Predictors of impaired neurodevelopmental outcomes at one year of age after infant cardiac surgery

Stephanie Fuller, Alexander Nord, Marsha Gerdes, Gil Wernovsky, Gail P. Jarvik, Judy Bernbaum, Elaine Zackai, James William Gaynor

Research output: Contribution to journalArticle

74 Citations (Scopus)

Abstract

Objective: For most newborns, congenital heart defects (CHD) appear to be isolated anomalies and the brain is presumed to have normal developmental potential. Most studies of neurodevelopmental outcomes have focused on operative management strategies. Methods: Infants with complex CHD and no identified syndromes other than 22q11 microdeletions enrolled in a study of apolipoprotein E (APOE) polymorphisms and developmental outcome were evaluated at one year of age; including genetic evaluation and the Bayley Scales of Infant Development-II [mental (MDI) and psychomotor developmental indices (PDI)]. Results: Five hundred and fifty infants enrolled and 359 (20 with 22q11) of 501 survivors (72%) returned. Mean MDI was 90 ± 15 and PDI was 78 ± 18. Genetic syndromes not identified at birth were confirmed in 28 (8.1%) and suspected in 51 (15.0%). By multivariable analysis, suspected/confirmed genetic syndromes and APOE ε2 allele predicted lower MDI and PDI, all p < 0.04. Lower birth weight (p < 0.001) and preoperative intubation (p = 0.012) predicted lower MDI. Higher hematocrit during the initial operation was associated with higher MDI (p = 0.007). Longer postoperative length of stay was predictive of lower PDI (p = 0.002). Additional operations with cardiopulmonary bypass were associated with lower MDI and PDI (both p < 0.002), but use of deep hypothermic circulatory arrest was not. Conclusions: Patient factors (birth weight and preoperative status) are significant determinants of neurodevelopmental outcomes as opposed to operative management strategies. In this cohort, genetic syndromes unsuspected at birth were surprisingly common and correlate with poor neurodevelopmental outcomes. Without multiple congenital anomalies, syndromes may be missed in infancy. Genetic evaluation should be considered in all infants with CHD.

Original languageEnglish (US)
Pages (from-to)40-48
Number of pages9
JournalEuropean Journal of Cardio-thoracic Surgery
Volume36
Issue number1
DOIs
StatePublished - Jul 1 2009
Externally publishedYes

Fingerprint

Thoracic Surgery
Congenital Heart Defects
Birth Weight
Deep Hypothermia Induced Circulatory Arrest
Apolipoprotein E2
Parturition
Apolipoproteins E
Cardiopulmonary Bypass
Hematocrit
Intubation
Survivors
Length of Stay
Alleles
Outcome Assessment (Health Care)
Newborn Infant
Brain

Keywords

  • Apolipoprotein E
  • Congenital
  • Genetic predisposition to disease
  • Heart defects
  • Neurodevelopmental outcomes

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery
  • Pulmonary and Respiratory Medicine

Cite this

Predictors of impaired neurodevelopmental outcomes at one year of age after infant cardiac surgery. / Fuller, Stephanie; Nord, Alexander; Gerdes, Marsha; Wernovsky, Gil; Jarvik, Gail P.; Bernbaum, Judy; Zackai, Elaine; Gaynor, James William.

In: European Journal of Cardio-thoracic Surgery, Vol. 36, No. 1, 01.07.2009, p. 40-48.

