Neurodevelopmental impairment is a common and important long-term morbidity among infants with congenital heart disease (CHD). More than half of those with complex CHD will demonstrate some form of neurodevelopmental, neurocognitive, and/or psychosocial dysfunction requiring specialized care and impacting long-term quality of life. Preventing brain injury and treating long-term neurologic sequelae in this high-risk clinical population is imperative for improving neurodevelopmental and psychosocial outcomes. Thus, cardiac neurodevelopmental care is now at the forefront of clinical and research efforts. Initial research primarily focused on neurocritical care and operative strategies to mitigate brain injury. As the field has evolved, investigations have shifted to understanding the prenatal, genetic, and environmental contributions to impaired neurodevelopment. This article summarizes the recent literature detailing the brain abnormalities affecting neurodevelopment in children with CHD, the impact of genetics on neurodevelopmental outcomes, and the best practices for neonatal neurocritical care, focusing on developmental care and parental support as new areas of importance. A framework is also provided for the infrastructure and resources needed to support CHD families across the continuum of care settings.

Advances in medical and surgical management of infants with congenital heart disease (CHD) have improved survival and contributed to a rapidly growing population of affected children, adolescents, and adults.1  These successes have created an urgent need to address the long-term sequelae of CHD, with neurodevelopmental impairment now recognized as the most common morbidity experienced by infants who undergo cardiac surgery.2  The seminal work of the Boston Circulatory Arrest Trial initially highlighted the importance of neurodevelopmental outcomes in this population.3  Since then, it has become well documented that nearly half of children with complex CHD experience neurodevelopmental and psychosocial impairments that impact their health-related quality of life.2,411  By adolescence, 65% receive educational and/or psychosocial services.10  Critically, these deficits persist into adulthood, affecting educational achievement, quality of life, employment, and insurance status (Table 1).12,13 

TABLE 1

Summary of Neurodevelopmental Outcomes by Age and Domain

Age GroupDomains AssessedOutcomeMissing Domain(s)
Infants
(birth to 12 mo) 
General Cognition
Motor Skills
Language
Memory 
Scores in normal range
Deficits observed
Scores in low-normal range
Deficits observed 
Executive Function
Audiology 
Toddlers
(1-3.5 y) 
General Cognition
Motor Skills
Language
Behavioral-emotional
Audiology 
Deficits observed
Deficits observed
Deficits observed
Deficits observed
Scores in normal range 
Executive Function
Memory 
Preschoolers
(3.5-5 y) 
General Cognition
Academic Achievement
Motor
Language
Processing Speed
ADHD Symptoms
Behavioral-Emotional
Audiology 
Scores in low-normal range
Scores in normal range
Deficits observed
Deficits observed for more complex measures
Deficits observed
Deficits observed
Deficits observed
Slight deficits observed 
Executive Function
Memory 
School-age children
(5-12 y) 
General Cognition
Academic Achievement
Language
Speech
Motor Skills
Memory
Executive Function
Attention (sustained)
ADHD Symptoms
Visuospatial Skills
Behavioral-Emotional
Audiology 
Scores in low-normal range
Deficits observed
Scores in low-normal range
Deficits observed
Deficits observed
Deficits observed
Deficits observed
Deficits observed
Deficits observed
Deficits observed
Deficits observed
Scores in normal range 
Some domains in single studies only, difficult to generalize
Quality of life 
Adolescents
(13-18 y) 
Academic Achievement
Executive Function
Memory
Visuospatial Skills
Attention (ADHD Symptoms)
Behavioral-Emotional 
Deficits observed
Deficits observed
Deficits observed
Deficits observed
Deficits observed
Deficits observed 
Motor skills
Audiology
Quality of life 
Age GroupDomains AssessedOutcomeMissing Domain(s)
Infants
(birth to 12 mo) 
General Cognition
Motor Skills
Language
Memory 
Scores in normal range
Deficits observed
Scores in low-normal range
Deficits observed 
Executive Function
Audiology 
Toddlers
(1-3.5 y) 
General Cognition
Motor Skills
Language
Behavioral-emotional
Audiology 
Deficits observed
Deficits observed
Deficits observed
Deficits observed
Scores in normal range 
Executive Function
Memory 
Preschoolers
(3.5-5 y) 
General Cognition
Academic Achievement
Motor
Language
Processing Speed
ADHD Symptoms
Behavioral-Emotional
Audiology 
Scores in low-normal range
Scores in normal range
Deficits observed
Deficits observed for more complex measures
Deficits observed
Deficits observed
Deficits observed
Slight deficits observed 
Executive Function
Memory 
School-age children
(5-12 y) 
General Cognition
Academic Achievement
Language
Speech
Motor Skills
Memory
Executive Function
Attention (sustained)
ADHD Symptoms
Visuospatial Skills
Behavioral-Emotional
Audiology 
Scores in low-normal range
Deficits observed
Scores in low-normal range
Deficits observed
Deficits observed
Deficits observed
Deficits observed
Deficits observed
Deficits observed
Deficits observed
Deficits observed
Scores in normal range 
Some domains in single studies only, difficult to generalize
Quality of life 
Adolescents
(13-18 y) 
Academic Achievement
Executive Function
Memory
Visuospatial Skills
Attention (ADHD Symptoms)
Behavioral-Emotional 
Deficits observed
Deficits observed
Deficits observed
Deficits observed
Deficits observed
Deficits observed 
Motor skills
Audiology
Quality of life 

Reprinted from Congenital Heart Disease and Neurodevelopment, first Edition, Kharitonova, M. and Marino B.S., An Emergent Phenotype: A Critical Review of Neurodevelopmental Outcomes for Complex Congenital Heart Disease Survivors During Infancy, Childhood, and Adolescence, pages 55-87, 2016 with permission from Elsevier.

Although optimization of neurodevelopmental outcomes initially centered on neurocritical care and operative strategies, prenatal, genetic, and environmental contributions have become important areas of focus as the field has evolved (Fig 1). Concomitantly, there has been increasing recognition of the need for infrastructure and resources to support CHD families as they navigate the continuum of neurodevelopmental care settings, including the transition to adult providers. This article summarizes the literature across these critical aspects of cardiac neurodevelopmental care.

FIGURE 1

Risk Factors for Neurologic and Neurodevelopmental Abnormalities. Schematic representation of prenatal, perioperative, and social/environmental factors that contribute to neurodevelopmental disabilities in CHD. Prenatally, abnormal cardiac anatomy can alter blood flow and oxygenation to the developing brain, leading to impaired brain development. Concurrently, other pregnancy exposures including abnormal placental development and function and parental well-being can also have a negative impact on the brain. Genetic disruption of brain development may also occur via underlying genetic syndromes or pathogenic variants. Susceptibility genes (ie, apolipoprotein E ε2) can further contribute to postoperative neurodevelopmental deficits. A variety of physiologic/intensive care exposures, such as hypoxemia, additional surgery, and drug exposure are important predictors in the neurocritical care arena. Finally, home environment and socioeconomic status can have a positive or negative influence, depending on the infant’s environment, whereas developmental care interventions that begin in the ICU and continue after discharge may have neurodevelopmental benefit. CBF, cerebral blood flow; ND, neurodevelopment; WM, white matter. Adapted with permission from J. William Gaynor.

