Neurodevelopmental Outcomes for Individuals With Congenital Heart Disease: Updates in Neuroprotection, Risk-Stratification, Evaluation, and Management: A Scientific Statement From the American Heart Association.

Over the past decade, new research has advanced scientific knowledge of neurodevelopmental trajectories, factors that increase neurodevelopmental risk, and neuroprotective strategies for individuals with congenital heart disease. In addition, best practices for evaluation and management of developmental delays and disorders in this high-risk patient population have been formulated based on literature review and expert consensus. This American Heart Association scientific statement serves as an update to the 2012 statement on the evaluation and management of neurodevelopmental outcomes in children with congenital heart disease. It includes revised risk categories for developmental delay or disorder and an updated list of factors that increase neurodevelopmental risk in individuals with congenital heart disease according to current evidence, including genetic predisposition, fetal and perinatal factors, surgical and perioperative factors, socioeconomic disadvantage, and parental psychological distress. It also includes an updated algorithm for referral, evaluation, and management of individuals at high risk. Risk stratification of individuals with congenital heart disease with the updated categories and risk factors will identify a large and growing population of survivors at high risk for developmental delay or disorder and associated impacts across the life span. Critical next steps must include efforts to prevent and mitigate developmental delays and disorders. The goal of this scientific statement is to inform health care professionals caring for patients with congenital heart disease and other key stakeholders about the current state of knowledge of neurodevelopmental outcomes for individuals with congenital heart disease and best practices for neuroprotection, risk stratification, evaluation, and management.

Implications of Cardiac Scarring on Body Image Disturbance Among Young Adults With Congenital Heart Defects.

BACKGROUND: Many individuals with congenital heart disease (CHD) undergo open heart surgery, resulting in prominent scarring. However, little research has assessed the impact of surgical scarring on body image in this population. OBJECTIVE: Within this cross-sectional study, associations between body image concerns, anxiety and depression symptoms, and health-related quality of life among individuals with CHD were evaluated. METHODS: Young adults with CHD (N = 138) completed the Body Image Disturbance Questionnaire along with other patient-reported outcome measures. RESULTS: Scores on the Body Image Disturbance Questionnaire were lower (mean, 1.3 ± 0.5) than populations with diabetes amputations, craniofacial conditions, idiopathic scoliosis, and clinical controls. Higher Body Image Disturbance Questionnaire scores were reported among women (P = .003) and were associated with greater anxiety (P = .000) and depression (P = .13) after controlling for sex. CONCLUSIONS: Body image concerns related to cardiac surgery scarring are especially prominent in women and associated with greater emotional distress, suggesting potential clinical relevance for evaluating body image concerns in persons with CHD.

Associations of Impulsivity and Risky Decision-Making with E-Cigarette-Related Outcomes Among Adolescents with Congenital Heart Disease: Variable- and Person-Oriented Approaches.

Adolescents with congenital heart disease (CHD) have elevated risk for acquired cardiovascular complications, increasing their vulnerability to e-cigarette-related health harms. Impulsivity and risky decision-making have been associated with adolescent substance use, but the relationships between these factors and e-cigarette-related outcomes among cardiovascular at-risk adolescents with CHD are unknown. This cross-sectional study aimed to (a) determine the associations of impulsivity and risky decision-making with e-cigarette-related outcomes (i.e. susceptibility, ever use, perceptions of harm and addictiveness) via variable-oriented analysis (logistic regression), (b) identify groups of adolescents with similar profiles of impulsivity and risky decision-making via exploratory person-oriented analysis (latent profile analysis; LPA), and (c) examine differences on e-cigarette-related outcomes between profile groups. Adolescents aged 12 to 18 years with CHD (N = 98) completed a survey assessing impulsivity facets (Short UPPS-P) and e-cigarette-related outcomes and were administered a risky decision-making task (Iowa Gambling Task, Version 2; IGT2). In variable-oriented analyses, impulsivity facets (negative urgency, positive urgency, lack of premeditation) but not risky decision-making were associated with e-cigarette susceptibility and ever use. The exploratory LPA identified two groups with similar patterns of responding on the Short UPPS-P and IGT2 labeled "Low Impulsivity" and "High Impulsivity," which were primarily characterized by significant differences in negative and positive urgency. Adolescents in the High Impulsivity group had increased odds of e-cigarette susceptibility but not ever use compared to the Low Impulsivity group. This work indicates that strategies to prevent e-cigarette use among adolescents with CHD may be enhanced by addressing impulsivity, particularly negative and positive urgency.

