Mental Health Conditions Among Children and Adolescents With Congenital Heart Disease: A Danish Population-Based Cohort Study.

BACKGROUND: Despite the known mental health burden among children with congenital heart disease (CHD), the literature is constrained by a lack of comparison cohorts and population-based follow-up data. We examined the incidence of mental health conditions among children with CHD, relative to 3 comparison cohorts. METHODS: This population-based cohort study identified all children with CHD (<18 years of age; n=16 473) in Denmark from 1996 to 2017, through linkage of individual-level data across national registries. This allowed for complete follow-up of the population. Comparison cohorts included children from the general population (n=162 204), siblings of children with CHD (n=20 079), and children with non-CHD major congenital anomalies (n=47 799). Mental health conditions were identified using inpatient and outpatient hospital discharge codes, prescription data, and data on use of community-based psychology, psychiatry, and psychotherapy services. We computed cumulative incidence by 18 years of age, incidence rates, and adjusted hazard ratios (aHRs) using Cox regression. aHRs accounted for sex, year of CHD diagnosis, parental mental health, and socioeconomic status. All estimates were stratified by age, sex, and CHD complexity. RESULTS: The cumulative incidence of mental health conditions by 18 years of age in the CHD cohort was 35.1% (95% CI, 34.0%-36.1%), corresponding to aHRs of 1.64 (95% CI, 1.58-1.71), 1.41 (95% CI, 1.30-1.52), and 1.02 (95% CI, 0.98-1.07) compared with the general population, sibling, and major congenital anomaly cohorts, respectively. Mental health incidence rates showed prominent peaks in early childhood and adolescence. Males and children with severe or single-ventricle CHD demonstrated higher incidence rates of mental health conditions relative to females and children with mild or moderate CHD, respectively. Compared with the general population and sibling cohorts, incidence rates and aHRs in the CHD cohort were highest for severe stress reactions, attention deficit/hyperactivity disorder, intellectual disability, and autism spectrum disorder. Compared with children in the major congenital anomaly cohort, the aHRs were close to 1. CONCLUSIONS: More than one-third of children with CHD were diagnosed or treated for a mental health condition by 18 years of age. Mental health conditions began early in life and were most prominent among males and children with severe or single-ventricle heart disease.

Wellbeing in Children and Adolescents with Fontan Physiology.

OBJECTIVE: To assess health-related quality of life (HRQOL) and global quality of life (QOL) in children and adolescents with Fontan physiology and identify key predictors influencing these outcomes. STUDY DESIGN: Cross-sectional analysis of 73 children and adolescents enrolled in the Australia and New Zealand Fontan Registry aged 6-17 years, at least 12 months post-Fontan operation. Assessments included the Pediatric Quality of Life Inventory (PedsQL) for HRQOL and a developmentally-tailored visual analogue scale (0-10) for global QOL, along with validated sociodemographic, clinical, psychological, relational, and parental measures. Clinical data were provided by the Australia and New Zealand Fontan Registry. RESULTS: Participants (mean age: 11.5 ± 2.6 years, 62% male) reported lower overall HRQOL (P < .001), and lower scores across all HRQOL domains (all P < .0001), compared with normative data. Median global QOL score was 7.0 (IQR 5.8-8.0), with most participants (79%) rating their global QOL ≥6. Anxiety and depressive symptoms requiring clinical assessment were reported by 21% and 26% of participants, respectively. Age, sex, and perceived seriousness of congenital heart disease explained 15% of the variation in HRQOL scores, while depressive symptoms and treatment-related anxiety explained an additional 37% (final model: 52% of variance explained). For global QOL, sociodemographic and clinical factors explained 13% of the variance in scores, while depressive symptoms explained a further 25% (final model: 38% of variance explained). Parental factors were not associated with child QOL outcomes. CONCLUSIONS: Children and adolescents with Fontan physiology experience lower HRQOL than community-based norms, despite reporting fair overall QOL. Psychological factors predominantly influenced QOL outcomes, indicating strategies to bolster psychological health could improve QOL in this population.

Mother-Infant Dyadic Synchrony and Interaction Patterns After Infant Cardiac Surgery.

