Language brain responses and neurodevelopmental outcome in preschoolers with congenital heart disease: A fNIRS study.

Neurodevelopmental disabilities affect up to 50% of survivors of congenital heart disease (CHD). Language difficulties are frequently identified during preschool period and can lead to academic, social, behavioral, and emotional difficulties. Structural brain alterations are associated with poorer neurodevelopmental outcomes in patients with CHD during infancy, childhood, and adolescence. However, evidence is lacking about the functional brain activity in children with CHD and its relationship with neurodevelopment. This study therefore aimed to characterize brain responses during a passive story-listening task in 3-year-old children with CHD, and to investigate the relationship between functional brain patterns of language processing and neurodevelopmental outcomes. To do so, we assessed hemodynamic concentration changes, using functional near-infrared spectroscopy (fNIRS), and neurodevelopmental outcomes, using the Wechsler Preschool and Primary Scale of Intelligence - 4th Edition (WPPSI-IV), in children with CHD (n = 19) and healthy controls (n = 23). Compared to their healthy peers, children with CHD had significantly lower scores on the Verbal comprehension index (VCI), the Vocabulary acquisition index (VAI), the General ability index (GAI), and the Information and the Picture Naming subtests of the WPPSI-IV. During the passive story-listening task, healthy controls showed significant hemodynamic brain responses in the temporal and the temporal posterior regions, with stronger activation in the temporal posterior than in the temporal regions. In contrast, children with CHD showed reduced activation in the temporal posterior regions compared to controls, with no difference of activation between regions. Reduced brain responses in the temporal posterior regions were also correlated with lower neurodevelopmental outcomes in both groups. This is the first study that reveals reduced brain functional responses in preschoolers with CHD during a receptive language task. It also suggests that the temporal posterior activation could be a potential brain marker of cognitive development. These findings provide support for the feasibility of identifying brain correlates of neurodevelopmental vulnerabilities in children with CHD.

A Parent-child yoga intervention for reducing attention deficits in children with congenital heart disease: the Yoga for Little Hearts Feasibility Study Protocol.

INTRODUCTION: Preschoolers and school-aged children with congenital heart disease (CHD) are at higher risk of attention deficit hyperactivity disorder (ADHD) compared with the general population. To this day, no randomised controlled trial (RCT) aiming to improve attention has been conducted in young children with CHD. There is emerging evidence indicating that parent-child yoga interventions improve attention and reduce ADHD symptoms in both typically developing and clinical populations. METHODS AND ANALYSIS: This is a single-blind, two-centre, two-arm trial during which 24 children with CHD and their parents will be randomly assigned to (1) a parent-child yoga intervention in addition to standard clinical care or (2) standard clinical care alone. All participants will undergo standardised assessments: (1) at baseline, (2) immediately post-treatment and (3) 6 months post-treatment. Descriptive statistics will be used to estimate the feasibility and neurodevelopmental outcomes. This feasibility study will evaluate: (1) recruitment capacity; (2) retention, drop-out and withdrawal rates during the yoga programme and at the 6-month follow-up; (3) adherence to the intervention; (4) acceptability of the randomisation process by families; (5) heterogeneity in the delivery of the intervention between instructors and use of home-based exercises between participants; (6) proportion of missing data in the neurodevelopmental assessments and (7) SD of primary outcomes of the full RCT in order to determine the future appropriate sample size. ETHICS AND DISSEMINATION: Ethical approval has been obtained by the Research Ethics Board of the Sainte-Justine University Hospital. The findings will be disseminated in peer-reviewed journals and conferences and presented to the Canadian paediatric grand round meetings. TRIAL REGISTRATION NUMBER: NCT05997680.

Relationship between 4-month functional brain network topology and 24-month neurodevelopmental outcome in children with congenital heart disease.

