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Neurodevelopmental Outcomes in Congenital Heart Disease: A Review

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Jennifer Milillo
Jennifer Milillo
Jennifer Milillo
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Adnan Bakar
Adnan Bakar
Adnan Bakar
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Abstract

Purpose of Review We review the etiologies of neurodevelopmental outcomes in patients with congenital heart disease. Recent Findings Developmental delays have been identified in patients with congenital heart disease (CHD). Though historically neurodevelopmental morbidity in patients with CHD was thought to be surgically related, recent advances in imaging technology and the ability to prospectively provide longitudinal follow-up indicate multiple complex etiologies of neurodevelopmental delay in this group. It has been suggested that alterations in cerebral perfusion is related to cerebral dysgenesis and immaturity. Additionally, while there has been significant improvement in surgical technique, the brain is still vulnerable to ischemia and injury, further placing the patient at risk for developmental delays. Summary The neurodevelopmental and cognitive delays span many domains and may not be detected until adolescence when cognitive and neurobehavioral demands increase. Given these comorbidities, there is a need for early and frequent multidisciplinary assessments of the patient and early identification of delays to provide access to resources to improve these patients’ quality of life.
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INTENSIVE CARE MEDICINE (E CHEUNG AND T CONNORS, SECTION EDITORS)
Neurodevelopmental Outcomes in Congenital Heart Disease: A
Review
Jennifer Milillo
1
&Adnan Bakar
1
Accepted: 8 September 2020
#Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract
Purpose of Review We review the etiologies of neurodevelopmental outcomes in patients with congenital heart disease.
Recent Findings Developmental delays have been identified in patients with congenital heart disease (CHD). Though historically
neurodevelopmental morbidity in patients with CHD was thought to be surgically related, recent advances in imaging technology
and the ability to prospectively provide longitudinal follow-up indicate multiple complex etiologies of neurodevelopmental delay
in this group. It has been suggested that alterations in cerebral perfusion is related to cerebral dysgenesis and immaturity.
Additionally, while there has been significant improvement in surgical technique, the brain is still vulnerable to ischemia and
injury, further placing the patient at risk for developmental delays.
Summary The neurodevelopmental and cognitive delays span many domains and may not be detected until adolescence when
cognitive and neurobehavioral demands increase. Given these comorbidities, there is a need for early and frequent multidisci-
plinary assessments of the patient and early identification of delays to provide access to resources to improve these patients’
quality of life.
Keywords Congenital heart disease .Cardiothoracic surgery .Neurodevelopmental outcomes .Neurocognitive outcomes .
Developmental delay
Introduction
The population of individuals living with congenital heart
disease (CHD) is growing, and with it is a growing need for
access to medical services. This growing population is attrib-
uted to improved survival due to advances in surgical tech-
nique and the development of a medical community focused
on the specialized needs of these patients [1,2]. These spe-
cialized needs include addressing neurodevelopmental and
cognitive impairments which have been recognized as the
most common morbidities in patients with CHD [3].
Children with CHD have been the focus of a great deal of
studies. To date, many of these studies have aimed to identify
the critical times, patient specific, and treatment related, when
the developing brain is most vulnerable to insult.
Neurologic insults place the patients at risk for develop-
mental delays across many domains that extend beyond mea-
surements of IQ; often the impact of these delays does not
manifest until years later [4]. It is important for care providers
within the patient’s medical home to recognize the covert and
overt neurodevelopmental delays and cognitive impairments
that can be seen in patients with CHD, such that interventions
may be employed to improve their neurodevelopmental, cog-
nitive, and behavioral outcomes [5••]. It is therefore helpful to
understand the possible etiologies of these long-term morbid-
ities, which are complex and multifactorial. In particular, as
these children survive into adulthood, there are greater de-
mands of psychosocial interactions and executive function;
frequent re-evaluations may be necessary.
In this review, we aim to review the critical times and risk
for neurologic insult in CHD patients including the antenatal,
perioperative, and postoperative periods. We will further em-
phasize the importance of a multidisciplinary approach in con-
tinual screening for developmental delays as these may evolve
over time with new developmental demands.
This article is part of the Topical Collection on Intensive Care Medicine
*Jennifer Milillo
JMilillo@northwell.edu
1
Department of Pediatrics, Division of Critical Care, Donald and
Barbara Zucker School of Medicine at Hofstra/Northwell, 269-01
76th Avenue, New Hyde Park, NY 11040, USA
https://doi.org/10.1007/s40124-020-00229-2
/ Published online: 21 September 2020
Current Pediatrics Reports (2020) 8:170–175
Content courtesy of Springer Nature, terms of use apply. Rights reserved.
ResearchGate has not been able to resolve any citations for this publication.
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Congenital heart disease (CHD) constitutes the most common congenital malformation, with moderate or severe CHD occurring in around 6 in 1000 live births. Due to advances in medical care, survival rates have increased significantly. Thus, the majority of children with CHD survive until adolescence and adulthood. Children with CHD requiring cardiopulmonary bypass surgery are at risk for neurodevelopmental impairments in various domains, including mild impairments in cognitive and neuromotor functions, difficulties with social interaction, inattention, emotional symptoms, and impaired executive function. The prevalence for these impairments ranges from 20% to 60% depending on age and domain ("high prevalence-low severity"). Domains are often affected simultaneously, leading to school problems with the need for learning support and special interventions. The etiology of neurodevelopmental impairments is complex, consisting of a combination of delayed intrauterine brain development and newly occurring perioperative brain injuries. Mechanisms include altered intrauterine hemodynamic flow as well as neonatal hypoxia and reduced cerebral blood flow. The surgical procedure and postoperative phase add to this cascade of factors interfering with normal brain development. Early identification of children at high risk through structured follow-up programs is mandated to provide individually tailored early interventions and counseling to improve developmental health.
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Neonatal Brain Injury and Timing of Neurodevelopmental Assessment in Patients With Congenital Heart Disease
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Neurodevelopmental Profiles of Children with Congenital Heart Disease at School Age
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Infant Motor Skills After a Cardiac Operation: The Need for Developmental Monitoring and Care
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Neurodevelopmental Abnormalities and Congenital Heart Disease: Insights Into Altered Brain Maturation
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In the past 2 decades, it has become evident that individuals born with congenital heart disease (CHD) are at risk of developing life-long neurological deficits. Multifactorial risk factors contributing to neurodevelopmental abnormalities associated with CHD have been identified; however, the underlying causes remain largely unknown, and efforts to address this issue have only recently begun. There has been a dramatic shift in focus from newly acquired brain injuries associated with corrective and palliative heart surgery to antenatal and preoperative factors governing altered brain maturation in CHD. In this review, we describe key time windows of development during which the immature brain is vulnerable to injury. Special emphasis is placed on the dynamic nature of cellular events and how CHD may adversely impact the cellular units and networks necessary for proper cognitive and motor function. In addition, we describe current gaps in knowledge and offer perspectives about what can be done to improve our understanding of neurological deficits in CHD. Ultimately, a multidisciplinary approach will be essential to prevent or improve adverse neurodevelopmental outcomes in individuals surviving CHD.
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