OBJECTIVE: Parents and their infants with complex congenital heart disease (CHD) face relational challenges, including marked distress, early separations, and infant hospitalizations and medical procedures, yet the prevalence of parent-infant interaction difficulties remains unclear. Using a standardized observational paradigm, this study investigated mother-infant dyadic synchrony, interactional patterns, and associated predictors in mother-infant pairs affected by CHD, compared with typically-developing pairs. METHODS: In this prospective, longitudinal cohort study, mothers and their infants requiring cardiac surgery before age 6-months (n=110 pairs) and an age- and sex-matched Australian community sample (n=85 pairs) participated in a filmed, free-play interaction at 6.9±1.0 months. Mother-infant dyadic synchrony, maternal and infant interactional patterns, and relational risk were assessed using the Child-Adult Relationship Experimental (CARE) Index. Maternal and infant predictors were assessed at 32 weeks gestation, 3- and 6-months postpartum. RESULTS: Most mother-infant interactions were classified as "high risk" or "inept" (cardiac: 94%, control: 81%; p=.007). Dyadic synchrony (p<.001), maternal sensitivity (p=.001), and infant cooperativeness (p=.001) were lower for cardiac than control pairs. Higher maternal traumatic stress at 6-months postpartum predicted lower dyadic synchrony for mother-infant pairs affected by CHD (B=-.04, p=.03). Dyadic synchrony was higher among older infants in the total (B=.40, p=.003) but not cardiac sample (B=.24, p=.06). CONCLUSIONS: Relational difficulties were almost universal among mother-infant pairs affected by CHD and were also high in the Australian community sample. Widespread education initiatives are recommended to increase awareness of heightened mother-infant relational risk in congenital heart care and well-child settings, alongside relationally-focused prevention and early intervention programs.

Cognitive Functioning and Psychosocial Outcomes in Adults with Complex Congenital Heart Disease: A Cross-sectional Pilot Study.

Adults with complex congenital heart disease (CHD) are at risk for cognitive dysfunction. However, associations between cognitive dysfunction and psychosocial outcomes are poorly defined. Between June and November 2022, we prospectively recruited 39 adults with complex CHD who completed a computerized cognitive assessment (Cogstate) and validated psychosocial scales measuring psychological distress, health-related quality of life (HRQOL), and resilience. Participants had a mean age of 36.4 ± 11.2 years. Over half (62%) were women, most (79%) had complex biventricular CHD, and 21% had Fontan physiology. Prevalence of cognitive dysfunction was greatest in the domains of attention (29%), working memory (25%), and psychomotor speed (21%). Adjusting for age and sex, Pearson partial correlations between Cogstate z-scores and self-reported cognitive problems were small. Participants who lived in the most disadvantaged areas and those with a below-average annual household income had lower global cognitive z-scores (p = 0.02 and p = 0.03, respectively). Two-thirds (64%) reported elevated symptoms of depression, anxiety, and/or stress. Small correlations were observed between psychological distress and cognitive performance. Greater resilience was associated with lower psychological distress (r ≥ -0.5, p < 0.001) and higher HRQOL (r = 0.33, p = 0.02). Our findings demonstrate that adults with complex CHD have a high risk of cognitive dysfunction, though may not recognize or report their cognitive challenges. Lower socioeconomic status may be an indicator for those at risk of poorer cognitive functioning. Psychological distress is common though may not be a strong correlate of performance-based cognitive functioning. Formal cognitive evaluation in this patient population is essential. Optimizing resilience may be a protective strategy to minimize psychological distress and bolster HRQOL.

Australian National Standards of Care for Childhood-onset Heart Disease (CoHD Standards). 1st Edition.

These first Australian National Standards of Care for Childhood-onset Heart Disease (CoHD Standards) have been developed to inform the healthcare requirements for CoHD services and enable all Australian patients, families and carers impacted by CoHD (paediatric CoHD and adult congenital heart disease [ACHD]) to live their best and healthiest lives. The CoHD Standards are designed to provide the clarity and certainty required for healthcare services to deliver excellent, comprehensive, inclusive, and equitable CoHD care across Australia for patients, families and carers, and offer an iterative roadmap to the future of these services. The CoHD Standards provide a framework for excellent CoHD care, encompassing key requirements and expectations for whole-of-life, holistic and connected healthcare service delivery. The CoHD Standards should be implemented in health services in conjunction with the National Safety and Quality Health Service Standards developed by the Australian Commission on Safety and Quality in Health Care. All healthcare services should comply with the CoHD Standards, as well as working to their organisation's or jurisdiction's agreed clinical governance framework, to guide the implementation of structures and processes that support safe care.