Survivors of complex forms of congenital heart disease (CHD)∗ are at high risk of neurodevelopmental disabilities. Neuroimaging studies have pointed to brain anomalies and immature networks in infants with CHD, yet less is known about their functional network topology and associations with neurodevelopment. To characterize the functional network topology in 4-month-old infants with repaired CHD, we compared graph theory metrics measured using resting-state functional near-infrared spectroscopy (rs-fNIRS) between infants with CHD (n = 22) and healthy controls (n = 30). We also investigated the moderating effect of graph theory metrics on the relationship between group (CHD vs. Controls) and developmental outcomes at 24 months. At 4 months, both groups presented similar functional brain network topology. At 24 months, children with CHD had lower scores on the language scale and the expressive communication subscale of the Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III), as well as lower scores on the Grammatical Form scale of the MacArthur-Bates Communicative Development Inventory (MBCDI). The relationship between group and expressive language was moderated by the normalized characteristic path length (λ) and the degree (k). Although infants with CHD have functional brain topology similar to that of healthy controls, our findings suggest that they do not benefit from an optimal functional brain organization in comparison with healthy infants.

Functional brain connectivity after corrective cardiac surgery for critical congenital heart disease: a preliminary near-infrared spectroscopy (NIRS) report.

Patients with congenital heart disease (CHD) requiring cardiac surgery in infancy are at high risk for neurodevelopmental impairments. Neonatal imaging studies have reported disruptions of brain functional organization before surgery. Yet, the extent to which functional network alterations are present after cardiac repair remains unexplored. This preliminary study aimed at investigating cortical functional connectivity in 4-month-old infants with repaired CHD, using resting-state functional near-infrared spectroscopy (fNIRS). After fNIRS signal frequency decomposition, we compared values of magnitude-squared coherence as a measure of connectivity strength, between 21 infants with corrected CHD and 31 healthy controls. We identified a subset of connections with differences between groups at an uncorrected statistical level of p < .05 while controlling for sex and maternal socioeconomic status, with most of these connections showing reduced connectivity in infants with CHD. Although none of these differences reach statistical significance after FDR correction, likely due to the small sample size, moderate to large effect sizes were found for group-differences. If replicated, these results would therefore suggest preliminary evidence that alterations of brain functional connectivity are present in the months after cardiac surgery. Additional studies involving larger sample size are needed to replicate our data, and comparisons between pre- and postoperative findings would allow to further delineate alterations of functional brain connectivity in this population.

Social cognition and competence in preschoolers with congenital heart disease.

OBJECTIVE: Children born with congenital heart disease (CHD) are at an increased risk for various neurodevelopmental impairments. However, little is known regarding social outcomes associated with CHD, particularly during early childhood. The present study aimed to characterize the sociocognitive profile and to assess the contribution of language, executive functions (EF), and social cognition to social competence (SC) in preschoolers with CHD. METHOD: Five-year-old children with CHD (n = 55) completed a standardized neuropsychological assessment. Performance on sociocognitive skills was compared to test norms using one-sample t tests. Hierarchical regression was conducted to examine the associations between language skills, affect recognition (AR), theory of mind (ToM), EF (performance-based and parent-rated), and social competence. RESULTS: Children with CHD performed significantly worse than norms in language and ToM, whereas EF and social competence appeared generally preserved in our sample. In hierarchical regression analysis, cognitive functions (language score, AR, ToM, EF performance) accounted for a significant 24.3% of the variance. Parent-rated EF added another 24.8% to the total explained variance. CONCLUSIONS: These findings provide new evidence for understanding social cognition and competence among preschoolers with CHD, showing vulnerability in social cognition and language skills but not in social competence more generally. The model suggests a combined contribution of social cognition, language, and EF on social outcomes. Remedial programs addressing these intervention targets could be useful in promoting social development in this vulnerable population. (PsycInfo Database Record (c) 2022 APA, all rights reserved).

Utility of the Ages and Stages Questionnaires 3rd Edition for Developmental Screening in Children with Surgically Repaired Congenital Heart Disease.

Aim: This study sought to evaluate the accuracy of the Ages and Stages Questionnaires 3rd Edition (ASQ-3) in identifying developmental delay (DD) in children with congenital heart disease (CHD) born at term who underwent surgical repair.Methods: Participants had to complete ASQ-3 and Bayley Scales of Infant and Toddler Development 3rd Edition (BSID-III) at 12 and 24 months. A child was considered at risk of DD for a ASQ-3 domain when he scored below the cutoff (≤-1SD or ≤-2SD). A child had a DD in a BSID-III domain when the score was ≤-1SD. The validity for each ASQ-3 domain and for overall ASQ-3 was measured.Results: At 12 months (n = 64), overall ASQ-3 (≤-2SD) sensitivity was 88%, specificity 74%. At 24 months (n = 82), overall ASQ-3 (≤-2SD) sensitivity was 74%, specificity 88%.Conclusion: The results support the utility of the ASQ-3 for screening the overall risk of DD in children with CHD.

