Clinician Perceptions of Family-Centered Care in Pediatric and Congenital Heart Settings.

Importance: Family-centered care recognizes families as central to child health and well-being and prioritizes clinician collaboration with families to ensure optimal pediatric care and outcomes. Clinician interpersonal sensitivity and communication skills are key to this approach. Objective: To examine perceptions of and factors associated with family-centered care among clinicians working in pediatric and congenital heart care. Design, Setting, and Participants: In this cross-sectional study, participants from diverse clinical disciplines (pediatric cardiology, cardiothoracic surgery, nursing, anesthesia, neonatology, intensive care, psychology, and others), completed an online survey between June 2020 and February 2021. Participants included physicians, surgeons, nurses, and allied and mental health professionals at an Australian quaternary pediatric hospital network. Statistical analysis was performed from August 2022 to June 2023. Main Outcomes and Measures: Family-centered care across 4 domains (showing interpersonal sensitivity, treating people respectfully, providing general information, and communicating specific information) was measured using the validated Measure of Processes of Care for Service Providers. Clinician burnout (emotional exhaustion, depersonalization, and personal accomplishment), confidence responding to families' psychosocial needs, and psychological, clinical role, and sociodemographic factors were also assessed. Informed by theory, hierarchical linear regression was used to identify factors associated with family-centered care. Results: There were 212 clinicians (177 women [84.3%]; 153 nurses [72.2%], 32 physicians [15.1%], 22 allied and mental health professionals [10.4%], 5 surgeons [2.3%]; 170 [80.2%] aged 20-49 years) who participated (55% response rate). Of the 4 family-centered care domains, scores for treating people respectfully were highest and associated with greater clinician confidence responding to families' psychosocial needs (effect size [ß], 0.59 [95% CI, 0.46 to 0.72]; P < .001), lower depersonalization (ß, 0.04 [95% CI, -0.07 to -0.01]; P = .02), and a greater sense of personal accomplishment at work (ß, 0.02 [95% CI, 0.01 to 0.04]; P = 0.04). Greater interpersonal sensitivity was associated with greater confidence responding to families' psychosocial needs (ß, 0.80 [95% CI, 0.62 to 0.97]; P < .001), a greater sense of personal accomplishment at work (ß, 0.03 [95% CI, 0.01 to 0.05]; P = .04), and lower use of approach-based coping, such as problem-solving (ß, 0.37 [95% CI, -0.71 to -0.02]; P = .04). Conclusions and Relevance: In this cross-sectional study, burnout and confidence responding to families' psychosocial needs were associated with clinicians' perceptions of family-centered care. These findings suggest that targeted interventions to address these factors may benefit clinicians and also potentially strengthen the practice of family-centered care in pediatric and congenital heart settings.

What does good care look like to people living with congenital heart disease in the 21st century? Qualitative online, asynchronous discussion forums.

OBJECTIVES: As part of a wider study, our aim was to elicit perspectives of people with congenital heart disease (CHD) and/or their parents/carers about their experiences of healthcare and what is important to them when receiving care. DESIGN AND SETTING: A qualitative study involving a series of closed, asynchronous, online discussion forums underpinned by an interpretivist framework and set up and moderated by three patient charities via their Facebook pages. PARTICIPANTS: People with CHD and parents/carers of people with CHD from the UK. RESULTS: Five forums were run for 12-24 weeks across the three charities, and 343 participants signed up to the forums. Four linked themes related to processes of care were identified following thematic analysis of the transcripts: relationships and communication; access and coordination; experience of discrete episodes of care and psychological support. These impacted how care was experienced and, for some patients, outcomes of CHD and its treatment as well as broader health outcomes. In addition, context relating to stages of the patient journey was described, together with patient-related factors such as patients' knowledge and expertise in their own condition. CONCLUSIONS: People with CHD and their parents/carers want individualised, person-centred care delivered within an appropriately resourced, multidisciplinary service. Although examples of excellent care were provided it is evident that, from the perspective of patients and parents/carers, some National Health Service Standards for people with CHD were not being met.