FIGURE 1

Risk Factors for Neurologic and Neurodevelopmental Abnormalities. Schematic representation of prenatal, perioperative, and social/environmental factors that contribute to neurodevelopmental disabilities in CHD. Prenatally, abnormal cardiac anatomy can alter blood flow and oxygenation to the developing brain, leading to impaired brain development. Concurrently, other pregnancy exposures including abnormal placental development and function and parental well-being can also have a negative impact on the brain. Genetic disruption of brain development may also occur via underlying genetic syndromes or pathogenic variants. Susceptibility genes (ie, apolipoprotein E ε2) can further contribute to postoperative neurodevelopmental deficits. A variety of physiologic/intensive care exposures, such as hypoxemia, additional surgery, and drug exposure are important predictors in the neurocritical care arena. Finally, home environment and socioeconomic status can have a positive or negative influence, depending on the infant’s environment, whereas developmental care interventions that begin in the ICU and continue after discharge may have neurodevelopmental benefit. CBF, cerebral blood flow; ND, neurodevelopment; WM, white matter. Adapted with permission from J. William Gaynor.

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The research strategy used to inform this article involved searching Pubmed MeSH settings to identify peer-reviewed articles on neurodevelopmental and psychiatric outcomes in CHD published since 2000. Additional studies were included on the basis of the authors’ personal knowledge (including publications before 2000). Nonfull-text and non-English publications were excluded. Authors independently reviewed studies and included/excluded on the basis of the evidence strength. This article is part of a larger series of articles simultaneously published as a supplement in Pediatrics by the Neonatal Cardiac Care Collaborative. Please refer to the executive committee introductory paper for discussion on Class of Recommendations and Level of Evidence (LOE), writing committee organization, and document review and approval.

Recent literature has characterized the timing and patterns of brain injury and altered brain development in CHD (Fig 2). Over the last decade, it has become clear that brain development is altered in utero in CHD fetuses.14  These abnormalities persist through adulthood without evidence of “catch-up” growth.1517  Prenatal aberrations in brain development are evident on ultrasound and MRI by the second and third trimesters of pregnancy.1823  A large, population-based study of ultrasound biometry identified CHD-related brain alterations by 20 weeks’ gestation.18  Smaller fetal MRI cohorts have shown altered cortical development at 22 to 25 weeks19,20  preceding reductions in brain volumes that occur by 30 weeks.14,19 

FIGURE 2

Timing and Pattern of Neuroimaging Abnormalities. This timeline depicts abnormalities identified across key brain regions in neuroimaging studies of brain development in patients with CHD, beginning during the fetal period and extending into adolescence/adulthood. Representative T2-weighted images are provided for each developmental epoch depicting the significant change in brain size and structure that occurs during this period. As demonstrated, brain abnormalities in CHD are global in nature, involving white and gray matter regions at the micro- and macro-structural level. BPD, biparietal diameter; HC, head circumference; TBV, total brain volume.

FIGURE 2

Timing and Pattern of Neuroimaging Abnormalities. This timeline depicts abnormalities identified across key brain regions in neuroimaging studies of brain development in patients with CHD, beginning during the fetal period and extending into adolescence/adulthood. Representative T2-weighted images are provided for each developmental epoch depicting the significant change in brain size and structure that occurs during this period. As demonstrated, brain abnormalities in CHD are global in nature, involving white and gray matter regions at the micro- and macro-structural level. BPD, biparietal diameter; HC, head circumference; TBV, total brain volume.

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This in utero vulnerability is not surprising given the negative impact of aberrant cardiac anatomy on fetal cerebral hemodynamics and substrate delivery (eg, glucose, oxygen) during a period of rapid brain development. Aerobic glycolysis is a critical component of typical brain development throughout this window,24  with increased oxygen delivery facilitating synapse formation, gyrification, sulcation, and increased brain weight from the second to third trimester.25  Normal fetal–placental–cardiac hemodynamics are essential for supplying oxygen and nutrients to the fetus, and placental abnormalities, including decreased weight, vascular abnormalities, and altered perfusion, are prevalent in CHD.2629  Proangiogenic genetic variants are also common and associated with altered placental function and smaller birth head circumference.30  Abnormal cardiac anatomy may also disrupt cerebral oxygen/nutrient delivery via hypoxia and/or altered cerebral blood flow.31  In 1 cohort, CHD fetuses demonstrated 10% and 32% reductions in cerebral oxygen saturation and consumption, respectively, with both associated with smaller brain size.23  Further, CHD fetuses have elevated lactate on MRI spectroscopy, indicative of cerebral anaerobic metabolism.14 

Preterm models indicate the oligodendroglial lineage plays an important role in this impact of CHD-related hypoxia on brain development.32  Selective vulnerability of premyelinating oligodendrocytes to hypoxia/hypoperfusion causes arrested white matter maturation and hypomyelination. Cortical abnormalities follow, driven by impairments in thalamocortical and cortico–cortical connectivity. Intrauterine and perinatal chronic hypoxia models mimicking CHD physiology also demonstrate decreased neuronal progenitor cells in the subventricular zone, as well as hypomyelination and abnormal cortical development.33,34  Similar findings are evident in neuropathological specimens from CHD infants.34 

Up to 40% of CHD infants who undergo cardiothoracic surgery have preoperative brain injury, and another third display new injury postoperatively.3538  The most common pattern is white matter injury (WMI), though stroke/infarct also occur.35,38  Infants with abnormal brain development on prenatal and/or preoperative MRI are at heightened risk of perioperative injury.35,36,39  Further, moderate–severe injury on pre- and/or postoperative MRI is associated with slower brain growth over the 2 to 3 weeks surrounding cardiac surgery,40  suggesting a “two-hit” phenomenon. Data linking WMI with neurodevelopmental deficits have been mixed, but moderate–severe injury is clearly associated with worse neurodevelopmental outcomes.38 

Longer-term brain abnormalities in CHD include reductions in white and gray matter volumes and abnormal gyrification, sulcation, microstructure, metabolism, and cerebral connectivity, many of which are already present preoperatively (Fig 2).1517,35,4151  Partly because of these abnormalities, children with complex CHD have high rates of deficits across academic and neurodevelopmental performance domains, altered language and motor skills, and lower health-related quality of life.2,10,13,1517,35,4143,5157  These data highlight the complexity of brain development in CHD, where fetal brain abnormalities predispose the infant to perioperative injury and ongoing aberrations in brain development that impact neurodevelopmental and psychiatric outcomes long after cardiac surgery.2,13 