The role of parenting stress in anxiety and sleep outcomes in toddlers with congenital heart disease.

Objectives: This retrospective cohort study investigates how parenting stress, measured at 4 months of age by use of a classic three-dimensional parent-reported scale (Parenting Stress Index, 4th Ed. or PSI-4), can predict anxiety symptoms and quality of sleep at 24 months in toddlers with congenital heart disease (CHD). Study Design: Sixty-six toddlers with CHD followed at our cardiac neurodevelopmental follow-up clinic were included in this study. As part of their systematic developmental assessment program, parents completed questionnaires on their stress level (PSI-4) when their child was 4 months old, and on their child's anxiety symptoms and quality of sleep at 24 months. Eight multiple linear regression models were built on the two measures collected at 24 months using the PSI-4 scores collected at 4 months. For each measure, four models were built from the PSI-4 total score and its three subscales (Parental Distress, Parent-Child Dysfunctional Interaction, Difficult Child), controlling for sex and socioeconomic status. Results: The PSI-4 Difficult Child subscale, which focuses on parenting anxiety related to the child's behavioral problems and poor psychosocial adjustment, accounted for 17% of the child's anxiety symptoms at 24 months. The two other PSI-4 subscales (Parental Distress and Parent-Child Dysfunctional Interaction) and the PSI-4 total score did not contribute significantly to the models. None of the four regression models on perceived quality of sleep were significant. It is important to note that 33% of parents responded defensively to the PSI-4. Conclusions: Parenting stress related to the child's behavioral problems and poor psychosocial adjustment, measured when the child is 4 months old, is associated with the child's ulterior anxiety symptoms. As very few standardized tools are available to assess the behavioral and psychoaffective development of infants, this study highlights the importance of early psychosocial screening in parents of infants with CHD. The high rate of significant Defensive Responding Indices reminds us to not take parent reports at face value, as their actual stress levels might be higher.

Neurodevelopmental Outcome of Children with Congenital Heart Disease: A Cohort Study from Infancy to Preschool Age.

OBJECTIVE: To characterize the neuropsychological outcome of children with congenital heart disease (CHD) at age 5 years; the stability of cognitive and language abilities across childhood; and to identify early neurodevelopmental markers of neuropsychological outcomes in these children. STUDY DESIGN: Five-year-old children (n = 55) with complex CHD were assessed using standardized and comprehensive neuropsychological measures. Stability of language and cognitive performance was assessed by comparing standardized scores between ages 1, 2, and 5 years old. Association between 5-year-old skills and scores obtained in early childhood was studied to identify potential early markers of preschool performance. Receiver operating characteristic curves were used to evaluate the classification accuracy of Bayley Scales of Infant Development, Third Edition scales in identifying later impairments. RESULTS: At age 5 years, our cohort obtained scores significantly below the norms on most developmental domains, with 35% to 65% of participants showing impaired short-term/working memory, attention, and preacademic skills. Developmental patterns measured between ages 1 and 5 years were different for cognitive and language domains, with a decline with age for cognitive functioning and stable results for expressive language. The Bayley Scales of Infant Development, Third Edition language scores at age 2 years provided a good predictive value in identifying children with impaired language at age 5 years. CONCLUSIONS: In our cohort, we found a high prevalence of impairments affecting higher-order cognitive domains. Although language difficulties can be detected as early as 2 years of age, other neuropsychological impairments, such as attention and pre-academic skills, only appear later during development, which reinforces the need for long-term monitoring and systematic assessment before school entry.

Gross Motor Development of Children with Congenital Heart Disease Receiving Early Systematic Surveillance and Individualized Intervention: Brief Report.