Quality of Life and Well-Being in Adults With Fontan Physiology: Findings From the Australian and New Zealand Fontan Registry Quality of Life Study.

BACKGROUND: To characterize global and health-related quality of life (QOL) among adults with Fontan physiology enrolled in the Australian and New Zealand Fontan Registry (ANZFR), and identify sociodemographic, clinical, psychological, and relational factors associated with outcomes. METHODS AND RESULTS: Using a cross-sectional survey design, 66 adults with Fontan physiology (58% women; mean age, 29.6±7.7 years; range, 18-50 years) completed validated self-report measures. Health-related QOL was assessed using the Pediatric Quality of Life Inventory, and global QOL was assessed using a visual analog scale (0-10). Participants reported lower total health-related QOL (P<0.001), as well as lower physical (P<0.001) and social (P=0.002) functioning compared with normative data. Median global QOL was 7.0 (interquartile range: 5.0-8.0) and most participants (71%) rated their QOL ≥6. For health-related QOL, age, sex, university education, and length of hospital stay in the past 12 months explained 27% of the variance in scores, while general psychological stress, medical traumatic stress, communication problems, and access to emotional support explained a further 44% of variance (final model: 71% of variance explained). For global QOL, sociodemographic and clinical factors explained 20% of the variance in scores, while psychological stress and sense of coherence explained a further 24% (final model: 44% of variance explained). CONCLUSIONS: Adults with Fontan physiology reported lower overall health-related QOL compared with community-based norms. Variance in QOL outcomes were predominantly attributable to psychological and relational factors. Tailored screening and assessment to identify Fontan patients at greatest risk of lower QOL, and a proactive approach to supportive care, are needed.

Efficacy of a Transition Program in Adolescents and Young Adults With Congenital Heart Disease: The TRANSITION-CHD Randomized Controlled Trial.

PURPOSE: Currently, nearly 90% of patients with congenital heart disease (CHD) reach adulthood in relatively good health. Structured transition programs have emerged to support adolescents and young adults in transitioning to adult care structures, improve their autonomy, and limit healthcare ruptures. The TRANSITION-CHD randomized controlled trial aimed to assess the impact of a transition program on health-related quality of life (HRQoL) in adolescents and young adults with CHD. METHODS: From January 2017 to February 2020, 200 subjects with a CHD, aged 13-25 years, were enrolled in a prospective, controlled, multicenter study and randomized in two balanced groups (transition program vs. standard of care). The primary outcome was the change in PedsQL self-reported HRQoL score between baseline and 12-month follow-up, using an intention-to-treat analysis. The secondary outcomes were the change in disease knowledge, physical health (cardiopulmonary fitness, physical activity), and mental health (anxiety, depression). RESULTS: The change in HRQoL differed significantly between the transition group and the control group (mean difference = 3.03, 95% confidence interval (CI) = [0.08; 5.98]; p = .044; effect size = 0.30), in favor of the intervention group. A significant increase was also observed in the self-reported psychosocial HRQoL (mean difference = 3.33, 95% CI = [0.01; 6.64]; p = .049; effect size = 0.29), in the proxy-reported physical HRQoL (mean difference = 9.18, 95% CI = [1.86; 16.51]; p = .015; effect size = 0.53), and in disease knowledge (mean difference = 3.13, 95% CI = [1.54; 4.72]; p < .001; effect size = 0.64). DISCUSSION: The TRANSITION-CHD program improved HRQoL and disease knowledge in adolescents and young adults with CHD, supporting the generalization and systematization of similar preventive interventions in pediatric and congenital cardiology.

Beyond the heart: Cognitive and verbal outcomes in Arab children with congenital heart diseases.