PrEP and HIV prevention decision-making among social network members of women who have experienced incarceration: a qualitative study.

ABSTRACTIncarceration and HIV are a syndemic for US women, yet very few women who have experienced incarceration use pre-exposure prophylaxis (PrEP) for HIV. We conducted semi-structured interviews with 32 participants recruited by women who have experienced incarceration from their social networks, informed by the modified social ecological model for PrEP. Emergent themes from the interviews included individual-level (low personal HIV risk assessment, personal responsibility for HIV prevention, and decisions in addiction versus recovery), network-level (influential sex partners and the importance of trust, supportive treatment peers, and high-risk but indifferent drug use networks), community-level (stigma, and mitigation of stigma in supportive substance use disorder treatment environments), and public policy-level (incarceration and PrEP cost and access) determinants. PrEP interventions for women who have experienced incarceration and their networks will need to incorporate contingency planning into HIV risk assessment, navigate complex network dynamics, and be situated in trusted contexts to address structural barriers.

The Association of Global and Disease-Related Stress With Susceptibility to and Use of E-Cigarettes and Marijuana Among Adolescents With Congenital Heart Disease.

OBJECTIVE: Adolescents with congenital heart disease (CHD) are exposed to disease-related stressors and have elevated risk for cardiovascular and cognitive complications that are exacerbated by e-cigarettes and marijuana. The aims of this cross-sectional study are to: (1) identify the association between perceived global and disease-related stress and susceptibility to e-cigarettes and marijuana, (2) determine if the association between stress and susceptibility differs by gender, and (3) explore the association between stress and ever use of e-cigarettes and marijuana among adolescents with CHD. METHODS: Adolescents with CHD (N = 98; aged 12-18 years) completed self-report measures of susceptibility to/ever use of e-cigarettes and marijuana and global and disease-related stress. RESULTS: Susceptibility to e-cigarettes and marijuana was reported by 31.3% and 40.2% of adolescents, respectively. Ever use of e-cigarettes and marijuana was reported by 15.3% and 14.3% of adolescents, respectively. Global stress was associated with susceptibility to and ever use of e-cigarettes and marijuana. Disease-related stress was associated with susceptibility to marijuana. Females reported more global and disease-related stress than males, but the association of stress with susceptibility to e-cigarettes and marijuana did not differ by gender. CONCLUSIONS: Susceptibility to e-cigarettes and marijuana is common among adolescents with CHD and is associated with stress. Future work to examine the longitudinal associations between susceptibility, stress, and use of e-cigarettes and marijuana is warranted. Global stress may be an important consideration in the development of strategies to prevent these risky health behaviors among adolescents with CHD.

The influence of social relationships on PrEP attitudes among women with incarceration experience in the Southeastern USA.

Women who have experienced incarceration face a disproportionately high risk of acquiring HIV. Despite efficacy of pre-exposure prophylaxis (PrEP) for HIV, very few women with incarceration histories are using PrEP. Our objective was to learn how sexual, drug-use and social relationships shape decisions about PrEP among women who have experienced incarceration. We used an inductive approach to analyse data from four focus groups undertaken with women who had previously experienced incarceration recruited from three community-based organisations. We identified public policy (medical distrust, lack of prevention in prisons and jails, and cost and coverage of PrEP); community (incarceration stigma, gossip as prevention); social and sexual network (positive peer and parenting relationships, distrust of sexual partners, and networks as a source of risk); and individual-level (active addiction or recovery, change after incarceration, and medical concerns) factors influencing the use of PrEP. Actions and interventions to improve PrEP uptake among women who have experienced incarceration must take account of the multilevel context of HIV prevention decisions.