Purpose: This retrospective study aims to describe the gross motor development of children aged 4 to 24 months with congenital heart disease (CHD) enrolled in a systematic developmental follow-up program and to describe the frequency of physical therapy sessions they received between 4 and 8 months of age. Methods: Twenty-nine infants with CHD underwent motor evaluations using the AIMS at 4 months, and the Bayley-III at 12 and 24 months. Results: Based on AIMS, 79% of 4-month-old infants had a gross motor delay and required physical therapy. Among these, 56.5% received one to two physical therapy sessions, and 43.5% received three to six sessions. Infants who benefited from regular interventions tended to show a better improvement in motor scores from 12 to 24 months. Conclusion: This study highlights the importance of early motor screening in infants with CHD and suggests a potential benefit of early physical therapy in at-risk children. Abbreviations: CHD: Congenital heart disease; AIMS: Alberta Infant Motor Scales; Bayley-III: Bayley Scales of Infant and Toddler Development, Third edition; Bayley-III/GM: Gross Motor section of the Bayley Scales of Infant and Toddler Development, Third edition.

Impacts of an Interdisciplinary Developmental Follow-Up Program on Neurodevelopment in Congenital Heart Disease: The CINC Study.

Objectives: This study investigates the impact of an early systematic interdisciplinary developmental follow-up and individualized intervention program on the neurodevelopment of children with complex congenital heart disease (CHD) who required cardiac surgery. Study Design: We prospectively enrolled 80 children with CHD: 41 were already followed at our neurocardiac developmental follow-up clinic from the age of 4 months, while 39 were born before the establishment of the program and therefore received standard health care. We conducted cognitive, motor, and behavioral assessments at 3 years of age. We used one-way multivariate analyses of variance to compare the neurodevelopmental outcome of both groups. Results: Between-group analyses revealed a distinct neurodevelopmental profile with clinically significant effect size (P < 0.001, partial η2 = 0.366). Children followed at our clinic demonstrated better receptive language performances (P = 0.048) and tended to show higher scores on visuo-constructive tasks (P = 0.080). Children who received standard health care exhibited greater performances in working memory tasks (P = 0.032). We found no group differences on global intellectual functioning, gross and fine motor skills, and behaviors. Referral rates for specific remedial services were higher in patients followed at our neurocardiac clinic compared to the historical cohort (P < 0.005). Conclusions: Overall, the impact of the developmental follow-up and individualized intervention program on neurodevelopmental outcomes remains subtle. Nevertheless, results, although limited by several factors, point toward an advantage for the children who took part in the program regarding receptive language skills over children who received standard health care. We hypothesize that group differences may be greater with growing age. Further research involving larger cohorts is needed to clearly assess the effectiveness of neurocardiac developmental follow-up programs at school age.

Epilepsy and seizures in children with congenital heart disease: A prospective study.

PURPOSE: Children with complex congenital heart disease (CHD) experience high incidence of perioperative seizures. Population-based studies also report high epilepsy co-morbidity in CHD. Given the increasing survival of patients with CHD and the interference of seizures and epilepsy with the long-term outcomes, characterizing them in this population is of high relevance. This study investigated the incidence and risk factors of perioperative clinical seizures (CS) and epilepsy in a prospective cohort of children with complex CHD who underwent cardiac surgery. METHODS: We included 128 consecutive children with CHD, followed for at least two years at the neurocardiac clinic of Montreal's Sainte-Justine University Hospital Center. We collected perinatal, surgical, critical care and clinical follow-up information and performed logistic regression to reveal risk factors of CS and epilepsy. RESULTS: Ten patients (7.8%) experienced perioperative CS. Four of them (40%) developed epilepsy. The incidence of epilepsy was therefore 3.1%. Higher surgical complexity scores, delayed sternal closure, extracorporeal membrane oxygenation (ECMO) use, longer intensive care and hospital stay were associated with CS. ECMO use and hospital stay were also associated with epilepsy. Nine (90%) patients with CS had brain injuries: five strokes, one white matter and three hypoxic-ischemic injury (HII). All patients with HII developed epilepsy, which became intractable in one of them. CONCLUSION: Our study reports high incidence, surgical risk factors and brain injury patterns underlying CS and epilepsy in CHD. Further studies are needed to investigate how epilepsy interferes with neurodevelopment and quality of life in CHD.

Superior Performance in Prone in Infants With Congenital Heart Disease Predicts an Earlier Onset of Walking.