Initial data suggest abnormal brain development on fetal MRI is associated with increased perioperative brain injury risk.39,58  However, the expertise and resources required for fetal MRI currently prevent its routine clinical application to obtain detailed brain measures. Postnatal imaging is more accessible and commonly used. Many centers routinely perform cranial ultrasound screening preoperatively in all CHD infants. Cranial ultrasound is 82% sensitive and 93% specific for intraventricular hemorrhage when compared with clinically acquired computed tomography or MRI.59  This same study showed the odds of developing intraventricular hemorrhage increased 1.3-fold for each week decrease in gestational age, resulting in an incidence >50% for very preterm CHD infants, although the majority was low-grade hemorrhage. However, ultrasound may miss many abnormalities seen on MRI, particularly WMI.60  There are currently no conclusive data supporting routine MRI to guide critical care approaches or surgical timing, with only 1 cohort reporting that clinically silent preoperative lesions do not worsen postoperatively.61  Neuroimaging studies are key components of ongoing research efforts that may inform future clinical practice. In the interim, detailed neurologic examinations and consultation with neurology and/or dedicated neurocritical care services may facilitate decision-making regarding neuroimaging.

  1. The utility of routine clinical fetal or postnatal MRI in CHD is unclear. However, regular, detailed neurologic examinations before and after cardiac surgery can be useful to identify infants who would benefit from neuroimaging (Class IIa, LOE C-EO [ie, firm scientific support is not available and the recommendation is consensus of expert opinion])

  2. Cranial ultrasound may be considered to screen for intraventricular hemorrhage or major structural abnormalities in all CHD infants, but may not be useful for WMI (Class IIb, LOE C-LD [ie, firm scientific support is not available because data are limited])

  3. When available, consultation with pediatric neurology and/or neurocritical care services can be beneficial for facilitating optimal neuroimaging use (Class IIa, LOE C-EO)

CHD occurs in common and rare genetic disorders and syndromes (Table 2), some of which are known to be associated with neurodevelopmental dysfunction, whereas other genetic abnormalities have less clear neurodevelopmental implications.62  Genetic factors have been previously recognized as major neurodevelopmental determinants in CHD, independent of cardiac defect or surgical repair.52,63  These include greater risk of motor and cognitive delays in early childhood and school-age,6467  and greater intellectual impairments, poorer academic achievement, worse executive functioning, increased autism symptoms, and lower health-related quality of life in adolescence.56,68,69  These relationships likely begin in the prenatal period and may be independent of or additive to the effects of hemodynamic alterations. Recently, impaired head growth in CHD fetuses showed better correlation with the presence of a genetic syndrome and/or extracardiac anomaly than abnormal fetal hemodynamics.70 

TABLE 2

Genetic Syndromes Associated with CHD

Common SyndromesRare Syndromes
Trisomy 21 LEOPARD syndrome 
Trisomy 18 Kabuki syndrome 
Trisomy 13 Costello syndrome 
22q11 deletion syndrome PHACES syndrome 
Turner syndrome Rubenstein-Taybi syndrome 
Williams syndrome Ellis-van Creveld syndrome 
Jacobsen syndrome Townes-Brocks syndrome 
Alagille syndrome Smith-Lemli-Opitz syndrome 
Holt Oram syndrome Goldenhar syndrome 
Heterotaxy Keutel syndrome 
CHARGE syndrome Cat eye syndrome 
Cornelia de Lange syndrome Char syndrome 
Noonan syndrome Simpson-Golabi-Behmel syndrome 
VACTERL Fryns syndrome 
 Adams-Oliver syndrome 
 Ritscher-Schinzel syndrome 
Common SyndromesRare Syndromes
Trisomy 21 LEOPARD syndrome 
Trisomy 18 Kabuki syndrome 
Trisomy 13 Costello syndrome 
22q11 deletion syndrome PHACES syndrome 
Turner syndrome Rubenstein-Taybi syndrome 
Williams syndrome Ellis-van Creveld syndrome 
Jacobsen syndrome Townes-Brocks syndrome 
Alagille syndrome Smith-Lemli-Opitz syndrome 
Holt Oram syndrome Goldenhar syndrome 
Heterotaxy Keutel syndrome 
CHARGE syndrome Cat eye syndrome 
Cornelia de Lange syndrome Char syndrome 
Noonan syndrome Simpson-Golabi-Behmel syndrome 
VACTERL Fryns syndrome 
 Adams-Oliver syndrome 
 Ritscher-Schinzel syndrome 

An increasing focus on the genetic underpinnings of CHD have established that genetic variants outside of known syndromes are also important. For example, genotyping apolipoprotein E, a cholesterol metabolism regulator involved in neuronal and white matter repair, may be valuable because of associations between the ε2 allele and motor and behavioral deficits in CHD.71,72  New sequencing technologies and strategies for analyzing genetic data also provide key insights. Studies utilizing rare variant burden analyses implicate neurotransmitter, axon guidance, and RASopathy gene pathways in neurodevelopmental disability associated with CHD.73  Further, novel and known pathogenic copy number variants have been associated with motor delay,74  and damaging de novo variants in high-heart, high-brain expressing genes are upregulated in CHD patients with neurodevelopmental disability.75  Importantly, adolescents with these variants demonstrate atypical left hemisphere sulcal development on MRI,76  with similar abnormalities in CHD fetuses.20  These data suggest genetic pathways for abnormal brain development in CHD.

Early diagnosis of genetic disorders is a key component of assessing neurodevelopmental risk and optimizing outcomes.2,62  Genetic evaluation begins prenatally for CHD as reviewed by Haxel and colleagues elsewhere in this issue. A scientific statement from the American Heart Association (AHA) provides recommendations for clinical genetic testing, including karyotype, chromosomal microarray, and targeted testing, as well as ethical considerations and use of genetic counselors.62  Testing results and limitations of the testing should be discussed among clinicians and genetic specialists, communicated to parents, and documented in the medical record. Genetic specialists can also address cost effectiveness and financial burden.

