The burden of psychological trauma and post-traumatic stress disorder among adults with congenital heart disease: PTSD in ACHD.

Psychological trauma, symptoms of post-traumatic stress disorder (PTSD), and mental health conditions are common in adult congenital heart disease (ACHD). There is a gap in research examining PTSD in ACHD using the current Diagnostic and Statistical Manual of Mental Disorders, fifth edition (DSM-5) criteria in assessing patient characteristics and experiences with trauma-focused treatment. Surveys were offered to outpatients over a 6-month enrollment period to be completed by way of a QR code on their personal smart phone. Patient-reported items include a detailed medical and psychosocial history, the Oslo social support scale, adverse childhood experiences survey, and the PTSD checklist for DSM-5. Of 158 patients (77% moderate or complex heart disease) who provided complete data, a provisional diagnosis of PTSD was found in 48 patients (30%) using a PTSD checklist for DSM-5 cut-off score of ≥31. A positive PTSD screen was associated with younger age, nonwhite race, presence of heart failure, lower New York Heart Association functional class, lower linear quality of life score, lower Oslo social support scale score, an insecure caregiver relation, period of unemployment, emergency department visits, medication nonadherence, and coexisting mental health disorders. Complexity of heart disease and number of surgical and/or catheter-based interventions were not associated with PTSD, although having undergone no cardiac surgeries until adulthood (aged ≥18 years) was associated with a lower prevalence of PTSD. Those who screened positive for PTSD were more likely to report multiple traumatic events, including noncardiac traumatic events. Only 14 of 48 patients (29%) reported a known diagnosis of PTSD, although 44 patients (92%) reported having ever seen a mental health provider. A total of 18 patients (38%) reported currently having a mental health provider. A total of 30 patients (62%) had heard of at least 1 evidence-based trauma-informed therapy, and 14 (29%) had tried at least 1. In conclusion, using the DSM-5 criteria, we observed a high prevalence of potential PTSD in ACHD associated with several novel cardiac and psychosocial patient factors. Future longitudinal studies will be necessary to establish causality. Few patients with ACHD have been formally diagnosed with PTSD or have experience with evidence-based trauma-informed therapies.

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.

Quality of life in Arab children with congenital heart disease.

BACKGROUND AND PURPOSE: Management strategies for children with congenital health diseases (CHDs) should encompass more than just the medical aspect of the disease and consider how heart diseases affect their everyday activities and, subsequently, their quality of life (QoL). Global studies witnessed a greater emphasis on studying the QoL associated with CHD. However, there is still a great lag in such data in the Arab region. The purpose of this study was to evaluate QoL in children with CHD using an Arab sample from Jordan. The specific objectives were twofold: (1) to contrast the assessments of children's QoL reported by their parents with those reported by the children themselves, and (2) to assess the factors that influence the QoL of children with CHD. METHODS: A total of 79 children aged 2-18 with a confirmed diagnosis of CHD were included in the study, along with their mothers. Of them, 38.0% were girls, 67.1% were diagnosed with non-cyanotic CHD, 58.2% had a severe CHD, 92.4% had undergone at least one operation, 81.0% had repaired defects, 13.9% underwent palliated procedures, and 24.1% were admitted to a neonatal intensive care unit after delivery. The Pediatric Quality of Life Inventory was used to assess QoL of children with CHD. Both children's and parents' reports of QoL were analyzed using paired-sample t-tests, ANOVAs, and multiple linear regression. RESULTS: Older children reported significantly lower QoL scores, whereas there were no differences in parents-reported QoL scores across different children age groups. There was a divergence in perceptions of QoL between parents-reported and children-reported scores with parents reporting significantly lower scores. The children-reported QoL in this study seemed to be significantly associated with their gender, age, and the presence of learning difficulties, whereas the parent-reported QoL was only associated with the presence of learning difficulties. CONCLUSIONS: Responses from both children and parents need to be considered to understand the similarities and differences between them and to provide further insight into the optimal way to help children with CHD effectively navigate the transition into adulthood. Future research studies of outcomes for survivors of children with CHD are needed to identify high-risk survivors for worse psychosocial functioning and assess prevention measures and treatment interventions to improve their QoL.