Healthcare Use Among Black and White Congenital Heart Disease Medicaid Enrollees.

Congenital heart disease (CHD) is the most common birth anomaly in the US. Research shows lost-to-follow-up trends and racial disparities in healthcare use. This study examines racial differences in healthcare use among Medicaid-covered children with CHD. Using 2010-2019 claims data from a pediatric Medicaid Accountable Care Organization, 960 Black and White children with complex CHD and ≥ 3 years of continuous Medicaid coverage were identified. Three cohorts were constructed (starting age: < 1-year-olds, 1-5-year-olds, 6-15-year-olds) and followed for 3 years. Multivariate analysis assessed annual healthcare use (cardiology, primary care, emergency department) by race, adjusting for patient and provider covariates. Overall, 51% of patients had an annual cardiology visit, and 54% had an annual primary care visit. Among the 1-5-year-old cohort, Black children were predicted to be 13% less likely to have an annual cardiology visit compared to their White counterparts (p = 0.001). Older Black children were predicted to be more likely to have a primary care visit compared to their White counterparts. Nearly half of Medicaid-enrolled children with complex CHD did not receive recommended cardiology care. Young Black children were less likely to receive an annual cardiac visit, while older Black children were more likely to receive primary care. While the percentage with an annual cardiac visit was low, the majority had seen a cardiologist within the 3-year window, suggesting these children are still receiving cardiology care, if less frequently than recommended. Opportunities exist for cardiology and primary care to collaborate to ensure patients receive timely recommended care.

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.

A Randomized Clinical Trial Demonstrating Feasibility and Preliminary Efficacy of a Videoconference-Delivered Physical Activity Lifestyle Intervention Among Adolescents With a Congenital Heart Defect.

BACKGROUND: Individuals with congenital heart defects are at increased risk for developing further cardiovascular complications, which can be mitigated by increasing physical activity. Given that positive health behaviors begin declining during older adolescence, it is vital to promote lifestyle changes in this population. PURPOSE: The current study aims to (a) determine the feasibility/acceptability of the Congenital Heart Disease Physical Activity Lifestyle (CHD-PAL) intervention among adolescents (ages 15-18) with moderate and complex congenital heart defects, and (b) estimate the preliminary efficacy of CHD-PAL for increasing time spent in moderate-to-vigorous physical activity (MVPA) and cardiorespiratory fitness and decreasing sedentary behavior. METHODS: Eligible participants were randomized into either CHD-PAL (eight 30-min videoconferencing sessions over 20 weeks with an interventionist + Fitbit + exercise prescription) or a comparator (Fitbit + exercise prescription). RESULTS: Sixty adolescents were randomized (76% recruitment rate; 94% of participants were retained from baseline to follow-up). Most adolescents (73%) and their parents/guardians (76%) reported that the trial was enjoyable. While there was no effect of arm on change in MVPA, sedentary behavior, or cardiorespiratory fitness for the entire sample, among those who engaged in <21 min of MVPA on average at baseline, adolescents in the CHD-PAL intervention had an increase of 16 min/day of MVPA more than comparators (d = 0.90). CONCLUSIONS: The CHD-PAL intervention warrants examination in a larger trial to establish efficacy among those adolescents with a congenital heart defect who engage in <21 min of MVPA/day and should include follow-up assessments to examine effect durability. CLINICAL TRIALS REGISTRATION: NCT03335475.

Gender Differences in Physical Activity Engagement Among Adolescents With Congenital Heart Disease.