  1. Prenatal and/or postnatal genetic testing, including karyotype and chromosomal microarray, can be effective for informing neurodevelopmental risk in CHD infants (Class IIa, LOE B-NR [ie, moderate-quality evidence from nonrandomized studies])

  2. Additional targeted genetic testing may be considered on the basis of individual phenotypes; the role of routine expanded genetic sequencing remains unclear (Class IIb, LOE C-LD)

  3. Vigilant neurodevelopmental surveillance should be incorporated into routine care for CHD infants with genetic syndromes (Class I, LOE B-NR)

Infants with a prenatal diagnosis of CHD demonstrate less frequent and less severe brain injury and better brain development compared with those with a postnatal diagnosis.77  Prenatal diagnosis also correlates with improved cognition in children with transposition of the great arteries.78  This neuroprotective effect is presumably related to the expectant care provided for the known diagnosis postnatally, leading to more favorable postnatal hemodynamics.38  Gestational age at birth is also important for CHD infants. Elective delivery at <39 weeks’ gestation is associated with increased mortality, morbidity, and worse outcomes.79  Lower gestational age also increases the likelihood and severity of brain injury and abnormal brain development,8082  and is a key predictor of adverse motor, cognitive, language, and psychiatric outcomes.79,83,84  The AHA scientific statement on fetal cardiac medicine supports multidisciplinary approaches to avoid elective delivery at <39 weeks, to create specialized care plans that optimize postnatal hemodynamics, and to consider maternal complications and fetal well-being in delivery decision-making.85  Additional data and recommendations regarding delivery timing are discussed by Haxel and colleagues elsewhere in this issue.

Cardiac intensive care strategies and operative management are modifiable variables that contribute to optimizing neurodevelopmental outcomes. Risk factors for perioperative brain injury and worse neurodevelopmental outcomes include hypoxemia; impaired cerebral oxygenation and hemodynamics; longer time from birth to surgery; prolonged cardiopulmonary bypass and circulatory arrest; larger base deficit during cardiopulmonary bypass; inadequate levels of hypothermia while on cardiopulmonary bypass and/or hyperthermia during the early postoperative period; greater hemodilution on cardiopulmonary bypass; pre- and postoperative cardiac arrest and the need for cardiopulmonary resuscitation; utilization of pre- or postoperative extracorporeal membrane oxygenation support; and single ventricle physiology and aortic arch obstruction.35,8697  Cardiac surgery-related brain injury appears to occur via hypoxic/ischemic effects on oligodendrocyte precursor cells, which is exacerbated by previous hypoxic exposures and alters white matter development.98100  ICU events and postoperative complications that increase length of stay and/or result in repetitive anesthetic exposure contribute to worse neurodevelopmental outcomes. These include higher inotropic score, extubation failure, infection (eg, bacteremia/sepsis, mediastinitis), arrhythmias, postoperative seizures, EEG abnormalities, extracorporeal membrane oxygenation, and additional cardiac and noncardiac operations (eg, residual lesions requiring reintervention, gastrostomy tube).10,52,101110 

In an attempt to minimize perioperative neurologic injury risk, many centers have adopted the use of continuous, noninvasive monitors that provide surrogate markers of oxygen delivery, such as near-infrared spectroscopy (NIRS). This technology provides information on regional tissue oxygenation, expressed as regional oxygen saturation (rSO2). In the days after birth, cerebral oxygenation declines and reaches a threshold where WMI risk likely increases.89  In 1 cohort, lower cerebral rSO2 intraoperatively predicted acute neurologic injury postoperatively.111  Reduced rSO2 variability in the immediate postoperative period may reflect impaired cerebral autoregulation, leading to poor outcomes.112  Further, utilizing NIRS in combination with blood lactate postoperatively enhances prediction of mortality and poor neurodevelopmental outcomes.113  Perioperative neurologic management may also include monitoring for seizure activity. Approximately 10% to 30% of CHD infants have electrographic seizures perioperatively, and up to 60% have abnormal background patterns.114119  Brain injury and altered brain development are associated with abnormal EEG, and new postoperative seizures may suggest new brain injury.116,119,120  Although amplitude-integrated EEG can detect seizures and provide trends in background activity, conventional video EEG remains the standard of care. Adverse neurodevelopmental outcomes are seen in children who have seizures on conventional EEG,102,121  whereas background patterns, but not seizures, relate to outcome for amplitude-integrated EEG.114  Similar to neuroimaging, there are currently no standardized recommendations for use of NIRS or EEG in all CHD infants, although the American Clinical Neurophysiology Society recommends EEG monitoring in neonates undergoing early cardiopulmonary bypass.122  Despite data showing an association of reduced rSO2 and seizures with poorer neurodevelopmental outcomes, there is not yet evidence that incorporation of these monitoring strategies improves neurodevelopmental outcomes.

Although perioperative management is critical for mitigating neurologic risk, operative and postoperative factors explain ≤5% of neurodevelopmental outcome variance, whereas preoperative and patient-specific factors account for ∼25%.97  Nonmodifiable factors associated with adverse neurodevelopmental outcomes include lower maternal education (eg, less than high school compared with graduate school), lower socioeconomic status, genetic disorders/variants, prematurity, and cardiac diagnosis.97  These data highlight the need to optimize current practices while exploring new neuroprotective strategies.

CHD infants require surgical interventions during periods of developmental immaturity. Therefore, in addition to providing optimal medical care, the critical care environment must foster autonomic and behavioral subsystem development.123  Implementation of developmental and kangaroo care improves outcomes in premature infants.124126  Recently, individualized, family-centered developmental care has been identified as a promising neuroprotective model for the cardiac intensive care environment.127  Straightforward modifications include cycled lighting to maintain circadian rhythms; music exposure to minimize noxious stimulation; physiologic positioning with flexion, spinal alignment, and swaddling; and nonpharmacologic comfort measures.128133  Early engagement with therapy services is often essential for instituting these modifications.132  Kangaroo care has not only been shown to be safe for CHD infants, but also improves cardiopulmonary status after extubation and optimizes feeding tolerance.134137 

Oromotor development and coordination of sucking and feeding are among the earliest manifestations of motor control.138  Feeding dysfunction in CHD can lead to impaired somatic growth, which remains critical to motor and cognitive outcomes.135  Furthermore, CHD infants who breastfeed have higher weight-for-age scores,139  likely secondary to fewer episodes of desaturation and temperature decreases.140  Breastfeeding allows mothers to actively participate in their child’s care, leading to improved maternal–child bonding.141,142  Orally fed CHD infants demonstrate more rapid white and gray matter brain maturation compared with their tube-fed counterparts,135  emphasizing the importance of practices facilitating feeding via bottle or breast.