Age moderates change in disease-related stress among congenital heart disease survivors: a 6-year follow-up.

AIMS: As congenital heart disease (CHD) survivors age, they are confronted with elevated risk of cardiovascular morbidity and increasingly complex disease self-management demands. Given that stress is associated with poor physical and psychosocial outcomes, it is crucial to examine how disease-related stress changes over time in this population. However, this outcome has received little research attention to date. This study aimed to identify demographic and clinical predictors of change in disease-related stress over 6 years among CHD survivors. METHODS AND RESULTS: Congenital heart disease survivors (N = 252, Mage = 25.6 ± 7.1, 52.9% female) completed the first 13 items of the Responses to Stress Questionnaire, adapted for use among CHD survivors, to assess disease-related stressors at study entry (T1) and 6-year follow-up (T2). Age, gender, estimated family income, and New York Heart Association (NYHA) functional class at T1 were entered into mixed linear models to determine their impact on change in disease-related stress. Older age (P < 0.001), lower income (P < 0.001), and presence of functional limitations (NYHA ≥ II) (P < 0.001) predicted greater increases in disease-related stress. When controlling for NYHA, functional class, and income, a significant time by age interaction was identified such that disease-related stress increased over time among those who were adolescents at T1 [b = 4.20, P = 0.010, 95% confidence interval (1.01, 7.40)], but remained stable among young adults. CONCLUSION: The transition from adolescence to adulthood may be a period of increasing disease-related stress. Healthcare providers should consider screening adolescents for elevated disease-related stress during transition education and provide resources to bolster resilience.

A randomized controlled trial to assess the impact of psychoeducation on the quality of life of parents with children with congenital heart defects-Quantitative component.

PURPOSE: To develop, implement and assess the results of psychoeducation to improve the QoL of parents with CHD newborns. METHODS: Participants were parents of inpatient newborns with the diagnosis of non-syndromic CHD. We conducted a parallel RCT with an allocation ratio of 1:1 (intervention vs. control), considering the newborns, using mixed methods research. The intervention group received psychoeducation (Parental Psychoeducation in CHD [PPeCHD]) and the usual routines, and the control group received just the regular practices. The allocation concealment was assured. PI was involved in enrolling participants, developing and implementing the intervention, data collection and data analysis. We followed the Consolidated Standards of Reporting Trials (CONSORT) guidelines. RESULTS: Parents of eight newborns were allocated to the intervention group (n = 15 parents) and eight to the control group (n = 13 parents). It was performed as an intention-to-treat (ITT) analysis. In M2 (4 weeks), the intervention group presented better QoL levels in the physical, psychological, and environmental domains of World Health Organization Quality of Life instrument (WHOQOL-Bref). In M3 (16 weeks), scores in physical and psychological domains maintained a statistically significant difference between the groups. CONCLUSIONS: The PPeCHD, the psychoeducational intervention we developed, positively impacted parental QoL. These results support the initial hypothesis. This study is a fundamental milestone in this research field, adding new essential information to the literature.

Illness identity and well-being in congenital heart disease: Directionality of effects and developmental trajectories.