OBJECTIVE: The current study aimed to (a) describe moderate-to-vigorous physical activity (MVPA), sedentary behavior (SB), and cardiorespiratory fitness (VO2Peak) via objective assessment among adolescents with congenital heart disease (CHD), (b) examine gender differences on MVPA, SB, VO2Peak, and the Theory of Planned Behavior elements, and (c) identify whether gender moderates the relationships between the Theory of Planned Behavior elements and MVPA, SB, and VO2Peak. METHODS: Adolescent CHD survivors (N = 86; ages 15-18 years) wore an accelerometer to assess MVPA and SB, underwent an exercise stress test to assess VO2Peak, and completed a survey of the Theory of Planned Behavior elements as measured by perceived benefits (attitudes), family/friend support and perceived norms (social norms), and self-efficacy and barriers (perceived behavioral control) to engaging in physical activity. RESULTS: On average, CHD survivors engaged in 22.3 min (SD = 15.3) of MVPA/day and 9 hr of SB/day (M = 565.8, SD = 102.5 min). Females engaged in less MVPA but not more SB had a lower mean VO2Peak, reported lower self-efficacy, and perceived greater barriers than males. In a regression model, barriers explained unique variance in MVPA and VO2Peak, but the relationship between barriers and MVPA/VO2Peak did not vary by gender. Self-efficacy did not explain unique variance in MVPA and VO2Peak when included in a model with gender and barriers. CONCLUSIONS: Family/friend support for physical activity engagement may be an important consideration when developing physical activity interventions for adolescent CHD survivors. The role of gender differences in self-efficacy and perceived barriers on physical activity engagement warrants further investigation.

Phenotypes of adults with congenital heart disease around the globe: a cluster analysis.

OBJECTIVE: To derive cluster analysis-based groupings for adults with congenital heart disease (ACHD) when it comes to perceived health, psychological functioning, health behaviours and quality of life (QoL). METHODS: This study was part of a larger worldwide multicentre study called APPROACH-IS; a cross sectional study which recruited 4028 patients (2013-2015) from 15 participating countries. A hierarchical cluster analysis was performed using Ward's method in order to group patients with similar psychological characteristics, which were defined by taking into consideration the scores of the following tests: Sense Of Coherence, Health Behavior Scale (physical exercise score), Hospital Anxiety Depression Scale, Illness Perception Questionnaire, Satisfaction with Life Scale and the Visual Analogue Scale scores of the EQ-5D perceived health scale and a linear analogue scale (0-100) measuring QoL. RESULTS: 3768 patients with complete data were divided into 3 clusters. The first and second clusters represented 89.6% of patients in the analysis who reported a good health perception, QoL, psychological functioning and the greatest amount of exercise. Patients in the third cluster reported substantially lower scores in all PROs. This cluster was characterised by a significantly higher proportion of females, a higher average age the lowest education level, more complex forms of congenital heart disease and more medical comorbidities. CONCLUSIONS: This study suggests that certain demographic and clinical characteristics may be linked to less favourable health perception, quality of life, psychological functioning, and health behaviours in ACHD. This information may be used to improve psychosocial screening and the timely provision of psychosocial care.

Patient decision aid for trial of labor after cesarean (TOLAC) versus planned repeat cesarean delivery: a quasi-experimental pre-post study.

OBJECTIVE: To assess the impact of a web-based decision aid on patient-centered decision making outcomes among women considering a trial of labor after cesarean (TOLAC) versus planned repeat cesarean delivery. METHODS: The Birth Decision Aid Study (B-READY) was a quasi-experimental pre-post study of two sequential cohorts. From June 18, 2018 to July 31, 2019, 50 women were enrolled in routine care, followed by 50 women who were enrolled in the decision aid group. Inclusion criteria were singleton pregnancies between 19/0 to 36/6 weeks, ≤2 prior cesareans, and no contraindications to TOLAC. The decision aid group viewed the online Healthwise® "Pregnancy: Birth Options After Cesarean" program. Both groups received the same birth options counseling and completed the same online assessment. Primary patient-centered outcomes were knowledge about birth options and shared decision making at online assessment, and informed, patient-centered decision making about her preferred mode of delivery at delivery admission. RESULTS: Among 100 women participated in this study (50 per group), the mean gestational age at enrollment was 31 weeks, and 71% or 63/89 women who consented to delivery data abstraction had a cesarean delivery. Women in the patient decision aid group gained more knowledge (defined as score ≥ 75%) about birth options compared to those in the routine care group (72% vs. 32%; adjusted odds ratio, AOR: 6.15 [95% CI: 2.34 to 16.14]), and were more likely to make an informed, patient-centered decision (60% vs. 26%; AOR: 3.30 [95% CI: 1.20 to 9.04]. Women in both groups reported similar involvement in shared decision making, as well as satisfaction and values. More than 90% of decision aid users reported it was a useful tool and would recommend it to other TOLAC-eligible women. CONCLUSIONS: A web-based birth options patient-centered decision aid for TOLAC eligible women can be integrated into prenatal Telehealth and may improve the quality of decision making about mode of delivery. TRIAL REGISTRATION: The study was registered with ClinincalTrials.gov and the ID# was NCT04053413 . Registered 12 August 2019 - Retrospectively registered.