Growing evidence suggests the long-term behavioral, social, and emotional difficulties of children with CHD may be partially attributable to parental mental health beginning prenatally.143145  A systematic review found 80% of CHD parents report trauma symptoms, 25% to 50% report elevated depression and/or anxiety symptoms, and 30% to 80% report severe psychological distress.146  Prenatal exposure to maternal psychological distress has been associated with altered fetal brain metabolism, hippocampal growth, and cortical development in healthy pregnancies.147  Further, abnormal hippocampal and cerebellar development are seen in CHD fetuses exposed to maternal stress and anxiety.148  In the hospital setting, parental stressors include feelings of inadequate preparation and knowledge, concerns about outcomes, infant appearance, the intensive care environment, altered parental roles, financial burdens, and inadequate support.149,150  Importantly, stress is experienced differently by parents. Although mothers tend to focus on the baby, fathers tend to focus on supporting mother and child.150 

Parental mental health interventions are necessary to support CHD families and optimize neurodevelopment. A recent review of parental mental health during intensive care identified only 5 trials of infants with congenital anomalies (4 included CHD; 2 included fathers).151  Although available evidence is minimal, these data suggest interventions are efficacious for reducing parental anxiety and improving maternal coping, mother–infant attachment, parenting confidence, clinical care satisfaction, and infant development.151 

To date, no data have been published linking neurodevelopmental outcomes to the discharge process and transition to home. However, this is a period of unanticipated physical and emotional transitions where greater parental education and support are needed.152,153  Use of discharge specialists increases parents’ perceived discharge readiness.154  Parental education on referral for early intervention services and outpatient therapies should be a priority at discharge to facilitate this transition. Support of parental well-being during this period is also critical.

  1. Intensive care management should minimize clinical complications and exposure to clinical factors that increase neuro developmental risk (Class I, LOE B-NR)

  2. Incorporation of NIRS and EEG into intensive care management may be considered to identify CHD infants at higher neurologic risk (Class IIb, LOE C-LD)

  3. Incorporation of developmental care approaches, including appropriate positioning, cycled lighting, noxious stimuli minimization, nonpharmacologic comfort measure use, kangaroo care, and oral/breastfeeding, may be considered in the ICU to optimize neurodevelopmental outcomes (Class IIb, LOE C-LD)

  4. Screening for parental psychological distress may be reasonable in the intensive care environment as a component of neurodevelopmental care (Class IIb, LOE C-LD)

Children with CHD have a distinct neurodevelopmental profile of early motor deficits, cognitive delays, language impairments, executive dysfunction, inattention, emotional and behavioral disorders, and issues with social cognition.2,13  Up to 50% display impairments across 1 or more domains.7  Increasing neurodevelopmental risk occurs with increasing CHD severity.7  However, significant variation in neurodevelopmental outcomes is present among those with the same cardiac diagnosis,155  likely because of patient-specific risk factors outlined in the AHA and American Academy of Pediatrics (AAP) scientific statement on CHD outcomes (Table 3).2 

TABLE 3

Categories of Pediatric CHD Patients at High Risk for Developmental Disorders or Disabilities

1. Neonates/infants requiring open heart surgery (cyanotic and acyanotic types), for example, HLHS, IAA, PA/IVS, TAPVC, TGA, TOF, tricuspid atresia. 
2. Children with other cyanotic heart lesions not requiring open heart surgery during the neonatal or infant period, for example, TOF with PA and MAPCA(s), TOF with shunt without use of CPB, Ebstein anomaly. 
3. Any combination of CHD and the following comorbidities: 
  3.1. Prematurity (<37 wk) 
  3.2. Developmental delay recognized in infancy 
  3.3. Suspected genetic abnormality or syndrome associated with DD 
  3.4. History of mechanical support (ECMO or VAD use) 
  3.5. Heart transplantation 
  3.6. Cardiopulmonary resuscitation at any point 
  3.7. Prolonged hospitalization (postoperative LOS >2 wk in the hospital) 
  3.8. Perioperative seizures related to CHD surgery 
  3.9. Significant abnormalities on neuroimaging or microcephaly* 
4. Other conditions determined at the discretion of the medical home providers 
1. Neonates/infants requiring open heart surgery (cyanotic and acyanotic types), for example, HLHS, IAA, PA/IVS, TAPVC, TGA, TOF, tricuspid atresia. 
2. Children with other cyanotic heart lesions not requiring open heart surgery during the neonatal or infant period, for example, TOF with PA and MAPCA(s), TOF with shunt without use of CPB, Ebstein anomaly. 
3. Any combination of CHD and the following comorbidities: 
  3.1. Prematurity (<37 wk) 
  3.2. Developmental delay recognized in infancy 
  3.3. Suspected genetic abnormality or syndrome associated with DD 
  3.4. History of mechanical support (ECMO or VAD use) 
  3.5. Heart transplantation 
  3.6. Cardiopulmonary resuscitation at any point 
  3.7. Prolonged hospitalization (postoperative LOS >2 wk in the hospital) 
  3.8. Perioperative seizures related to CHD surgery 
  3.9. Significant abnormalities on neuroimaging or microcephaly* 
4. Other conditions determined at the discretion of the medical home providers 

CHD indicates congenital heart disease; HLHS, hypoplastic left heart syndrome; IAA, interrupted aortic arch; PA/IVS, pulmonary atresia with intact ventricular septum; TA, truncus arteriosus; TAPVC, total anomalous pulmonary venous connection; TGA, transposition of the great arteries; TOF, tetralogy of Fallot; PA, pulmonary atresia; MAPCA, major aortopulmonary collateral arteries; CPB, cardiopulmonary bypass; DD, developmental disorder or disability; ECMO, extracorporeal membrane oxygenation; VAD, ventricular assist device; and LOS, length of stay.

Normative data by sex, including percentiles and z scores, are available from the World Health Organization (www.who.int/childgrowth; accessed February 2010).

Reprinted with permission

Circulation.2012;126:1143-1172

©2012 American Heart Association, Inc.

It is important to recognize the dynamic nature of neurodevelopment in children with CHD.156  Motor delay tends to be prevalent in the first year of life and may improve during early childhood.157,158  However, even at age 10 years, deficits are still common, particularly within adaptive motor function, static balance, and movement quality.159  Infant surgery and exposure to the intensive care environment are key factors affecting motor development.160  Lower socioeconomic status and maternal education are associated with worsening fine motor skills.161  Cognitive and language impairments are also present early, but generally do not demonstrate improvement and may worsen, particularly in children with genetic syndromes and lower socioeconomic status.157,158,161,162  Of interest, a cognitively stimulating home environment is associated with better cognitive development,163  whereas parental stress is associated with worse cognition.164  At school age, cognitive ability typically falls in the low–normal range.165  Lower scores in language, attention, and executive function measures are common.8  In a recent cohort, parent-reported executive dysfunction was present in >60% of school-aged children166  and strongly correlated with health-related quality of life.57,167  Working memory and flexibility appear to be notably problematic, though behavioral dysregulation is prominent in children born preterm, of male sex, or with arch obstruction.166  Behavioral and emotional deficits become more evident throughout childhood and are associated with impairments in other domains, including motor and language development.159,168,169 