Are Parent Discharge Readiness Scores Effective for Patients With Congenital Heart Disease After Cardiac Surgery?

OBJECTIVE: To assess discharge readiness and clinical engagement post-discharge in families of children undergoing congenital heart surgery. STUDY DESIGN: This prospective cross-sectional study was performed at a major tertiary pediatric cardiac referral center. Eligible parents and caregivers completed a discharge readiness tool, the Readiness for Hospital Discharge Scale for Parents of Hospitalized Children, via online survey on the day of discharge. Clinical engagement data included subsequent phone calls, clinic visits, emergency department visits, and hospital readmissions. Readiness for Hospital Discharge Scale for Parents of Hospitalized Children scores were measured as follows: very high (9-10), high (8-8.9), moderate (7-7.9), and low (<7). Descriptive statistics were used to describe demographic data. RESULTS: In total, 128 families enrolled between April and December 2021. Parent discharge readiness scores ranged from "high" to "very high." Families with lower socioeconomic status and younger patients (especially single-ventricle infants or "interstage") had a greater proportion of clinic visits, emergency department visits, and hospital readmissions within 30-days postdischarge compared with other groups. CONCLUSIONS: Discharge readiness scores were not associated with clinical engagement. We identified vulnerable populations as evidenced by a greater frequency of clinical engagement in the immediate postoperative period, particularly younger patients and first-time surgeries. Although these visits may be appropriate, novel programs could enhance education and emotional support to prevent delay in seeking care or creating excessive stress and anxiety after discharge.

Systematic Review of Instruments Assessing Psychosocial Adaptation and Outcomes Among Families of Children With Congenital Heart Disease.

OBJECTIVE: This systematic review identified instruments quantitatively assessing psychosocial adaptation and outcomes in families of children with congenital heart disease (CHD) and evaluated instrument psychometrics. METHODS: Following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines and a prospectively registered protocol, electronic databases (CINAHL, Embase, PubMed/MEDLINE, PsycINFO, and SCOPUS) were searched from inception until June 20, 2021 for peer-reviewed articles published in English, reporting quantitative data on psychosocial outcomes among parents/caregivers, siblings, or family system. Instrument characteristics and psychometrics were extracted, and adapted COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) criteria were applied to assess instrument quality. Descriptive statistics and narrative synthesis were used for analysis. RESULTS: Overall, 108 articles reporting on 107 distinct samples across 26 countries met inclusion. Across those articles, 40 instruments assessed psychological functioning or distress, 12 assessed coping, 11 assessed quality of life constructs, 10 assessed parenting stress/caregiver burden, 10 assessed family functioning/impact, 10 assessed stress appraisal, 5 assessed sibling psychosocial outcomes, and 2 assessed couple relationship satisfaction/strain. Applying COSMIN criteria to available data on original instrument development articles/manuals for English language instruments (n = 54), 67% scored a positive property evidence rating for content validity, 39% for internal consistency, 4% for test-retest reliability, and 9% for responsiveness (longitudinal validity). CONCLUSIONS: Studies vary widely in instruments used to assess psychosocial adaptation and outcomes among families of children with CHD. Instrument selection informed by robust key psychometrics, increased psychometric reporting, development of both a "toolkit" approach and a comprehensive CHD-specific family instrument are among key recommendations.

Evaluation of the use of visual storytelling as an educational intervention in the cardiac ICU: reaching parents before they are in crisis.

OBJECTIVE: To evaluate the acceptability and safety of educational videos utilising visual storytelling to provide information about the cardiac ICU and post-operative care to parents. Videos were designed to educate, further encourage parents to engage in their child's cardiac care, and address common sources of distress. STUDY DESIGN: Two educational videos and survey were sent to 29 families of children previously admitted to the cardiac ICU (April 2020-March 2021). Views regarding information quality, quantity, format, and relevance were assessed, as were parents' emotional responses. Quantitative thresholds for safety and acceptability were set a priori. An inductive approach to content analysis was applied to identify themes in qualitative data. RESULTS: Sixteen parents participated (response rate: 55%). All acceptability and safety thresholds were met; 92% of parents rated the videos as helpful and 85% were "very" or "extremely likely" to recommend them to other families of children with CHD. No participants reported significant distress after viewing the videos. Expressions of parental engagement with their child's care team were common (92%). In qualitative responses, parents perceived the videos as potentially helpful in reducing distress if viewed prior to cardiac ICU admission. CONCLUSION: Visual storytelling to orient parents to the cardiac ICU and address common stressors was found to be safe and acceptable when tested with parents of children previously admitted to the cardiac ICU. Further prospective studies are needed to test intervention effects when videos are viewed before or during cardiac ICU admission, especially for mitigating anxiety and traumatic stress associated with admission.

