Diagnosis and Intervention Strategies to Address Neurodevelopmental and Psychosocial Challenges in School‐Aged Children With Congenital Heart Disease

In 2012, the American Heart Association (AHA) published the first Scientific Statement addressing the evaluation and management of neurodevelopmental outcomes in children with congenital heart disease (CHD), which was approved by the American Academy of Pediatrics. ¹ It found that children with CHD are at increased risk for neurodevelopmental disorders and disabilities. A management algorithm was devised that stratified children with CHD on the basis of established risk factors, such as genetic syndrome and/or predisposition, fetal and perinatal factors, brain volumes, and surgical and perioperative factors, although all together these risk factors explain <50% of the variation in neurodevelopmental outcomes. ² Subsequent touchpoints for surveillance, evaluation, and management were recommended, and neurodevelopmental evaluation and reevaluation were characterized as essential steps to obtain appropriate interventions and optimize overall development for children with CHD. It also identified that further research is needed to refine high‐risk criteria, study the effectiveness of interventions, and identify funding and reimbursement mechanisms to support the provision of neurodevelopmental evaluations and interventions.

In 2018, the Cardiac Neurodevelopmental Outcome Collaborative (CNOC), an international organization comprising multidisciplinary professionals working to improve neurodevelopmental and psychosocial outcomes for individuals with CHD, ³ convened working groups with the support of an R13 grant from the National Heart, Lung, and Blood Institute. Goals included to better delineate the current understanding of neurodevelopmental and psychosocial care of children with CHD and propose future directions for research. One working group set out to identify knowledge gaps with respect to the neurodevelopmental and psychosocial outcomes of patients with CHD. ⁴ It suggested that future investigations need to shift towards improving methods, measurement, and analyses of outcomes in specific CHD populations as well as identification of clinically meaningful outcomes for key stakeholders. Another working group provided an overview of the current neurodevelopmental and psychosocial intervention research findings and recommended broadening interventions to address self‐regulatory, cognitive, and social–emotional challenges to address these difficulties in individuals with CHD. ⁵

While initial CHD outcomes studies focused primarily on medical comorbidities, ⁶ recent research has further identified an array of developmental difficulties in individuals with CHD across the lifespan. The developmental phenotype in CHD often begins with significant gross motor delays, as well as relatively milder speech/language and feeding/growth delays. These difficulties evolve over time to include neurocognitive and psychosocial challenges in the older, school‐aged child. ⁷ Executive functioning challenges persist across the lifespan and increasingly impact adaptive and psychosocial functioning in young adulthood and beyond. ⁸ It is therefore important to look beyond global intellectual quotient measures to include areas of specific neurocognitive domains that may be impacted for the child with CHD, even when intellectual quotient is relatively preserved.

Another recent publication from the CNOC provides an expanded set of neurodevelopmental evaluation strategies and considerations for clinicians working with school‐aged children and adolescents. ⁹ This approach includes not only medical risk factors but also takes into account both family and patient factors (eg, developmental levels, primary language, and cultural norms). The evaluation includes a core battery assessing the domains of intelligence, academic achievement, attention, executive functioning, memory, fine motor skills, and social, emotional, behavioral, and adaptive functioning, as well as an extended comprehensive clinical battery. Establishing best practices for neurodevelopmental assessment serves to harmonize data elements for future multisite clinical research collaborations and supports the ultimate goal of improving the quality of neurodevelopmental care for children with CHD and their families.

Research updates in the past decade have improved identification of individuals at high risk for neurodevelopmental delay or disability and have highlighted factors that increase that risk. Unfortunately, we continue to know more about the problems associated with CHD than we have interventions to help mitigate them. In 2022, the AHA published a Scientific Statement summarizing the psychological challenges that exist across the lifespan for patients with CHD, with psychiatric disorders representing the most common morbidity. ⁸ This statement highlighted that data from psychotherapeutic, educational, and pharmacological intervention studies with this population were limited, but promising. It recommended the integration of mental health professionals within CHD teams to optimize patient quality of life. In March 2024, the AHA published an updated Scientific Statement on neurodevelopmental outcomes for individuals with CHD, reviewing the research and advanced scientific knowledge of developmental trajectories and predictors of risk and resilience. ¹⁰ An updated list of factors that may lead to neurodevelopmental delay included those mentioned in the 2012 statement, as well as social determinants of health and parental psychological distress. It emphasized the need to focus efforts to prevent and mitigate neurodevelopmental delays and disorders, including best practices for neuroprotection at different points along the continuum of care, as well as updated recommendations for evaluation and management.

