Neurodevelopmental Outcome in Preschool Survivors of Complex Congenital Heart Disease: Implications for Clinical Practice

doi:10.1016/j.pedhc.2006.03.008

Journal of Pediatric Health Care

Volume 21, Issue 1, January–February 2007, Pages 3-12

https://doi.org/10.1016/j.pedhc.2006.03.008 Get rights and content

Abstract

Introduction

The purpose of this study was to describe the neurodevelopmental outcome of preschool survivors of Hypoplastic Left Heart Syndrome (HLHS) and Transposition of the Great Arteries (TGA) in the modern surgical era.

Methods

A cross-sectional design was used to evaluate 26 children (13 HLHS, 13 TGA), ages 3.5 to 6 years, at a Midwestern children’s hospital. Measures included McCarthy Scales of Children’s Abilities, Woodcock Johnson III Tests of Achievement, Developmental Test of Visual-Motor Integration, Receptive One-Word Vocabulary Test, Expressive One-Word Vocabulary Test, and Child Behavior Checklist.

Results

Although intelligence quotient scores fell within the average range for both groups (TGA = 110.5 vs. HLHS = 97), the difference between groups was clinically meaningful (effect size = .79). The HLHS group showed more problems than did the TGA group with visual-motor skills, expressive language, attention, and externalizing behavior.

Discussion

Results suggest that even in the modern surgical era, regular developmental screening for these patients is critical. Pediatric nurse practitioners play an important role in educating parents about the potential developmental risks to these children.

Section snippets

Patient Population

The target population consisted of two groups of subjects. One group consisted of consecutive children who had survived the Norwood procedure for HLHS or other CHD with significant obstruction to systemic blood flow, ages 3 to 6 years. This age group was selected because it allowed for a more comprehensive assessment of neurodevelopmental skills and school readiness than is possible in a younger child. In addition, these children had their surgeries at a point in time when the combined

Demographics

Mean age at the time of neurodevelopmental assessment was 4.7 years (range 3-6 years old, SD = 10 months). Seventy-three percent of the subjects were male, and 96% were White. Mean socioeconomic status, as measured by the Hollingshead Four Factor Index of Social Status (Hollingshead, 1975) was middle class. Twelve percent of subjects were enrolled in Special Education services. No significant differences existed between groups on any of the demographic variables, except age. Members of the TGA

Discussion

Overall, this small sample of preschool-aged children with CHD appears to be doing quite well from a neurodevelopmental standpoint. Most children were of average intelligence and did not differ significantly from population norms of intellectual functioning, which is in contrast to previous research that showed IQ scores in the low average range (Bellinger et al 1999, Griffin et al 2003). IQ results may differ due to the different instruments used, the different ages of subjects assessed,

Cheryl L. Brosig, Medical College of Wisconsin, Department of Pediatrics, Milwaukee, Wis.