Although neurodevelopmental disabilities are common, deficits are mild for many CHD children and may not be detected without formal testing.64  The dynamic nature of neurodevelopmental outcomes and evolution of children’s risk category highlight the need for continual surveillance and use of early intervention services.170  The AHA/AAP 2012 scientific statement provides guidelines for neurodevelopmental evaluation in CHD, using a medical home model that includes the family, primary care providers, and specialty services.2  Consistent with general AAP recommendations, primary care providers are the foundation for the medical home, providing neurodevelopmental surveillance at every visit; standardized developmental screening at 9, 18, and 30 months of age; and autism screening at 18 and 24 months.2,171,172  CHD-specific guidelines expand these recommendations by incorporating a risk assessment for all children with CHD.2  Those identified as high risk should be referred for formal developmental and medical evaluations (Table 3). Developmental evaluation includes referral to early intervention/special education services and multidisciplinary cardiac neurodevelopmental follow-up programs. The medical evaluation includes a developmental history, growth and feeding assessment, motor and audiologic examinations, evaluation by a developmental pediatrician or pediatric neurologist and, if indicated, a geneticist. Medical and developmental reevaluation is recommended between ages 12 to 24 months, 3 to 5 years, and 11 to 12 years.2  A variety of neurodevelopmental assessment tools are available across ages and have been summarized in 2 recent publications from the Cardiac Neurodevelopmental Outcome Collaborative.173,174  An important component of formal neurodevelopmental evaluation across all ages is the communication of results, and any limitations of the testing (eg, related to cooperation of the child) between clinicians and the family, as well as documentation of these discussions in the medical record.

Navigating school systems and providing school-based interventions are essential components of neurodevelopmental care (Table 4).175,176  Neuropsychological or educational evaluations are undertaken to facilitate individualized education plans and 504 plans.177  Testing is repeated every 2 to 3 years, with individualized education plans and 504 plans updated annually to provide home–school communication. Homebound instruction can be established for medically indicated absences. It is beneficial to have an education specialist, or school liaison, facilitate the interaction between the parents/guardians and the school system to provide support and implement recommendations.178  The education specialist facilitates school-based therapies and curriculum modifications, which include extra time for tests/homework, note-taking, recording classes, reading or scribing during tests, and quiet testing environments.179  Education of school staff regarding the child’s medical condition, disability risk, and learning needs is another important component. Recently, children with CHD with executive dysfunction were found to receive similar school services as those without difficulties.166  This highlights the critical need for continued education among schools and the benefit of school liaisons, who can encourage parents to become empowered advocates. As cardiac neurodevelopmental programs evolve, education specialists/school liaisons will be an important component of care that can be supported through a number of mechanisms, including local health and school systems.

TABLE 4

School Based Interventions to Support Learning Needs

Type of InterventionExamples
Regular evaluations including psychological, education, and speech and language Evaluations within neurodevelopmental clinic or school setting 
Collaboration between education liaison in the neurodevelopmental clinic and school setting to share diagnostic information and recommendations Meeting to share medical and developmental history and explanation of possible neurodevelopmental impact of CHD
Annual meetings with school staff and at all transitions to new school buildings 
Development of formal educational plans IEP’s
504 Plans 
Homebound instruction Full-time or partial to maintain schoolwork while missing school for medical reasons 
Development of formal medical plan Activity restrictions
Administration of medication
Emergency plan 
Development of accommodation and modification plans Extended time
Quiet testing environment
Scribe
Reader
Access to teacher notes/outlines 
School based interventions Occupational therapy
Physical therapy
Speech/language therapy 
Vocational skill training or programming  
Advocacy assistance Family education from school liaison regarding special education rights and supports available
Assistance navigating the special education process 
Type of InterventionExamples
Regular evaluations including psychological, education, and speech and language Evaluations within neurodevelopmental clinic or school setting 
Collaboration between education liaison in the neurodevelopmental clinic and school setting to share diagnostic information and recommendations Meeting to share medical and developmental history and explanation of possible neurodevelopmental impact of CHD
Annual meetings with school staff and at all transitions to new school buildings 
Development of formal educational plans IEP’s
504 Plans 
Homebound instruction Full-time or partial to maintain schoolwork while missing school for medical reasons 
Development of formal medical plan Activity restrictions
Administration of medication
Emergency plan 
Development of accommodation and modification plans Extended time
Quiet testing environment
Scribe
Reader
Access to teacher notes/outlines 
School based interventions Occupational therapy
Physical therapy
Speech/language therapy 
Vocational skill training or programming  
Advocacy assistance Family education from school liaison regarding special education rights and supports available
Assistance navigating the special education process 

Created with assistance from Vicki Baker, MS, LPC, St Louis Children’s Hospital.

Caring for a child with CHD can place significant strain on the family dynamic as parents navigate the complexity of their child’s care. Implementation of targeted support strategies for mothers, fathers, and siblings are essential and span medical, classroom, and home arenas (Table 5). Navigating early intervention referrals and routine neurodevelopmental screening can be challenging. A cohort from the Single Ventricle Reconstruction Extension Study identified that less than half of children were receiving early intervention services at age 1 year, and over one-third never received services despite high rates of neurodevelopmental delay.170  These findings highlight referral patterns and the need to support parents. Multidisciplinary cardiac neurodevelopmental follow-up programs can provide a collaborative platform for primary care providers to use as a “homebase” for accessing early interventions, rehabilitative services, behavioral management, counseling, school-based support, and care transitions. Family-specific support typically focuses on parental well-being and support networks, and can be provided by primary care providers and/or cardiac neurodevelopmental follow-up programs,151,180,181  with referral to mental health specialists as indicated. Recent data have identified that parents of children with medical complexity are often unsure of where to find community help or resources for mental health needs, suggesting that policymakers and health care organizations consider family mental health as a component of improving health systems.182 