DIAGNOSTIC APPROACHES

School‐aged children and adolescents with CHD are at increased risk for a wide range of neurodevelopmental and psychosocial difficulties. Challenges with attention and executive functioning, processing speed, and visual‐motor skills are most commonly noted. ⁷ These, in turn, lead to difficulties with academic learning, including a higher rate of specific learning disorders and, more broadly, academic underachievement. Diagnoses of attention‐deficit/hyperactivity disorder, autism spectrum disorder, anxiety, and depression are also more common in children with CHD compared with the general population. ⁷ Taken together, these challenges result in decreased vocational and educational attainment in adulthood, and reduced levels of functional independence. ⁸ That said, other children and adolescents with CHD do well from neurodevelopmental and psychosocial perspectives, and more research on resiliency and protective factors is needed.

Access to neurodevelopmental and psychosocial care remains a challenge for most children and adolescents with CHD. While many tertiary children's hospitals have developed a cardiac neurodevelopmental program, ¹¹ even highly resourced centers may have only 1 or 2 pediatric psychologists or neuropsychologists providing diagnostic evaluations for children with CHD (CNOC leadership, personal communication). Outside of the United States and Canada, access to multidisciplinary cardiac care teams including pediatric psychologists and neuropsychologists is even more scarce, even in developed countries, ¹² highlighting a need to address clinical resource gaps on a global level. Increased staffing is not likely to occur quickly given that nationwide there are only 5.4 clinical child and adolescent psychologists per 100 000 children younger than 18 years. ¹³ When services are available, lengthy waitlists (eg, ≈1 year) are common and patients/families may experience barriers to attending these multihour appointments such as costs associated with travel, time away from work/school, lack of insurance coverage for neurodevelopmental evaluations, and childcare for siblings. Importantly, these barriers may disproportionately impact families experiencing social disadvantage, which is a crucial barrier to overcome as social determinants of health are consistent predictors of poorer neurodevelopmental outcomes, ¹⁴ resulting in reduced access to care for the children with CHD who need it the most. A recent CNOC publication highlighted a daunting statistic that, among CNOC centers, only 29% of young children meeting AHA high‐risk criteria attended a neurodevelopmental evaluation before 3 years of age. ¹⁵ School‐age neurodevelopmental evaluations are often costlier and more time‐consuming than infant/toddler evaluations, but the rate of return in the school‐aged CHD population is unknown.

Given high interindividual variability in neurodevelopmental and psychosocial outcomes, and the time‐ and resource‐intensive nature of comprehensive assessment, a preventative approach to neurodevelopmental and psychosocial evaluation and management ¹⁶ , ¹⁷ may serve to improve access to care and benefit both patients and families, as well as care centers (Figure). Such an approach may include practical recommendations for neurodevelopmental and psychosocial monitoring, screening, and triaging that can provide timely, focused consultation and brief intervention, as well as assess the need for and optimal timing of formal neurodevelopmental or psychosocial evaluation or care. ¹⁷ Establishing multidisciplinary care teams as standard of care at tertiary pediatric heart centers and building collaborative partnerships with rehabilitation specialists and community resources may serve as additional, practical next steps towards optimizing outcomes. Asking questions about emotional health as part of routine cardiac visits will also help reduce stigma associated with seeking mental health care. The American Board of Pediatrics, in its Roadmap Initiative, aims to improve the resilience and emotional health of children with chronic illness and their families by taking a preventative approach, encouraging medical providers to begin conversations about emotional health with patients and families early, and to address this topic at most visits. ¹⁶ Similarly, the US Preventative Services Task Force has recommended routine screening for anxiety, depression, and suicide risk in children and adolescents, with a goal of early detection and connection to care. ¹⁸

Figure 1. Strategies for optimizing neurodevelopmental and psychosocial care for school‐aged children and adolescents with congenital heart disease.

During the COVID‐19 pandemic, the majority of CNOC programs began leveraging telehealth for various aspects of cardiac neurodevelopmental and psychosocial care, including diagnostic intake interviews, feedback discussions with families, selected neurodevelopmental testing, and psychological interventions. ¹⁹ Telehealth was noted to potentially address many barriers to accessing neurodevelopmental care for patients with CHD, particularly those with social disadvantages, and high satisfaction was reported for telehealth use among patients and families, as well as cardiac neurodevelopmental providers. Conducting standardized neurodevelopmental testing via telehealth was noted to be relatively more challenging; however, as only a subset of CNOC‐recommended tests was feasible to administer via telehealth (primarily language‐mediated tasks, such as verbal comprehension, literacy, verbal memory, and verbal fluency tasks), and concerns regarding the validity of test results were noted. Future research is needed to adapt more neurodevelopmental testing procedures for telehealth administration and to provide best practices for ensuring test validity.