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Children with hypoplastic left heart syndrome are particularly at risk of neurodevelopmental abnormalities including development delay and attention deficit hyperactivity disorder (Donofrio and Massaro, 2010; de Los Reyes and Roach, 2014). When compared to patients with transposition of the great arteries, patients with hypoplastic left heart syndrome have more problems with visual-motor skills, expressive language, attention, and externalizing behaviors (Brosig et al., 2007). Another study showed that neurodevelopment was better in children with transposition of the great arteries than in those with tetralogy of Fallot (Bellinger et al., 2001).
Improved medical management and surgical outcomes have significantly decreased mortality in children with congenital heart disease; however, with increased survival, there is a greater lifetime exposure to neurologic complications with serious long-term neurodevelopmental consequences. Thus, recent focus has shifted to recognition and reduction of these extracardiac comorbidities. Vascular and infective complications, such as arterial ischemic stroke, infective endocarditis, and localization-related epilepsy are some of the most common neurologic comorbidities of congenital heart disease. In addition, it is now well recognized that congenital heart disease has an impact on overall brain development and contributes to adverse neurodevelopmental outcomes across multiple domains. The goal of this chapter is to summarize the most common neurologic comorbidities of congenital heart disease and its management.
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A focused review was performed of all articles in English that address the topic from 1980 to 2018.
Innate patient factors, rather than intraoperative factors, affect the development of neurodevelopmental outcomes, yet a large portion of causality factors remains unexplained. There are also limits to how we currently measure neurodevelopmental outcomes. Limited studies in adulthood and a lack of longitudinal studies hamper our full understanding of how neurodevelopment progresses across time, but estimates are that adults with neurodevelopmental impairments will be affected by abnormal brain aging and early-onset dementia. Intervention services and their efficacy have not been researched in depth and have not been researched across the lifespan.
Further research into neurodevelopmental testing that accurately predicts future functioning is needed. The complexity of implementing systematic and effective neurodevelopmental interventions in the busy lives of patients and families, who often have multiple other clinical demands, needs a complex and thoughtful solution within the context of a multidisciplinary care team approach. The role of targeted psychological and educational interventions that decrease maternal worry and improve family functioning should also continue to be explored in patients with a Fontan circulation.
Neurodevelopmental outcomes of children with congenital heart disease: A review
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Neurodevelopmental disability is the most common and impactful consequence of CHD. Research has consistently pointed to general vulnerabilities with regard to visual motor skills, expressive language abilities, academic trajectories and executive functioning skills.139–141 Additionally, behavioral, quality of life and emotional issues have been found to be prevalent and in need of continued monitoring and intervention.62,139,142,143
Congenital heart defects are the most common birth anomaly affecting approximately 1% of births. With improved survival in this population, there is enhanced ability to assess long-term morbidities including neurodevelopment. There is a wide range of congenital heart defects, from those with minimal physiologic consequence that do not require medical or surgical intervention, to complex structural anomalies requiring highly specialized medical management and intricate surgical repair or palliation. The impact of congenital heart disease on neurodevelopment is multifactorial. Susceptibility for adverse neurodevelopment increases with advancing severity of the defect with initial risk factors originating during gestation. Complex structural heart anomalies may pre-dispose the fetus to abnormal circulatory patterns in utero that ultimately impact delivery of oxygen rich blood to the fetal brain. Thus, the brain of a neonate born with complex congenital heart disease may be particularly vulnerable from the outset. That vulnerability is compounded during the newborn period and through childhood, as this population endures a myriad of medical and surgical interventions. For each individual patient, these factors are likely cumulative and synergistic with progression from fetal life through childhood. This review discusses the spectrum of risk factors that may impact neurodevelopment in children with congenital heart disease, describes current recommendations and practices for neurodevelopmental follow-up of children with congenital heart disease and reviews important neurodevelopmental trends in this high risk population.
Need for Routine Screening of Health-Related Quality of Life in Families of Young Children with Complex Congenital Heart Disease
2019, Journal of Pediatrics
To assess health-related quality of life (HRQOL) in families of young children with complex congenital heart disease (CHD), and identify the demographic, clinical, and psychosocial factors that place these children and their mothers at greater risk of vulnerability.
This cross-sectional study took place from June 2015 to October 2016 at The Sydney Children's Hospitals Network Cardiac Service, Australia. Mothers of a child aged 1-5 years with either single ventricle CHD or CHD requiring neonatal biventricular repair were invited to participate. Eighty-seven mothers completed a suite of validated measures, including the Pediatric Quality of Life Inventory, which assessed the outcomes of child and maternal HRQOL.
Sixty percent of children with single ventricle CHD and 25% of children with biventricular repair had total Pediatric Quality of Life Inventory scores within the at-risk range. Lower child HRQOL was strongly associated with single ventricle CHD (β = −0.38; P < .001), physical comorbidity (β = −0.32; P = .001), feeding difficulties (β = −0.26; P = .008), and greater maternal psychological stress (β = −0.18; P = .045), accounting for 52% of the variance in child HRQOL. Lower maternal HRQOL was strongly associated with poorer family functioning (β = 0.61; P < .001), greater maternal psychological stress (β = −0.23; P = .004), child physical comorbidity (β = −0.17; P = .01), and a ‘difficult’ child temperament (β = −0.14; P = .01), accounting for 73% of the variance in maternal HRQOL.
Lower HRQOL is common in young children with complex CHD, particularly single ventricle CHD. Several predictors of HRQOL are potentially modifiable, offering possible pathways for prevention and early intervention. Routine screening is a necessary first step toward developing models of care to improve HRQOL in this population.
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Due to increasing incidence and improved survival, greater numbers of children with congenital heart disease (CHD) are surviving to their teenage years and beyond. This patient group have complex medical needs that interact with the emotional and developmental challenges of adolescence. Teenagers with CHD are at increased risk of both neurodevelopmental and emotional disorders. Quality of life is also significantly impaired in this group, and strategies should be considered to maximise this and other holistic outcomes. As teenage patients become adults, their care is transitioned to adult services, a potentially stressful time for these patients. It is important that this process is well understood, and managed in a streamlined and organised manner to minimise numbers lost to follow-up. It is at this stage that patient educational information, probably previously given to parents or carers, is reiterated to the patient themselves. This should include advice around the patient's specific lesion, as well as general advice about exercise tolerance, endocarditis risk, and pregnancy.

View all citing articles on Scopus

Cheryl L. Brosig, Medical College of Wisconsin, Department of Pediatrics, Milwaukee, Wis.

Kathleen A. Mussatto, Children’s Hospital of Wisconsin, Herma Heart Center, Milwaukee, Wis.

Evelyn M. Kuhn, Children’s Hospital and Health System, National Outcomes Center, Inc, Milwaukee, Wis.

James S. Tweddell, Medical College of Wisconsin, Department of Surgery, Milwaukee, Wis.

This study was supported by the Julie Lathrop Nursing Research Award from the Children’s Hospital Foundation.

Reprints are not available from author.

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Original articleNeurodevelopmental Outcome in Preschool Survivors of Complex Congenital Heart Disease: Implications for Clinical Practice

Abstract

Introduction

Methods

Results

Discussion

Section snippets

Patient Population

Demographics

Discussion

American Journal of Surgery

Journal of Thoracic and Cardiovascular Surgery

Journal of Pediatrics

Journal of Thoracic and Cardiovascular Surgery

Journal of Pediatrics

Child Behavior Checklist for Ages 1½-5

Cognitive and neuropsychological outcomes: More than just IQ scores

MRRD Research Reviews

Visual-spatial skills in children after open-heart surgery

Journal of Developmental and Behavioral Pediatrics

Developmental and neurological status of children at 4 years of age after heart surgery with hypothermic circulatory arrest or low-flow cardiopulmonary bypass

Circulation

Statistical power analysis for the behavioral sciences

Original article
Neurodevelopmental Outcome in Preschool Survivors of Complex Congenital Heart Disease: Implications for Clinical Practice