OBJECTIVE: This longitudinal study explores the relationship between illness identity and well-being in emerging adults with congenital heart disease (CHD), aiming to understand the factors contributing to well-being in individuals with CHD. METHOD: Dutch-speaking emerging adults with CHD (N = 254, age range = 24-28 years) participated in a three-wave study, which is part of the I-DETACH 2 project. Cross-lagged analyses examined the directionality of effects between illness identity and well-being. Multivariate latent class growth analysis identified developmental trajectory classes of illness identity. Multigroup latent growth curve modeling investigated differences in the development of well-being among these classes. RESULTS: Bidirectional associations were uncovered between illness identity and well-being. For instance, acceptance predicted better quality of life and less depressive symptoms over time. Three trajectory classes of illness identity were identified: high (i.e., as compared to the sample mean) acceptance and enrichment with low rejection and engulfment (Class 1), high rejection with low levels in the other dimensions (Class 2), and high rejection and engulfment along with high enrichment and low acceptance (Class 3). Individuals in Class 3 experienced the worse well-being. In addition, individuals with complex heart defects were strongly represented in this class. CONCLUSIONS: This study demonstrates the significance of illness identity in understanding individual differences in well-being among emerging adults with CHD. Additionally, this study provided valuable insight in the development of illness identity and its longitudinal relationship with well-being. (PsycInfo Database Record (c) 2024 APA, all rights reserved).

Association of genetic and sulcal traits with executive function in congenital heart disease.

OBJECTIVE: Persons with congenital heart disease (CHD) are at increased risk of neurodevelopmental disabilities, including impairments to executive function. Sulcal pattern features correlate with executive function in adolescents with single-ventricle heart disease and tetralogy of Fallot. However, the interaction of sulcal pattern features with genetic and participant factors in predicting executive dysfunction is unknown. METHODS: We studied sulcal pattern features, participant factors, and genetic risk for executive function impairment in a cohort with multiple CHD types using stepwise linear regression and machine learning. RESULTS: Genetic factors, including predicted damaging de novo or rare inherited variants in neurodevelopmental disabilities risk genes, apolipoprotein E genotype, and principal components of sulcal pattern features were associated with executive function measures after adjusting for age at testing, sex, mother's education, and biventricular versus single-ventricle CHD in a linear regression model. Using regression trees and bootstrap validation, younger participant age and larger alterations in sulcal pattern features were consistently identified as important predictors of decreased cognitive flexibility with left hemisphere graph topology often selected as the most important predictor. Inclusion of both sulcal pattern and genetic factors improved model fit compared to either alone. INTERPRETATION: We conclude that sulcal measures remain important predictors of cognitive flexibility, and the model predicting executive outcomes is improved by inclusion of potential genetic sources of neurodevelopmental risk. If confirmed, measures of sulcal patterning may serve as early imaging biomarkers to identify those at heightened risk for future neurodevelopmental disabilities.

Resilient Hearts: Measuring Resiliency in Young People With Congenital Heart Disease.

Background Congenital heart disease (CHD) is a life-long disease with long-term consequences on physical and mental health. Patients with CHD face multifaceted physical and psychosocial challenges. Resilience is an important factor that can be protective and positively impact mental health. We studied resiliency and its associated factors in teenagers and young adults with and without CHD using a social media-delivered survey. Resilience was measured using the 25-item Connor-Davidson Resilience Scale, a validated metric with a historical mean of 80.4/100 in the general adult population. Methods and Results Individuals with and without CHD, aged 10 to 25 years, were prospectively recruited on social media to complete an online survey. The survey was completed from January to February 2022. Respondents provided information on their demographics and CHD details (where applicable) and completed the Connor-Davidson Resilience Scale. As a group, participants with CHD had higher resilience scores compared with same-aged healthy individuals (65.3±16.1 versus 55.4±13.8; P<0.001). For both cohorts, sex, race, and age were not associated with differences in resilience score. For individuals with CHD, lower resilience was associated with more hospital admissions, lack of exercise, presence of a mental health diagnosis, and no participation in support groups or disease-specific camps. Conclusions Young people with CHD had higher resilience than individuals without CHD in our sample. We identified several factors, both modifiable and nonmodifiable, that are associated with higher resilience. Awareness of resiliency and its contributors in the population with CHD may assist medical teams in improving patient physical and psychological well-being.

Psychiatric co-morbidity in children and adolescents with CHDs: a systematic review.