Smoking among adult congenital heart disease survivors in the United States: Prevalence and relationship with illness perceptions.

The relationship between smoking and illness perceptions among congenital heart disease (CHD) survivors is unknown. The primary aims of the present study were to compare the smoking prevalence among CHD survivors to a nationally representative U.S. sample and examine the relationship between smoking and illness perceptions. CHD survivors (N = 744) from six U.S. sites participated in the study. The smoking prevalence among CHD survivors (9.3%) was lower than the general population (15.3%). However, 23.3% of CHD survivors with severe functional limitations smoked. Smoking prevalence differed by U.S. region, with a greater proportion of those attending CHD care in the Midwest reporting smoking (11.8%). The illness perception dimensions of Concern and Emotional Response were independently associated with smoking. Differences in illness perceptions enhance our understanding of smoking among CHD survivors and may guide interventions promoting positive health behaviors. The protocol for the study from which the present analyses were conducted was recorded at ClinicalTrials.gov: NCT02150603.

Perceptions About Lactation Consultant Support, Breastfeeding Experiences and Postpartum Psychosocial Outcomes.

INTRODUCTION: Many women seek lactation consultant support in the postpartum period. Lactation consultant support in community or clinical settings is often assumed to extend breastfeeding duration, improve breastfeeding experiences, and be well-received. Few studies have assessed women's perceptions of the support they received, nor have perceptions been examined in relationship to breastfeeding outcomes and maternal well-being. Our objective was to characterize the lactation consultant support women received and examine how women's perceptions about the support related to their breastfeeding outcomes, anxiety and depressive symptoms, and parenting stress. METHODS: This observational, cross-sectional study examined receipt of postpartum lactation consultant support among 210 US women. Perceptions of lactation consultant support were examined in relation to breastfeeding outcomes, anxiety and depressive symptoms, and parenting stress to explore outcomes of negative versus positive lactation consultant support experiences, using linear and proportional hazards regression. RESULTS: While overall perceptions of lactation consultant support were positive for most recipients (71%, n = 98), 29% (n = 40) reported negative perceptions of lactation consultant support. Negative perceptions were associated with lower breastfeeding self-efficacy (ß = - 11.7, 95% CI - 17.3, - 6.0), a less successful breastfeeding experience (ß = - 19.5, CI - 27.8, - 11.3), greater general anxiety (ß = 6.5, CI 2.1, 10.9), and shorter total duration of milk production (HR = 0.39, 95% CI 0.18, 0.84). Perceptions were not associated with depressive symptoms or parenting stress. DISCUSSION: Findings highlight the importance of ensuring that postpartum breastfeeding support provided by lactation consultants is perceived as positive by women.

Health-Related Quality of Life Declines Over 3 Years for Congenital Heart Disease Survivors.