Infants with congenital heart disease are at risk of impaired neurodevelopment, which frequently manifests as motor delay during their first years of life. This delay is multifactorial in origin and environmental factors, such as a limited experience in prone, may play a role. In this study, we evaluated the motor development of a prospective cohort of 71 infants (37 males) with congenital heart disease at 4 months of age using the Alberta Infant Motor Scales (AIMS). We used regression analyses to determine whether the 4-month AIMS scores predict the ability to walk by 18 months. The influence of demographic and clinical variables was also assessed. Fifty-one infants (71.8%) were able to maintain the prone prop position (AIMS score of ≥3 in prone) at 4 months. Of those, 47 (92.2%) were able to walk by 18 months compared to only 12/20 (60%) of those who did not maintain the position. Higher AIMS scores were predictive of a greater likelihood of walking by 18 months ( P < .001), with the scores in prone having a higher predictive ability compared to those in other positions (Exp(B) 15.2 vs 4.0). Shorter hospital stays and female gender were also associated with an earlier onset of walking. In conclusion, our study demonstrates that early ventral performance in infants with congenital heart disease impacts the age of acquisition of walking and could be used to guide referral to rehabilitation.

Significant motor improvement in an infant with congenital heart disease and a rolandic stroke: The impact of early intervention.

OBJECTIVE: To report the impact of early motor intervention in an infant with congenital heart disease (CHD) and a stroke. METHODS AND RESULTS: A 35-week newborn with a complex CHD and a normal MRI presented with early motor developmental delay at 2 months. She began an intervention program, which included biweekly motor developmental therapy with a physiotherapist, parental education, and daily home exercises. At 4 months, she underwent cardiac surgery. Following surgery, she was diagnosed with a stroke involving the right sylvian artery territory. She continued her intensive intervention program. The 12-month assessment revealed an evident gain of motor function. Despite MRI evidence of a chronic infarct involving the primary motor cortex, the child had normal motor functions. CONCLUSION: This case report supports the positive impact of early intervention in infants with CHD and its potential effect on enhancing neuroplasticity, even in children with cerebro-vascular accidents involving areas of motor function.

Parental involvement in treatment decisions regarding their critically ill child: a comparative study of France and Quebec.

OBJECTIVE: To examine whether physicians or parents assume responsibility for treatment decisions for critically ill children and how this relates to subsequent parental experience. A significant controversy has emerged regarding the role of parents, relative to physicians, in relation to treatment decisions for critically ill children. Anglo-American settings have adopted decision-making models where parents are regarded as responsible for such life-support decisions, while in France physicians are commonly considered the decision makers. DESIGN: Grounded theory qualitative methodology. SETTING: Four pediatric intensive care units (two in France and two in Quebec, Canada). PATIENTS: Thirty-one parents of critically ill children; nine physicians and 13 nurses who cared for their children. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Semistructured interviews were conducted. In France, physicians were predominantly the decision makers for treatment decisions. In Quebec, decisional authority practices were more varied; parents were the most common decision maker, but sometimes it was physicians, while for some decisional responsibility depended on the type of decision to be made. French parents appeared more satisfied with their communication and relationship experiences than Quebec parents. French parents referred primarily to the importance of the quality of communication rather than decisional authority. There was no relationship between parents' actual responsibility for decisions and their subsequent guilt experience. CONCLUSIONS: It was remarkable that a certain degree of medical paternalism was unavoidable, regardless of the legal and ethical norms that were in place. This may not necessarily harm parents' moral experiences. Further research is required to examine parental decisional experience in other pediatric settings.

The moral experience of parents regarding life-support decisions for their critically-ill children: a preliminary study in France.

The common paediatric critical care practice in France is for physicians (rather than parents) to maintain the ultimate responsibility for lifesupport decisions in children. Some French literature asserts that it is inappropriate for parents to bear such responsibilities because they do not have the required knowledge and should be protected from feeling culpable for such decisions. The aim of this grounded theory preliminary study was to examine the moral experience of parents of critically-ill children that required life-support decisions in France. A convenience purposive sample of seven parents was recruited in Paris. Five principal themes emerged as significant from these interviews: (1) a need for more information; (2) physicians should be responsible for life-support decisions; (3) the child's concerns and wishes need to be better heard; (4) maternal guilt; and (5) physicians require better training in parent communication. These findings raise important issues for clinical practice and further research in France.