cardiac output syndrome in infants and children after corrective surgery for congenital heart disease. Circulation 2003; 107: 996-1002.
24. Wernovsky G, Wypij D, Jonas RA, 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. Circulation 1995; 92: 2226-2235.
25. Hovels-Gurich HH, Seghaye MC, Schnitker R, et al. Long-term neurodevelopmental outcomes in school-aged children after neonatal arterial switch operation. J Thorac Cardiovasc Surg 2002; 124: 448-458.
26. Clancy RR, McGaurn SA, Goin JE, et al. Allopurinol neurocardiac protection trial in infants undergoing heart surgery using deep hypothermic circulatory arrest. Pediatrics 2001; 108: 61-70.
27. Glauser TA, Rorke LB, Weinberg PM, et al. Congenital brain anomalies associated with the hypoplastic left heart syndrome. Pediatrics 1990; 85: 984-990.
28. Shillingford AJ, Marino BS, Ittenbach RF, et al. Microcephaly is common in neonates with hypoplastic left heart syndrome. Pediatr Cardiol 2005; 25: 581A.
29. Licht DJ, Wang JJ, Silvestre DW, et al. Preoperative cerebral blood flow is diminished in neonates with severe congenital heart defects. J Thorac Cardiovasc Surg 2004; 128: 841-849.
30. Mahle WT, Tavani F, Zimmerman RA, et al. An MRI study of neurological injury before and after congenital heart surgery. Circulation 2002; 106: I109-I114.
31. Chen CY, Zimmerman RA, Faro S, et al. MR of the cerebral operculum: topographic identification and measurement of interopercular distances in healthy infants and children. AJNR Am J Neuroradiol 1995; 16: 1677-1687.
32. Christen HJ, Hanefeld F, Kruse E, et al. Foix-Chavany-Marie (anterior operculum) syndrome in childhood: A reappraisal of Worster-Drought syndrome. Dev Med Child Neurol 2000; 42: 122-132.
33. Ogawa H, Ito S, Nomura T. Oral cavity representation at the frontal operculum of macaque monkeys. Neurosci Res 1989; 6: 283-298.
34. Limperopoulos C, Majnemer A, Shevell MI, et al. Neurologic status of newborns with congenital heart defects before open heart surgery. Pediatrics 1999; 103: 402-408.
35. Bellinger DC, Wypij D, Kuban KCK, 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. Circulation 1999; 100: 526-532.
36. Kirshbom PM, Myung RJ, Gaynor JW, et al. Preoperative pulmonary venous obstruction affects long-term outcome for survivors of total anomalous pulmonary venous connection repair. Ann Thorac Surg 2002; 74: 1616-1620.
37. Galli KK, Zimmerman RA, Jarvik GP, et al. Periventricular leukomalacia is common after neonatal cardiac surgery. J Thorac Cardiovasc Surg 2004; 127: 692-704.
38. Chen CC, Huang CB, Chung MY, et al. Periventricular echogenicity is related to delayed neurodevelopment of preterm infants 1. Am J Perinatol 2004; 21: 483-489.
39. Monset-Couchard M, de Bethmann O, Kastler B. Mid- and long-term outcome of 166 premature infants weighing less than 1,000 g at birch, all small for gestational age 2. Biol Neonate 2002; 81: 244-254.
40. Van Houten JP, Rothman A, Bejar R. High incidence of cranial ultrasound abnormalities in full-term infants with congenital heart disease. Am J Perinatol 1996; 13: 47-53.
41. Kramer HH, Majewski F, Trampisch HJ, et al. Malformation patterns in children with congenital heart disease 1. Am J Dis Child 1987; 141: 789-795.
42. Jones M. Anomalies of the brain and congenital heart disease: A study of 52 necropsy cases 1. Pediatr Pathol 1991; 11: 721-736.
43. Miller G, Eggli K, Contant C, et al. Postoperative neurologic complications after open heart surgery on young infants. Arch Pediatr Adolesc Med 1995; 149: 764-768.
44. Donofrio MT, Bremer YA, Schieken RM, et al. Autoregulation of cerebral blood flow in fetuses with congenital heart disease: The brain sparing effect. Pediatr Cardiol 2003; 24: 436-443.
45. Kaltman JR, Di H, Tian Z, et al. Impact of congenital heart disease on cerebrovascular blood flow dynamics in the fetus. Ultrasound Obst Gyn 2005; 25: 32-36.
46. Naeye RL. Transposition of the great arteries and prenatal growth. Arch Path 1966; 82: 412-418.
47. Ricci M, Lombardi P, Galindo A, et al. Single-ventricle physiology reduces cerebral oxygen delivery in a piglet model. Ann Thorac Surg 2005; 80: 686-694.