INTERVENTION RECOMMENDATIONS

While many publications discuss diagnostic assessment strategies for school‐aged children with CHD, data from studies investigating specific neurodevelopmental, academic, or psychosocial interventions in this population are limited. ⁸ Despite the increased prevalence of attention challenges and attention‐deficit/hyperactivity disorder diagnosis in children and adolescents with CHD, no prospective trials have assessed the efficacy of attention medication such as stimulants in the CHD population, although retrospective cohort studies support safety and tolerability. ²⁰ Computerized cognitive remediation programs (eg, © Cogmed) have shown moderate success in improving several aspects of executive functioning for children with CHD but require high time commitments from patients and families and have limited generalizability to academic or functional outcomes. ²¹ Research on the efficacy and tolerability of psychiatric medication for individuals with CHD is also scarce.

Individuals with CHD who are currently experiencing neurodevelopmental or psychosocial challenges should not have to wait for future research to quantify the efficacy of interventions specific to CHD. Professionals who care for children with CHD may draw from extensive research in the fields of developmental and clinical psychology to facilitate timely intervention in order to optimize their patients' neurodevelopmental and psychosocial trajectories. For example, children with CHD who are struggling to read can be referred to an evidence‐based program for children with dyslexia, while those struggling with handwriting and fine motor fatigue can be referred to occupational therapy. Incorporating psychosocial screening and embedding psychologists in outpatient cardiology clinics may help identify children who are most at risk and in need of psychological intervention. ²² Once identified, children who are experiencing anxiety, depression, or posttraumatic stress disorder can be referred for cognitive behavioral therapy and, when appropriate, psychiatric medication consultation. Those who may be struggling with self‐esteem or social issues may benefit by participating in online chat/support groups, camps, or peer‐mentoring programs specific for patients with CHD. Outpatient cardiac rehabilitation programs may not only help to improve exercise capacity but may also have a positive impact on self‐concept, emotional functioning, and quality of life. ²³ Multidisciplinary care teams for children with CHD can serve to facilitate these referrals, as pediatric cardiologists may not always have the awareness of when or how to access these interventions for children with CHD.

Inpatient neurodevelopmental care interventions have received relatively more attention, including a recent science advisory from the AHA delineating the importance of patient‐ and family‐centered care practices. ²⁴ This literature has almost exclusively focused on infants and young children with CHD, however, and the neurodevelopmental and psychosocial needs of hospitalized school‐aged children with CHD have yet to be directly addressed. While most initial CHD surgeries take place early in life, older children may be admitted to the hospital for a variety of reasons including valve replacements, diagnostic catheterizations, or to await a heart transplant while on ventricular assist device support. These children may benefit from neurodevelopmental and psychosocial care as well, including optimization of sleep/wake cycles in order to reduce delirium and improve mood; cardiac rehabilitation/early mobility protocols, which may yield cognitive and motor benefits; inpatient school services to help minimize gaps in academic learning; and consultation with mental health providers who are part of the care team to promote coping and adjustment during the inpatient hospital stay.

CONCLUSIONS

The past 2 decades have seen incredible growth in research describing the causes and trajectories of neurodevelopmental and psychosocial outcomes for school‐aged children with CHD, as well as establishment of best practices for neurodevelopmental and psychosocial care. That said, several gaps remain with respect to the practical implementation of optimal neurodevelopmental and psychosocial care for school‐aged children and adolescents with CHD. First, barriers to accessing neurodevelopmental and psychological care must be addressed. Advocacy and policy efforts must work toward establishing neurodevelopmental and psychosocial care as essential rights for school‐aged children with CHD regardless of insurance coverage or family's ability to pay. These efforts should also include lobbying for increased reimbursement for these services, as well as increased funding for schools to provide the special education services that these children need. Pediatric cardiac centers and professionals, in turn, may work to incorporate preventative practices of neurodevelopmental and psychosocial screening and triage to ensure “right‐sized” care for each individual with CHD, within an optimal timeframe. Technology for improving access and advancing care should be incorporated, such as utilizing telehealth whenever feasible.

The time is now for leveraging empirically supported neurodevelopmental and psychosocial interventions for school‐aged children and adolescents with CHD. While clinical trials are certainly needed to advance the field and inform personalized medicine opportunities for developmental therapeutics, children with CHD and their families should be provided with timely resources and access to established developmental, psychological and behavioral therapies, medication trials (as appropriate), and rigorous academic remediation programs that have been shown in the general population to optimize outcomes.

Finally, it is critical that the voices of children with CHD and their families are heard to ensure that neurodevelopmental and psychosocial evaluations and interventions are accessible, culturally sensitive, and meaningful to them. Enhanced partnerships with community stakeholders such as schools will be needed, ¹⁰ as children spend the majority of their time outside of the tertiary medical setting. It is only through close collaboration among multidisciplinary clinical professionals, educators, policymakers, community service providers, and individuals with CHD and their families that all children and adolescents with CHD will be able to achieve their potential and optimize their quality of life.

Disclosures

None.