The population of long-term survivors with CHDs is increasing due to better diagnostics and treatment. This has revealed many co-morbidities including different neurocognitive difficulties. However, the prevalence of psychiatric disorders among children and adolescents and the specific types of disorders they may experience are unclear. We systematically reviewed the existing literature, where psychiatric diagnoses or psychiatric symptoms were investigated in children and adolescents (age: 2-18 aged) with CHDs and compared them with a heart-healthy control group or normative data. The searches were done in the three databases PubMed, psychINFO, and Embase. We included 20 articles reporting on 8035 unique patients with CHDs. Fourteen articles repoted on psychological symptoms, four reported on psychiatric diagnoses, and two reported on both symptoms and diagnoses. We found that children and adolescents with a CHD had a higher prevalence of attention deficit hyperactivity disorder (ranging between 1.4 and 9 times higher) and autism (ranging between 1.8 and 5 times higher) than controls, but inconsistent results regarding depression and anxiety.

[The Unknown Journey: Uncertain Experiences in Mothers of Infants With Congenital Heart Disease Before Undergoing Open Heart Surgery].

BACKGROUND: Most children with complex congenital heart disease (CHD) require open-heart surgery within one year of birth to survive. Thus, new mothers of infants with CHD are faced with making unexpected and difficult decisions. PURPOSE: This study was designed to explore the essence of the maternal uncertain experience prior to infants with CHD undergoing open-heart surgery. METHODS: In this study, a phenomenological approach was used and data were collected using open-ended interview guidelines structured around the Uncertainty in Illness Theory. Nine mothers of infants with CHD who had received open-heart surgery were interviewed in a hospital interview room within two weeks the operation. Colaizzi's (1978) data processing procedure was applied in the post-interview analysis. RESULTS: Five themes emerged: (1) Hit bottom and felt helpless; (2) Hit the road - An overwhelming sense of emergency; (3) The crunch - Do your best to accept destiny (4) Disease brought the unknown; (5) Hope in uncertainty. CONCLUSIONS / IMPLICATIONS FOR PRACTICE: The high-risk nature of and their unfamiliarity with the surgery made the participants experience illness uncertainty. The medical system should develop more-structured CHD information and provide emotional support in a timely manner to alleviate illness uncertainty in mothers of infants with CHD.

A Psychosocial Care Model for Families Affected by Congenital Heart Disease.

OBJECTIVES: To develop a model of family-based psychosocial care for congenital heart disease (CHD). DESIGN: Qualitative study using crowdsourced data collected from parents of young children with CHD who received care across 42 hospitals. SETTING: Yammer, a social networking platform used to facilitate online crowdsourcing and qualitative data collection. SUBJECTS: Geographically diverse sample of 100 parents (72 mothers and 28 fathers) of young children with CHD. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Parents joined a private group on Yammer and responded to 37 open-ended study questions over a 6-month period. Qualitative data were coded and analyzed using an iterative process. Three broad themes corresponding to pillars of family-based psychosocial care were identified: pillar 1) parent partnership in family-integrated medical care, pillar 2) supportive interactions focused on parent and family wellbeing, and pillar 3) integrated psychosocial care and peer support for parents and families. Each pillar was supported by subthemes corresponding to specific intervention strategies. Most parents described the need for intervention strategies across multiple pillars, with almost half reporting needs across all three pillars of psychosocial care. Parents' preferences for psychosocial support changed over time with changes to their child's medical status and across care settings (e.g., hospital, outpatient clinic). CONCLUSIONS: Results support a model of family-based psychosocial care that is multidimensional and flexible to meet the needs of families affected by CHD. All members of the healthcare team play an important role in providing psychosocial support. Future research incorporating components of implementation science is needed to promote uptake of these findings, with the goal of optimizing family-based psychosocial support in the hospital setting and beyond.

Preferences for disease-related information and transitional skills among adolescents with congenital heart disease in the early transitional stage.