48. du Plessis AJ, Jonas RA, Wypij D, et al. Perioperative effects of alpha-stat versus pH-stat strategies for deep hypothermic cardiopulmonary bypass in infants. J Thorac Cardiovasc Surg 1997; 114: 991-1000.
49. Bellinger DC, Wypij D, du Plessis AJ, et al. Developmental and neurologic effects of alpha-stat versus pH-stat strategies for deep hypothermic cardiopulmonary bypass in infants. J Thorac Cardiovasc Surg 2001; 121: 374-383.
50. Wypij D, Newburger JW, Rappaport LA, et al. The effect of duration of deep hypothermic circulatory arrest in infant heart surgery on late neurodevelopment: The Boston Circulatory Arrest Trial. J Thorac Cardiovasc Surg 2003; 126: 1397-1403.
51. Forbess JM, Visconti KJ, Bellinger DC, et al. Neurodevelopmental outcomes after biventricular repair of congenital heart defects. J Thorac Cardiovasc Surg 2002; 123: 631-639.
52. Kern JH, Hinton VJ, Nereo NE, et al. Early developmental outcome after the Norwood procedure for hypoplastic left heart syndrome. Pediatrics 1998; 102: 1148-1152.
53. Uzark K, Lincoln A, Lamberti JJ, et al. Neurodevelopmental outcomes in children with Fontan repair of functional single ventricle. Pediatrics 1998; 101: 630-633.
54. Wells FC, Coghill S, Caplan HL, et al. Duration of circulatory arrest does influence the psychological development of children after cardiac operation in early life. J Thorac Cardiovasc Surg 1983; 86: 823-831.
55. Baum M, Freier MC, Freeman K, et al. Neuropsychological outcome of infant heart transplant recipients. J Pediatr 2004; 145: 365-372.
56. Skaryak LA, Lodge AJ, Kirshbom PM, et al. Low-flow cardiopulmonary bypass produces greater pulmonary dysfunction than circulatory arrest. Ann Thorac Surg 1996; 62: 1284-1288.
57. McGrath E, Wypij D, Rappaport LA, et al. Prediction of IQ and achievement at age 8 years from neurodevelopmental status at age 1 year in children with D-transposition of the great arteries. Pediatrics 2004; 114: E572-E576.
58. Hack M, Taylor HG, Drotar D, et al. Poor predictive validity of the Bayley Scales of Infant Development for cognitive function of extremely low birch weight children at school age. Pediatrics 2005; 116: 333-341.
59. Newburger JW, Jonas RA, Wernovsky G, et al. A comparison of the perioperative neurologic effects of hypothermic circulatory arrest versus low-flow cardiopulmonary bypass in infant heart surgery. N Engl J Med 1993; 329: 1057-1064.
60. Bellinger DC, Jonas RA, Rappaport LA, et al. Developmental and neurologic status of children after heart surgery with hypothermic circulatory arrest or low-flow cardiopulmonary bypass. N Engl J Med 1995; 332: 549-555.
61. Shinoka T, ShumTim D, Jonas RA, et al. Higher hematocrit improves cerebral outcome after deep hypothermic circulatory arrest. J Thorac Cardiovasc Surg 1996; 112: 1610-1620.
62. Jonas RA, Wypij D, Roth SJ, et al. The influence of hemodilution on outcome after hypothermic cardiopulmonary bypass: Results of a randomized trial in infants. J Thorac Cardiovasc Surg 2003; 126: 1765-1774.
63. Tweddell JS, Hoffman GM. Postoperative management in patients with complex congenital heart disease. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2002; 5: 187-205.
64. Parr GVS, Blackstone EH, Kirklin JW. Cardiac performance and mortality early after intracardiac surgery in infants and young children. Circulation 1975; 51: 867-874.
65. Ungerleider RM, Shen I, Yeh T, et al. Routine mechanical ventricular assist following the Norwood procedure - improved neurologic outcome and excellent hospital survival. Ann Thorac Surg 2004; 77: 18-22.
66. Greeley WJ, Ungerleider RM, Kern FH, et al. Effects of cardiopulmonary bypass on cerebral blood flow in neonates, infants, and children. Circulation 1989; 80: 209-215.
67. Greeley WJ, Kern FH, Ungerleider RM, et al. The effect of hypothermic cardiopulmonary bypass and total circulatory arrest on cerebral metabolism in neonates, infants and children. J Thorac Cardiovasc Surg 1991; 101: 783-794.
68. Mault JR, Ohtake S, Klingensmith ME, et al. Cerebral metabolism and circulatory arrest: Effects of duration and strategies for protection. Ann Thorac Surg 1993; 55: 57-64.