BACKGROUND: Because of medical advancements, many congenital heart disease (CHD) survivors are relatively symptom-free until adulthood, at which time complications may occur. Worsening health status likely drives a change in patient-reported outcomes, such as health-related quality of life (HRQoL), although change in HRQoL has not been investigated among adolescent and young adult CHD survivors. OBJECTIVE: The aims of the current mixed cross-sectional and longitudinal study were to (1) examine changes in HRQoL over 3 years and (2) identify any demographic (age, sex, estimated family income, and distance from medical center) and medical predictors (functional status and number of cardiac-related medications) of that change. METHODS: Baseline and 3-year follow-up data were obtained via an online survey of 172 CHD survivors (15-39 years old at baseline; 25% simple, 45% moderate, 30% complex) recruited from a pediatric hospital and an adult hospital. Medical predictors were abstracted from electronic medical records. RESULTS: After controlling for New York Heart Association functional class, mixed-effects models identified significant declines in all subscales of the Research and Development Corporation 36-Item Health Survey 1.0 across the 3-year timeframe. A lower estimated family income (≤$35 000) predicted more decline in physical functioning (b = 0.5, 95% confidence interval, 0.2-0.8; P = .001) and emotional functioning (b = 0.3, 95% confidence interval, 0.1-0.5; P = .017). No other significant demographic or medical predictors were identified. CONCLUSIONS: Study findings highlight the importance of tracking patient-reported outcomes over time, suggesting that medical staff should discuss HRQoL with CHD survivors during late adolescence and early adulthood before decline.

Healthcare system inputs and patient-reported outcomes: a study in adults with congenital heart defect from 15 countries.

BACKGROUND: The relationship between healthcare system inputs (e.g., human resources and infrastructure) and mortality has been extensively studied. However, the association between healthcare system inputs and patient-reported outcomes remains unclear. Hence, we explored the predictive value of human resources and infrastructures of the countries' healthcare system on patient-reported outcomes in adults with congenital heart disease. METHODS: This cross-sectional study included 3588 patients with congenital heart disease (median age = 31y; IQR = 16.0; 52% women; 26% simple, 49% moderate, and 25% complex defects) from 15 countries. The following patient-reported outcomes were measured: perceived physical and mental health, psychological distress, health behaviors, and quality of life. The assessed inputs of the healthcare system were: (i) human resources (i.e., density of physicians and nurses, both per 1000 people) and (ii) infrastructure (i.e., density of hospital beds per 10,000 people). Univariable, multivariable, and sensitivity analyses using general linear mixed models were conducted, adjusting for patient-specific variables and unmeasured country differences. RESULTS: Sensitivity analyses showed that higher density of physicians was significantly associated with better self-reported physical and mental health, less psychological distress, and better quality of life. A greater number of nurses was significantly associated with better self-reported physical health, less psychological distress, and less risky health behavior. No associations between a higher density of hospital beds and patient-reported outcomes were observed. CONCLUSIONS: This explorative study suggests that density of human resources for health, measured on country level, are associated with patient-reported outcomes in adults with congenital heart disease. More research needs to be conducted before firm conclusions about the relationships observed can be drawn. TRIAL REGISTRATION: ClinicalTrials.gov: NCT02150603. Registered 30 May 2014.

Differences in Obesity Among Black and White Children, Adolescents, and Adults With Congenital Heart Disease.

BACKGROUND: Congenital heart disease (CHD) survivors have an elevated risk for obesity-related comorbidities, but little is known about racial differences in obesity rates for this population. OBJECTIVE: The authors aimed to compare rates of obesity in CHD survivors to national estimates using National Health and Nutrition Examination Assessment Survey (NHANES) and to characterize racial disparities in obesity among CHD survivors across age ranges. METHODS: Retrospective chart review included 4496 CHD survivors (4050 white and 446 black) with a range of lesion severities from a pediatric and an adult medical center. RESULTS: White children with CHD had a higher prevalence of obesity compared with NHANES estimates. In contrast, white young adults with CHD had a lower prevalence of obesity compared with NHANES. Blacks with CHD had a 58% increased risk of obesity in young adulthood and a 33% increased risk in late adulthood compared with whites with CHD. CONCLUSIONS: Obesity interventions are needed among CHD survivors across the lifespan, particularly among adult non-Hispanic blacks.

Executive Function and Internalizing Symptoms in Adolescents and Young Adults With Congenital Heart Disease: The Role of Coping.