PURPOSE: The transition towards adult-focused healthcare comprises a complex process requiring careful, individualized guidance of adolescents with congenital heart disease (CHD). Detailed data on their preferences regarding disease-related information and acquirable transitional skills are mostly lacking. We examined the preferences of CHD adolescents in the early transition stage. METHODS: A cross-sectional descriptive study was performed with adolescents recruited from a transition program. Two questionnaires assessing needs for information and transitional skills were used. Only questionnaires completed in the early transition stage were included. RESULTS: Forty-nine adolescents participated (mean age 15.9 ± 1.2 years, 43% girls). 59% requested information about their heart and previous and/or future surgeries/interventions, 45% about sports and medication, and a maximum of 27% about psychosocial topics. More girls than boys requested surgical information (76% versus 46%; p = 0.04). Adolescents with severely complex CHD more often requested information about medication than those with moderately complex CHD (63% versus 28%; p = 0.02). Older adolescents were less likely to request surgical information (OR = 0.53; 95%CI [0.26-0.88]; p = 0.03). Up to 83% of the adolescents perceived their skills as insufficient, but less than 14% was interested in acquiring skills.    Conclusion: Adolescents were mainly interested in medical topics, followed by lifestyle information. The informational needs tended to decrease with age. Timely gauging individual needs and delivering information, ideally in the early teens, appear important when providing person-tailored transitional care in CHD. Paradoxically, although the transitional skills were low-perceived, there was limited motivation to improve them. This paradox requires further investigation to better tailor transition interventions.     Clinical trial registration: Not applicable. WHAT IS KNOWN: • Transition towards adult life and care requires careful patient guidance. A person-tailored approach is strongly encouraged. However, details on preferences regarding disease-related information and transitional skills are scarce in adolescents with CHD. WHAT IS NEW: • This study showed that adolescents with CHD in early transition mainly need medical-related information. Their informational needs tend to decrease with age necessitating timely gauging for interest and delivering information. Adolescents report a low need to acquire transitional skills despite low self-esteemed skills levels.

Behavioral and Emotional Outcomes in Children with Congenital Heart Disease: Effects of Disease Severity, Family Life Stress, Disease-Related Chronic Stress, and Psychosocial Adaptation.

OBJECTIVE: To evaluate whether effects of congenital heart disease (CHD) severity and family life stress on behavioral and emotional functioning are mediated by disease-related chronic stress and psychosocial adaptation. STUDY DESIGN: A cross-sectional analysis of the Pediatric Cardiac Quality of Life Inventory Testing Study was performed. Relationships between CHD severity (comprising 3 groups: mild heart disease, moderate biventricular disease, and single ventricle) and family life stress, on patient- and parent disease-related chronic stress, psychosocial adaptation, and behavioral-emotional outcomes were assessed using structural equation modeling. Patient and parent models were reported separately. RESULTS: There were 981 patient-parent dyads: 22% had mild heart disease, 63% biventricular, and 15% single ventricle; 19% of families reported moderate to major family life stress. Path models revealed that CHD severity and family life stress were mediated by disease-related chronic stress and psychosocial adaptation factors (R2 = 0.18-0.24 for patient outcomes and R2 = 0.33-0.34 for parent outcomes, P < .001, respectively). CONCLUSIONS: The effects of greater CHD severity and family life stress on behavioral-emotional outcomes were mediated by worse disease-related chronic stress and psychosocial adaptation factors. Both disease-related chronic stress and psychosocial adaptation factors may be targets for interventions to improve behavioral and emotional outcomes.

Adolescents and Adults With Congenital Heart Disease: Why Are They Lost to Follow-Up?