Research output: Contribution to journalArticle

Fuller, Stephanie ; Nord, Alexander ; Gerdes, Marsha ; Wernovsky, Gil ; Jarvik, Gail P. ; Bernbaum, Judy ; Zackai, Elaine ; Gaynor, James William. / Predictors of impaired neurodevelopmental outcomes at one year of age after infant cardiac surgery. In: European Journal of Cardio-thoracic Surgery. 2009 ; Vol. 36, No. 1. pp. 40-48.
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abstract = "Objective: For most newborns, congenital heart defects (CHD) appear to be isolated anomalies and the brain is presumed to have normal developmental potential. Most studies of neurodevelopmental outcomes have focused on operative management strategies. Methods: Infants with complex CHD and no identified syndromes other than 22q11 microdeletions enrolled in a study of apolipoprotein E (APOE) polymorphisms and developmental outcome were evaluated at one year of age; including genetic evaluation and the Bayley Scales of Infant Development-II [mental (MDI) and psychomotor developmental indices (PDI)]. Results: Five hundred and fifty infants enrolled and 359 (20 with 22q11) of 501 survivors (72{\%}) returned. Mean MDI was 90 ± 15 and PDI was 78 ± 18. Genetic syndromes not identified at birth were confirmed in 28 (8.1{\%}) and suspected in 51 (15.0{\%}). By multivariable analysis, suspected/confirmed genetic syndromes and APOE ε2 allele predicted lower MDI and PDI, all p < 0.04. Lower birth weight (p < 0.001) and preoperative intubation (p = 0.012) predicted lower MDI. Higher hematocrit during the initial operation was associated with higher MDI (p = 0.007). Longer postoperative length of stay was predictive of lower PDI (p = 0.002). Additional operations with cardiopulmonary bypass were associated with lower MDI and PDI (both p < 0.002), but use of deep hypothermic circulatory arrest was not. Conclusions: Patient factors (birth weight and preoperative status) are significant determinants of neurodevelopmental outcomes as opposed to operative management strategies. In this cohort, genetic syndromes unsuspected at birth were surprisingly common and correlate with poor neurodevelopmental outcomes. Without multiple congenital anomalies, syndromes may be missed in infancy. Genetic evaluation should be considered in all infants with CHD.",
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T1 - Predictors of impaired neurodevelopmental outcomes at one year of age after infant cardiac surgery

AU - Fuller, Stephanie

AU - Nord, Alexander

AU - Gerdes, Marsha

AU - Wernovsky, Gil

AU - Jarvik, Gail P.

AU - Bernbaum, Judy

AU - Zackai, Elaine

AU - Gaynor, James William

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AB - Objective: For most newborns, congenital heart defects (CHD) appear to be isolated anomalies and the brain is presumed to have normal developmental potential. Most studies of neurodevelopmental outcomes have focused on operative management strategies. Methods: Infants with complex CHD and no identified syndromes other than 22q11 microdeletions enrolled in a study of apolipoprotein E (APOE) polymorphisms and developmental outcome were evaluated at one year of age; including genetic evaluation and the Bayley Scales of Infant Development-II [mental (MDI) and psychomotor developmental indices (PDI)]. Results: Five hundred and fifty infants enrolled and 359 (20 with 22q11) of 501 survivors (72%) returned. Mean MDI was 90 ± 15 and PDI was 78 ± 18. Genetic syndromes not identified at birth were confirmed in 28 (8.1%) and suspected in 51 (15.0%). By multivariable analysis, suspected/confirmed genetic syndromes and APOE ε2 allele predicted lower MDI and PDI, all p < 0.04. Lower birth weight (p < 0.001) and preoperative intubation (p = 0.012) predicted lower MDI. Higher hematocrit during the initial operation was associated with higher MDI (p = 0.007). Longer postoperative length of stay was predictive of lower PDI (p = 0.002). Additional operations with cardiopulmonary bypass were associated with lower MDI and PDI (both p < 0.002), but use of deep hypothermic circulatory arrest was not. Conclusions: Patient factors (birth weight and preoperative status) are significant determinants of neurodevelopmental outcomes as opposed to operative management strategies. In this cohort, genetic syndromes unsuspected at birth were surprisingly common and correlate with poor neurodevelopmental outcomes. Without multiple congenital anomalies, syndromes may be missed in infancy. Genetic evaluation should be considered in all infants with CHD.

KW - Apolipoprotein E

KW - Congenital

KW - Genetic predisposition to disease

KW - Heart defects

KW - Neurodevelopmental outcomes

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