Telehealth services for cardiac neurodevelopmental care during the COVID-19 pandemic: a site survey from the Cardiac Neurodevelopmental Outcome Collaborative.

OBJECTIVE: COVID-19 has markedly impacted the provision of neurodevelopmental care. In response, the Cardiac Neurodevelopmental Outcome Collaborative established a Task Force to assess the telehealth practices of cardiac neurodevelopmental programmes during COVID-19, including adaptation of services, test protocols and interventions, and perceived obstacles, disparities, successes, and training needs. STUDY DESIGN: A 47-item online survey was sent to 42 Cardiac Neurodevelopmental Outcome Collaborative member sites across North America within a 3-week timeframe (22 July to 11 August 2020) to collect cross-sectional data on practices. RESULTS: Of the 30 participating sites (71.4% response rate), all were providing at least some clinical services at the time of the survey and 24 sites (80%) reported using telehealth. All but one of these sites were offering new telehealth services in response to COVID-19, with the most striking change being the capacity to offer new intervention services for children and their caregivers. Only a third of sites were able to carry out standardised, performance-based, neurodevelopmental testing with children and adolescents using telehealth, and none had completed comparable testing with infants and toddlers. Barriers associated with language, child ability, and access to technology were identified as contributing to disparities in telehealth access. CONCLUSIONS: Telehealth has enabled continuation of at least some cardiac neurodevelopmental services during COVID-19, despite the challenges experienced by providers, children, families, and health systems. The Cardiac Neurodevelopmental Outcome Collaborative provides a unique platform for sharing challenges and successes across sites, as we continue to shape an evidence-based, efficient, and consistent approach to the care of individuals with CHD.

Neurocognitive Dysfunction and Smaller Brain Volumes in Adolescents and Adults With a Fontan Circulation.

BACKGROUND: Neurocognitive outcomes beyond childhood in people with a Fontan circulation are not well defined. This study aimed to investigate neurocognitive functioning in adolescents and adults with a Fontan circulation and associations with structural brain injury, brain volumetry, and postnatal clinical factors. METHODS: In a binational study, participants with a Fontan circulation without a preexisting major neurological disability were prospectively recruited from the Australia and New Zealand Fontan Registry. Neurocognitive function was assessed by using Cogstate software in 107 participants with a Fontan circulation and compared with control groups with transposition of the great arteries (n=50) and a normal circulation (n=41). Brain MRI with volumetric analysis was performed in the participants with a Fontan circulation and compared with healthy control data from the ABIDE I and II (Autism Brain Imaging Data Exchange) and PING (Pediatric Imaging, Neurocognition, and Genetics) data repositories. Clinical data were retrospectively collected. RESULTS: Of the participants with a Fontan circulation who had a neurocognitive assessment, 55% were male and the mean age was 22.6 years (SD 7.8). Participants with a Fontan circulation performed worse in several areas of neurocognitive function compared with those with transposition of the great arteries and healthy controls (P<0.05). Clinical factors associated with worse neurocognitive outcomes included more inpatient days during childhood, younger age at Fontan surgery, and longer time since Fontan procedure (P<0.05). Adults with a Fontan circulation had more marked neurocognitive dysfunction than adolescents with a Fontan circulation in 2 domains (psychomotor function, P=0.01 and working memory, P=0.02). Structural brain injury was present in the entire Fontan cohort; the presence of white matter injury was associated with worse paired associate learning (P<0.001), but neither the presence nor severity of infarct, subcortical gray matter injury, and microhemorrhage was associated with neurocognitive outcomes. Compared with healthy controls, people with a Fontan circulation had smaller global brain volumes (P<0.001 in all regions) and smaller regional brain volumes in most cerebral cortical regions (P<0.05). Smaller global brain volumes were associated with worse neurocognitive functioning in several domains (P<0.05). A significant positive association was also identified between global brain volumes and resting oxygen saturations (P≤0.04). CONCLUSIONS: Neurocognitive impairment is common in adolescents and adults with a Fontan circulation and is associated with smaller gray and white matter brain volume. Understanding modifiable factors that contribute to brain injury to optimize neurocognitive function is paramount.