69. Bassan H, Gauvreau K, Newburger JW, et al. Identification of pressure passive cerebral perfusion and its mediators after infant cardiac surgery 2. Pediatr Res 2005; 57: 35-41.
70. Clancy RR, Sharif U, Ichord R, et al. Electrographic neonatal seizures after infant heart surgery. Epilepsia 2005; 46: 84-90.
71. Bellinger DC, Rappaport LA, Wypij D, et al. Patterns of developmental dysfunction after surgery during infancy to correct transposition of the great arteries. J Dev Behav Pediatr 1997; 18: 75-83.
72. Newburger JW, Wypij D, Bellinger DC, et al. Length of stay after infant heart surgery is related to cognitive outcome at age 8 years. J Pediatr 2003; 143: 67-73.
73. Newburger JW, Silbert AR, Buckley LP, et al. Cognitive function and age at repair of transposition of the great arteries in children. N Engl J Med 1984; 310: 1495-1499.
74. Aisenberg RB, Rosenthal A, Wolff P, et al. Hypoxemia and critical flicker frequency in congenital heart disease. AJDC 1974; 128: 335-341.
75. Aisenberg RB, Rosenthal A, Nadas AS, et al. Developmental delay in infants with congenital heart disease: Correlation with hypoxemia and congestive heart failure. Ped Cardiol 1982; 3: 133-137.
76. Linde LM, Rasof B, Dunn OJ. Longitudinal studies of intellectual and behavioral development in children with congenital heart disease. Acta Pediatr Scand 1970; 59: 169-176.
77. Feldt RH, Ewert JC, Stickler GB, et al. Children with congenital heart disease. Motor development and intelligence. Am J Dis Child 1969; 117: 281-287.
78. Aram DM, Ekelman BL, Ben-Shachar G, et al. Intelligence and hypoxemia in children with congenital heart disease: Fact or artifact? J Am Coll Cardiol 1985; 6: 889-893.
79. Kramer HH, Awiszus D, Sterzel U, et al. Development of personality in children with congenital heart disease. J Child Psychol Psychiatr 1989; 30: 299-308.
80. Silbert AR, Wolff PH, Mayer B, et al. Cyanotic heart disease and psychological development. Pediatrics 1969; 43: 192-200.
81. Wright M, Nolan T. Impact of cyanotic heart disease on school performance. Arch Dis Child 1994; 71: 64-70.
82. Rosenthal A. Visual simple reaction time in cyanotic heart disease. Am J Dis Child 1967; 114: 139-143.
83. Bass JL, Corwin M, Gozal D, et al. The effect of chronic or intermittent hypoxia on cognition in childhood: A review of the evidence. Pediatrics 2004; 114: 805-816.
84. Gibson D, Groeneweg G, Jerry P, et al. Age and pattern of intellectual decline among Down syndrome and other mentally retarded adults. Int J Rehabil Res 1988; 11: 47-55.
85. Barnea-Goraly N, Menon V, Krasnow B, et al. Investigation of white matter structure in velocardiofacial syndrome: A diffusion tensor imaging study. Am J Psychiat 2003; 160: 1863-1869.
86. Amelsvoort Tv T, Henry J, Morris R, et al. Cognitive deficits associated with schizophrenia in velo-cardio-facial syndrome. Schizophr Res 2004; 70: 223-232.
87. Coon PD, Rychik J, Novello RT, et al. Thrombus formation after the Fontan operation. Ann Thorac Surg 2001; 71: 1990-1994.
88. duPlessis AJ, Chang AC, Wessel DL, et al. Cerebrovascular accidents following the Fontan operation. Pediatr Neurol 1995; 12: 230-236.
89. Gentles TL, Gauvreau K, Mayer Jr JE, et al. Functional outcome after the Fontan operation: Factors influencing late morbidity. J Thorac Cardiovasc Surg 1997; 114: 392-403.
90. Gentles TL, Mayer Jr JE, Gauvreau K, et al. Fontan operation in five hundred consecutive patients: Factors influencing early and late outcome. J Thorac Cardiovasc Surg 1997; 114: 376-391.
91. Forbess JM, Visconti KJ, Bellinger DC, et al. Neurodevelopmental outcomes in children after the Fontan operation. Circulation 2001; 104: I127-I132.
92. Ikle L, Hale K, Fashaw L, et al. Developmental outcome of patients with hypoplastic left heart syndrome treated with heart transplantation. J Pediatr 2003; 142: 20-25.
93. Lozano S, Mossad E. Cerebral function monitors during pediatric cardiac surgery: Can they make a difference. J Cardiothor Vasc Anesth 2004; 18: 645-656.