Almost 90% of infants with congenital heart disease (CHD) now reach adulthood but require long-term surveillance to recognize and manage residual and/or evolving lesions. Yet many are lost to follow-up. A scoping review identified four specific domains that pose barriers to consistent follow-up. There are multiple issues associated with transition from pediatric to adult care which included-the lack of a seamless transfer, the establishment of a new trusting relationship, promoting the right balance of patient autonomy and addressing knowledge gaps. Additional issues related to logistic problems of time, distance, cost, and the availability of specialized care, are further compounded by the psychosocial factors and the heterogeneity of the cardiac abnormality affecting our patients. Further study of all these issues is warranted to improve ongoing engagement.

Psychosocial Risk Factors for Health-Related Quality of Life in Adult Congenital Heart Disease.

BACKGROUND: There is variability in the impact of adult congenital heart disease (ACHD) on health-related quality of life (HRQoL). A greater insight into the impact of ACHD may be gained from investigating HRQoL in various diagnostic groups and considering the importance of psychosocial risk factors for poor HRQoL. OBJECTIVE: We compared the HRQoL of people with ACHD with normative data from the general population and among 4 diagnostic groups and identified risk factors for poor HRQoL in ACHD from a comprehensive set of sociodemographic, clinical, and psychosocial factors. METHODS: We conducted a cross-sectional study with 303 participants from 4 diagnostic groups Simple, Tetralogy of Fallot, Transposition of the Great Arteries, Single Ventricle who completed measures of illness perceptions, coping, social support, mood, and generic and disease-specific HRQoL. Data were analyzed using 1-sample t tests, analysis of variance, and hierarchical multiple regressions. RESULTS: There was diminished psychosocial HRQoL in the Simple group compared with the general population. Consistently significant risk factors for poor HRQoL included younger age, a perception of more severe symptoms due to ACHD, depression, and anxiety. Clinical factors were poor predictors of HRQoL. CONCLUSIONS: The findings highlight the need to develop intervention studies aiming to improve HRQoL in people with ACHD and the routine assessment of illness perceptions and mood problems during key periods in people's lives. This will help address patient misconceptions that could be tackled by clinicians or specialist nurses during routine outpatient appointments and identify people in need of psychological support.

Impact of telemedicine on the mental status of parents of children with CHD: A meta-analysis.

BACKGROUND: Studies on telemedicine concentrate largely on children with congenital heart disease (CHD), in lieu of their respective parents. The psychological responses of parents of children with CHD may impact on their child's well-being. This study aimed to examine the impact of telemedicine on the mental status of parents of children with CHD. METHODS: A meta-analytic approach was used to analyze scores on the Self-reporting Anxiety Scale (SAS), the Self-reporting Depression Scale (SDS), and the World Health Organization Quality of Life, Brief Report (WHOQOL-BREF) of the parents of children with CHD. Scores were compared between the telemedicine group and the control group prior to and following the intervention using the measurement of mean difference (MD) with 95% confidence interval (CIs). RESULTS: A total of 209 studies were assessed, of which 10 were incorporated into the systematic review, while four other studies met the meta-analysis inclusion criteria. Following the intervention, the SAS and SDS scores were significantly lower in the telemedicine group in comparison to the control group (p < 0.001). Telemedicine also exerted a substantial influence on lowering the parents' SAS (p < 0.001) and SDS scores (p < 0.001), as well as improving the quality of life of the parents (p < 0.001). CONCLUSION: Telemedicine has a positive impact on lowering both anxiety and depression as well as improving the quality of life of parents of children with CHD.

Identifying developmental challenges of youth with congenital heart defects: A patient-oriented perspective.