TABLE 5

Toolkit for Supporting Parents

Domain Tools Available and Important Aspects of Support 
Neurodevelopmental Assessment • Summary of neurodevelopmental sequelae (Table 1)
• AHA Scientific Statement on evaluation and management of neurodevelopmental outcomes2 
• School based interventions to support learning (Table 4
Psychosocial Assessment • Early psychosocial intervention to evaluate caregiving needs and enhance parenting
• Use of standardized instruments and interviews to explore parental mental health - anxiety, depression, post-traumatic stress disorder, guilt, parent-child interaction
• Counseling services through psychologist, social worker, pastoral care, comfort care/palliative care team
• Promoting effective parent-infant transactions
• Inquiring about parental mental health, stress, and family functioning during medical check-ups and hospitalizations
• Development of coping strategies exercise, sleep, narrative therapy, cognitive behavioral therapy, stress management
• Mental health care for parents of babies with CHD151 
• Best practice guidelines: Supporting parents in the NICU200  
Care Coordination • Identification of care team for child, utilization of “medical home” model
• AAP Policy Statement for Patient and Family-Centered Care Coordination201 
• National Resource Center for Patient/Family-Centered Medical Home202 
• Understand role of each team member
• Clear and open communication between patient, family, and providers
• Ask questions
• Inpatient care conferences between medical team and family
• Sign up for MyChart for access to healthcare records
• Discharge coordinator to facilitate transition home
• Family resource center – therapeutic recreation, summer programs, parent networks, school resources 
Care Transition • AHA Scientific Statement on transition to adulthood in adolescents with congenital heart disease197 
• Maintenance of a schedule
• Home videoconferencing for families of children with complex CHD after discharge
• Treatment adherence
• Home based services – skilled nursing, respite care, early intervention, family support program
• Identification of resource needs – transportation, interpretive services
• Transition care facility 
Social Support Networks • Identification of existing social support systems - extended family and friends, parent and sibling support groups
• Focus on self and family preservation (spousal and sibling relationships)
• Support groups
• Sisters By Heart203 
• Pediatric Congenital Heart Association204 
• Mended Hearts205 
• Education regarding CHD
• National Heart, Lung, and Blood Institute, Health Topics, CHD206 
• Center for Disease Control and Prevention, CHD207 
• AAP Congenital Heart Public Health Consortium208 
• AHA Health Topics, CHD209  
Financial Assistance • Contact family financial advocate
• Learn about health plan
• Short and long term financial assistance
• Private assistance – charities, disease specific organizations, churches
• Government assistance – Medicaid and Supplemental Security Income (SSI)
• Debt management program
• Family and Medical Leave Act (FMLA) 
Domain Tools Available and Important Aspects of Support 
Neurodevelopmental Assessment • Summary of neurodevelopmental sequelae (Table 1)
• AHA Scientific Statement on evaluation and management of neurodevelopmental outcomes2 
• School based interventions to support learning (Table 4
Psychosocial Assessment • Early psychosocial intervention to evaluate caregiving needs and enhance parenting
• Use of standardized instruments and interviews to explore parental mental health - anxiety, depression, post-traumatic stress disorder, guilt, parent-child interaction
• Counseling services through psychologist, social worker, pastoral care, comfort care/palliative care team
• Promoting effective parent-infant transactions
• Inquiring about parental mental health, stress, and family functioning during medical check-ups and hospitalizations
• Development of coping strategies exercise, sleep, narrative therapy, cognitive behavioral therapy, stress management
• Mental health care for parents of babies with CHD151 
• Best practice guidelines: Supporting parents in the NICU200  
Care Coordination • Identification of care team for child, utilization of “medical home” model
• AAP Policy Statement for Patient and Family-Centered Care Coordination201 
• National Resource Center for Patient/Family-Centered Medical Home202 
• Understand role of each team member
• Clear and open communication between patient, family, and providers
• Ask questions
• Inpatient care conferences between medical team and family
• Sign up for MyChart for access to healthcare records
• Discharge coordinator to facilitate transition home
• Family resource center – therapeutic recreation, summer programs, parent networks, school resources 
Care Transition • AHA Scientific Statement on transition to adulthood in adolescents with congenital heart disease197 
• Maintenance of a schedule
• Home videoconferencing for families of children with complex CHD after discharge
• Treatment adherence
• Home based services – skilled nursing, respite care, early intervention, family support program
• Identification of resource needs – transportation, interpretive services
• Transition care facility 
Social Support Networks • Identification of existing social support systems - extended family and friends, parent and sibling support groups
• Focus on self and family preservation (spousal and sibling relationships)
• Support groups
• Sisters By Heart203 
• Pediatric Congenital Heart Association204 
• Mended Hearts205 
• Education regarding CHD
• National Heart, Lung, and Blood Institute, Health Topics, CHD206 
• Center for Disease Control and Prevention, CHD207 
• AAP Congenital Heart Public Health Consortium208 
• AHA Health Topics, CHD209  
Financial Assistance • Contact family financial advocate
• Learn about health plan
• Short and long term financial assistance
• Private assistance – charities, disease specific organizations, churches
• Government assistance – Medicaid and Supplemental Security Income (SSI)
• Debt management program
• Family and Medical Leave Act (FMLA) 

  1. Developmental surveillance by primary care providers at every well-child visit can be beneficial for all children with CHD. Standardized developmental screening can also be beneficial at 9, 18, and 30 months, with autism screening at 18 and 24 months. Primary care providers and/or subspecialists can perform this screening (Class IIa, LOE C-LD)

  2. A risk assessment should be performed in children with CHD, and those who meet the below criteria should be considered high risk for neurodevelopmental disabilities (Class I, LOE A):

    • a.

      Neonates/infants requiring open heart surgery

    • b.

      Children with cyanotic heart conditions not requiring open heart surgery during neonatal period/infancy

    • c.

      CHD with comorbidities (Table 3)

    • d.

      Other conditions at discretion of medical home providers

  3. Children with CHD who meet high- risk criteria can be referred to multidisciplinary cardiac neurodevelopmental follow-up and early intervention programs for developmental evaluation and to relevant providers for medical evaluation of growth, feeding, and audiologic assessment (Class IIa, LOE B-NR)

  4. Medical and developmental reevaluation by the primary care provider and/or a subspecialist can be beneficial between ages 12 and 24 months, 3 and 5 years, and 11 and 12 years (Class IIa, LOE C-LD)

  5. Education specialists can be beneficial for facilitating the interaction between parents/guardians and the school system and school-based interventions (Class IIa, LOE C-EO)

  6. Screening for parental stress and mental health problems, and referral for psychosocial intervention, during neurodevelopmental assessments may be reasonable to promote better family functioning and outcomes (Class IIb, LOE C-LD)

Improving survival for patients with even severe cardiac lesions has led to a larger population of adults than children with CHD,1  resulting in a rapid evolution of adolescent and adult CHD neurodevelopmental care. Neurodevelopmental disability occurs twice as commonly in adolescents with CHD than typically developing children.183  These deficits span working memory, perceptual reasoning, cognitive flexibility, and executive and motor function domains,183,184  (Table 1) and correlate with CHD complexity.185  The persistence of neurodevelopmental disability into adulthood, particularly in those with severe CHD, has been associated with higher unemployment, reliance on disability, and lower education levels.186  Evaluation of these deficits encompasses use of formal assessment tools.174  Efforts to improve outcomes include targeted childhood interventions, medical management of inattention/hyperactivity, and career counseling.187 