Objective: Executive functioning deficits have been documented among congenital heart disease (CHD) survivors and may contribute to emotional distress. Little research has investigated the role of coping in this association. This study examined the role of coping in accounting for the association between self-reported executive function problems and internalizing symptoms among adolescents and emerging adults (AEAs), as well as young adults (YAs) with CHD. Methods: Participants included 74 AEA (Mage = 19.32 ± 3.47 years, range 15-25 years) and 98 YA CHD survivors (Mage = 32.00 ± 3.69 years, range 26-39 years), recruited from pediatric and adult outpatient cardiology clinics. Participants completed self-report measures of executive function problems, coping (primary control, secondary control, and disengagement coping), and internalizing symptoms. Lesion severity classification and functional impairment due to symptoms of heart failure were determined from medical chart review. Results: Significant problems in executive function were reported by 5% of AEA and 13% of YA. Coping was not associated with executive function problems or internalizing symptoms for AEA. However, among YA, less use of adaptive coping strategies and more maladaptive coping responses was associated with both more executive function problems and internalizing symptoms. An indirect effect of executive function problems on internalizing symptoms via secondary control coping emerged for YA. Conclusions: Executive function problems may disrupt the ability to use important adaptive coping skills, such as cognitive reappraisal, positive thinking, and acceptance, thereby resulting in greater emotional distress among YA CHD survivors.

Adolescents and Young Adults Living with Congenital Heart Disease: Coping, Stress Reactivity, and Emotional Functioning.

Living with congenital heart disease (CHD) presents survivors with numerous stressors, which may contribute to emotional problems. This study examined (a) whether coping with CHD-related stress predicts symptoms of depression and anxiety, and (b) whether associations between coping and emotional distress are moderated by involuntary stress reactivity. Adolescents and young adults diagnosed with CHD (Mage = 26.4) were recruited from pediatric and adult outpatient cardiology clinics. Participants (N = 168) completed online self-report measures. Hierarchical multiple regression analyses revealed that secondary control coping (e.g., cognitive restructuring, positive thinking) predicted lower depression and anxiety. Primary control coping (e.g., problem-solving) and stress reactivity (e.g., rumination, emotional numbing) interacted to predict depression and anxiety: the higher individuals were in involuntary stress reactivity, the stronger was the association between primary control coping and lower depression and anxiety. These results can inform clinical efforts to prevent or reduce emotional distress among CHD survivors.

Regional variation in quality of life in patients with a Fontan circulation: A multinational perspective.

BACKGROUND: Impaired quality of life (QOL) is associated with congenital heart disease (CHD) and country of residence; however, few studies have compared QOL in patients with differing complexities of CHD across regional populations. The current study examined regional variation in QOL outcomes in a large multinational sample of patients with a Fontan relative to patients with atrial septal defects (ASDs) and ventricular septal defects (VSDs). METHODS: From the Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease-International Study (APPROACH-IS), 405 patients (163 Fontan and 242 ASD/VSD) across Asia, Europe, and North America provided consent for access to their medical records and completed a survey evaluating QOL (0 to 100 linear analog scale). Primary CHD diagnosis, disease complexity, surgical history, and documented history of mood and anxiety disorders were recorded. Differences in QOL, medical complications, and mood and anxiety disorders between Fontan and ASD/VSD patients, and across geographic regions, were examined using analysis of covariance. Hierarchical regression analyses were conducted to identify variables associated with the QOL ratings. RESULTS: Patients with a Fontan reported significantly lower QOL, and greater medical complications and mood and anxiety disorders relative to patients with ASD/VSD. Inpatient cardiac admissions, mood disorders, and anxiety disorders were associated with lower QOL among patients with a Fontan, and mood disorders were associated with lower QOL among patients with ASD/VSD. Regional differences for QOL were not observed in patients with a Fontan; however, significant differences were identified in patients with ASD/VSD. CONCLUSIONS: Regional variation of QOL is commonplace in adults with CHD; however, it appears affected by greater disease burden. Among patients with a Fontan, regional variation of QOL is lost. Specific attempts to screen for QOL and mood and anxiety disorders among CHD patients may improve the care of patients with the greatest disease burden.