BACKGROUND: Youth with congenital heart disease (CHD) are at high risk for a range of developmental impairments that become evident at different times across childhood and adolescence. This study aimed to explore perspectives of youth with CHD with respect to their developmental follow-up across childhood. METHODS: Interpretive description was used as a methodological approach for this qualitative study. Youth aged 12-22 years with CHD requiring open-heart surgery before 2 years of age and who had received health services in Canada since birth were enrolled. RESULTS: Ten youth with CHD, two males and eight females, aged 13-22 years (mean 19.8) participated in this study. With higher social and academic demands as well as increased level of autonomy associated with older age, some youth faced new challenges that they had not encountered as children. Youth with CHD identified four aspects of the continuum of care as needing to be changed to better respond to their needs. First, the format of developmental follow-up needs to be adapted to their unique challenges. Second, resources must be more easily accessible throughout childhood and adolescence. Third, planning for transition to adult care is essential to ensure continuity of services. Finally, they identified that the school system is an essential component of the continuum of care. CONCLUSIONS: Adolescents and young adults with CHD are at high risk of developing physical, academic and psychosocial challenges; however, timely identification of challenges does not appear to be optimal across domains and transition points, from the perspective of the youth themselves. Youth with CHD reported not having the resources and supports they required to optimize their functioning. Our findings suggest that several approaches could be adopted to enhance identification and outcomes to address the limitations of current Canadian practices.

Difficulties With Executive Function Are Associated With Risky Health Behaviors Among Young Adult Congenital Heart Defect Survivors.

BACKGROUND: Adults with congenital heart defects (CHDs) have an increased risk for cardiovascular complications, but few are in optimal cardiovascular health. Executive function deficits, which are elevated among CHD survivors, may contribute to engagement in behaviors that increase cardiovascular risk. OBJECTIVE: The aim of this study was to identify the association between executive function and the health risk behaviors of tobacco use, saturated fat intake, and insufficient physical activity among young adults with CHDs. METHODS: Young adults with CHDs completed the Behavior Rating Inventory of Executive Function-Adult Version, which yields composite and 9 individual domain scores (eg, inhibition, working memory). Participants also completed self-report measures of tobacco use, saturated fat intake, and physical activity. Linear and logistic regression models determined the association between executive function and risky health behaviors, adjusting for demographic factors, disease status, and emotional distress. RESULTS: One in 10 young adults with CHDs reported clinically significant difficulties with global executive function. Difficulties with global executive function were associated with increased tobacco use ( P = .018) and greater saturated fat intake ( P = .015) but not insufficient physical activity ( P = .902). Difficulties with inhibition were specifically associated with increased tobacco use ( P = .003) and greater saturated fat intake ( P = .007), and problems with self-monitoring were associated with increased tobacco use ( P = .017). CONCLUSIONS: Executive function difficulties are associated with self-reported engagement in health risk behaviors among young adults with CHDs. Health behavior interventions for adult CHD survivors may require additional modifications that consider executive function problems, particularly difficulties with inhibition.

Effects of Familial Relationships on School Adjustment of Adolescents and Young Adults With Congenital Heart Disease.

BACKGROUND: As more than 85% of patients with congenital heart disease (CHD) have grown to adulthood through improvement in treatment and surgery, the difficulties they experience are expanding into areas related to daily life. Accordingly, adjustment to school in adolescents and young adults (AYAs) with CHD is of increasing interest and is influenced by familial factors. OBJECTIVE: This was a cross-sectional descriptive study to examine the effects of parental positive emotional expressiveness and sibling relationships on school adjustment of AYAs with CHD. METHODS: In this study, a self-reported questionnaire survey was used to collect the data. The participants were 104 AYAs with CHD aged 13 to 21 years who were attending school and had siblings. RESULTS: Maternal positive emotional expressiveness ( r = 0.584, P < .01), paternal positive emotional expressiveness ( r = 0.584, P < .01), and sibling warmth/closeness ( r = 0.478, P < .01) were significantly correlated with school adjustment. However, the results of multiple regression analysis showed that only maternal positive emotional expressiveness (ß = 0.459, P < .05) and sibling warmth/closeness (ß = 0.236, P < .05) were significantly associated with school adjustment. CONCLUSIONS: Adolescents and young adults with CHD who reported higher maternal positive emotional expressiveness and sibling warmth/closeness exhibited better school adjustment. Findings suggest that intervention programs to increase parental positive expressiveness and enhance warmth/closeness of sibling relationships may contribute to improving school adjustment.