In addition to neurodevelopmental disabilities, adolescents and adults with CHD have an increased incidence of comorbid psychiatric disorders including anxiety, depression, posttraumatic stress disorder, attention difficulties, adjustment disorder, and early onset dementia.188,189  Up to 69% of CHD patients with mood or anxiety disorders do not receive psychotherapy or psychotropic drugs,190  highlighting the need for lowering thresholds for referral to and/or adoption of psychologists into comprehensive programs.191  Given this high prevalence and limitations in access to therapies, it is essential that routine care include screening for these disorders coupled with standardized treatment referral pathways. Approaches for mental health care in pediatric practices192  and psychosocial screening tools for adolescents and young adults193  are available from the AAP. Additional tools may be useful for adults with CHD.194,195 

The complex comorbidities found in the adult CHD population make it imperative that care is transitioned from pediatric- to adult-centered providers. Currently, there are many barriers to this transition (Table 6). It is estimated that only 48% of CHD adolescents successfully transfer to adult centers.196  The most recent AHA recommendations include a 3-step process spanning many years.197  The pretransition period occurs throughout childhood and is dedicated to creating a foundation for lifelong care. Ideally, at age 12 to 14 years, patients advance to a transition curriculum with inclusion of residual hemodynamic concerns, insurance planning, future education, and employment goals. The third phase is transfer, when responsibility shifts from the pediatric to adult provider, ideally by age 21. A well-planned and coordinated effort is paramount to success. Each individual should have a detailed plan, including their specific cardiac physiology, medical history, previous interventions, medication list, diagnostic studies, functional status, and comorbidities. Successful models include a dedicated staff member who assumes primary responsibility198  and use of resources including mobile applications and Web pages, such as gottransition.org.199 

TABLE 6

Barriers to Transition from Pediatric to Adult Centers

DomainBarriers to TransitionRecommendation
Health Care System • Limited/no insurance coverage
• Lack of ACHD provider availability/training
• Institutional deficits due to lack of staff/training in transition 
• Create structured transition plan using dedicated coordinator
• Offer resources to establish/maintain adequate insurance coverage 
Neurocognitive • Neurodevelopmental delay
• Knowledge barriers concerning diagnosis or transition process
• Maturity of patient to adhere to plan
• Ability to assume decision-making responsibilities 
• Use technology to target disease knowledge and self-management behaviors
• Formal evaluation for signs of transition readiness 
Psychosocial • Deficit in self-management skills
• Impaired psychosocial function including anxiety/depression
• Unstable life circumstances 
• Evaluate/refer for management of comorbid diagnoses
• Access to social worker 
Relationships • Parent/patient attachment to provider
• Different beliefs regarding quality between pediatric/adult providers
• Lack of expectation to transition
• Legal issues regarding healthcare power of attorney 
• Allow patient to meet/interact with provider before transition
• Create joint clinic visits attended by pediatric and adult providers
• Begin transition preparation early in adolescence 
DomainBarriers to TransitionRecommendation
Health Care System • Limited/no insurance coverage
• Lack of ACHD provider availability/training
• Institutional deficits due to lack of staff/training in transition 
• Create structured transition plan using dedicated coordinator
• Offer resources to establish/maintain adequate insurance coverage 
Neurocognitive • Neurodevelopmental delay
• Knowledge barriers concerning diagnosis or transition process
• Maturity of patient to adhere to plan
• Ability to assume decision-making responsibilities 
• Use technology to target disease knowledge and self-management behaviors
• Formal evaluation for signs of transition readiness 
Psychosocial • Deficit in self-management skills
• Impaired psychosocial function including anxiety/depression
• Unstable life circumstances 
• Evaluate/refer for management of comorbid diagnoses
• Access to social worker 
Relationships • Parent/patient attachment to provider
• Different beliefs regarding quality between pediatric/adult providers
• Lack of expectation to transition
• Legal issues regarding healthcare power of attorney 
• Allow patient to meet/interact with provider before transition
• Create joint clinic visits attended by pediatric and adult providers
• Begin transition preparation early in adolescence 

  1. Screening for neurodevelopmental and psychological disorders should continue in adults with CHD and be coupled with standardized treatment pathways (Class I, LOE C-LD)

  2. Transition of care from the pediatric to adult clinical care arena through the medical home model should use a structured plan including parental partnership (Class I, LOE C-LD)

Neurodevelopmental care has become a critical component of treating children with CHD. Brain injury and abnormal brain development occur through multifactorial pathways that begin prenatally and underlie longstanding neurodevelopmental deficits. A multifaceted, longitudinal, family-based approach to screening, evaluation, and treatment of neurodevelopmental disabilities is essential for optimizing neurodevelopmental outcomes. Recognition of high-risk clinical factors, incorporation of parental and family support, and implementation of developmental care strategies in the intensive care environment are important aspects of clinical practice. Genetic testing, neuroimaging, NIRS, EEG, and parental mental health screening may be useful in evaluating neurodevelopmental risk and are areas of ongoing investigation. Beyond the inpatient setting, routinely screening and evaluating neurodevelopment throughout childhood and adolescence, incorporating school liaisons in neurodevelopmental programs, and providing support and resources as children transition to adult care providers all facilitate a comprehensive longitudinal neurodevelopmental approach.

Drs Ortinau, Smyser, and Arthur conceptualized and designed the manuscript, drafted the initial manuscript, contributed figures and tables, and reviewed and revised the manuscript; Dr Gordon conceptualized and designed the manuscript, drafted the initial manuscript, and reviewed and revised the manuscript; Drs Heydarian and Marino conceptualized and designed the manuscript, contributed figures and tables, and reviewed and revised the manuscript; Drs Wolovits, Nedrelow, and Levy conceptualized and designed the manuscript, and reviewed and revised the manuscript; and all authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

FUNDING: The Neonatal Heart Society contributed an educational grant to the project, NeoC3. The Neonatal Heart Society, on a regular basis, applies and receives several unrestrictive educational grants for several internal projects from the following organizations and companies: Abbott Formula, Mead Johnson, Cheisi, Mallinckrodt, Prolacta, and Medtronic. This work was also supported by the National Institutes of Health (grant no. K23HL141602, CMO, and grant no. P50HD103525, CDS). The grants received from industry partners were used solely to offset the cost of publishing this supplement in Pediatrics. The industry supporters did not contribute to the design or conduct of this study.

CONFLICT OF INTEREST DISCLAIMER: The authors have indicated they have no conflicts of interest relevant to this article to disclose.

The guidelines/recommendations in this article are not American Academy of Pediatrics policy, and publication herein does not imply endorsement.

     
  • AAP

    American Academy of Pediatrics

  •  
  • AHA

    American Heart Association

  •  
  • CHD

    congenital heart disease

  •  
  • LOE

    level of evidence

  •  
  • NIRS

    near-infrared spectroscopy

  •  
  • rSO2

    regional oxygen saturation

  •  
  • WMI

    white matter injury

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