BACKGROUND: This study investigates how congenital heart diseases (CHD) characteristics and interventions affect cognitive and verbal skills in Arab children, while also uncovering previously unexplored connections between these skills and the quality of life (QoL) scores as perceived by both children and parents. METHODS: A cross-sectional study was conducted in Jordan, involving 62 children with CHD aged 6-16. Data were collected through standardized intelligence tests (namely The Raven's Progressive Matrices Test and The Wechsler Intelligence Scale for Children) and QoL assessments. RESULTS: Sex, disease severity, cyanosis, CHD defect status, conducted operations, and types of interventions did not significantly influence cognitive scores. However, a significant difference was observed in Wechsler's scores between cyanotic and non-cyanotic children (p < .01) and between severe and moderate cases (p = .01). Further, a significant positive correlation was identified between Wechsler's Scores and QoL reported by parents (r = 0.33, p < .01). This correlation was particularly pronounced in the social and school functioning dimensions of QoL. CONCLUSIONS: This study highlights the need for personalized care approaches for children with CHDs based on their individual characteristics. While cognitive abilities did not directly correlate with children's QoL reports, a significant positive correlation between verbal skills and QoL reported by parents underscores the importance of effective communication in assessing a child's overall well-being. Future research should further examine the cognitive development in this population, employing neurocognitive investigations and longitudinal studies to gain a deeper understanding of their cognitive profiles and trajectories.

Prioritizing family-centered developmental care: insights from parents of children with critical congenital heart disease: a qualitative study.

As survivors of early cardiac surgery are at high risk of neurodevelopmental impairments, systematic health observations of children with critical congenital heart disease (CCHD) throughout childhood are recommended to enable early diagnosis and offer interventions to optimize neurodevelopment. A qualitative study using thematic analysis was performed to explore parents' concerns, experiences, and needs regarding the development and received developmental care of their child (0-10 years) during hospital admission and beyond. Data were collected using semi-structured online interviews with 20 parents of children with CCHD. Four major themes were identified: (1) "impact of diagnosis and disease on the family-system," (2) "parental concerns from diagnoses and beyond," (3) "the need for information," and (4) "the need for individualized and family-centered care." The main themes can be divided into 13 sub-themes as impact, concerns, and needs are influenced by various impactful moments from diagnosis and afterwards.     Conclusion: This study confirms the importance of early identification of neurodevelopmental problems by experienced healthcare professionals, especially in the early years when parental expectations and concerns about their child's neurodevelopment are lower. A tailor-made family-centered follow-up program should be offered, which pays attention to both the neurodevelopment of patients with CCHD as well as the mental wellbeing of the entire family system. Furthermore, an online portal is recommended with a variety of reliable, controlled, understandable information from which parents can obtain the desired information to understand better the consequences of specific heart condition and to provide their child with the best possible guidance. What is Known: • Survivors of early cardiac surgery are at high risk of neurodevelopmental impairments; systematic health observations of children with CCHD throughout childhood are strongly recommended. What is New: • Parents need a tailor-made family-centered follow-up program, which pays attention to both the neurodevelopment of patients with CCHD as well as the mental wellbeing of the entire family system. • An online portal offering diverse, trustworthy information and sources would effectively meet parents' needs by providing accessible insights into the potential consequences of specific heart conditions and guiding them in supporting their child optimally.

Development and validation of a questionnaire to measure the congenital heart disease of children's family stressor.

Background: Families of children with congenital heart disease (CHD) face tremendous stressors in the process of coping with the disease, which threatens the health of families of children with CHD. Studies have shown that nursing interventions focusing on family stress management can improve parents' ability to cope with illness and promote family health. At present, there is no measuring tool for family stressors of CHD. Methods: The items of the scale were generated through qualitative interviews and a literature review. Initial items were evaluated by seven experts to determine content validity. Factor analysis and reliability testing were conducted with a convenience sample of 670 family members. The criterion-related validity of the scale was calculated using scores on the Self-Rating Anxiety Scale (SAS). Results: The CHD Children's Family Stressor Scale consisted of six dimensions and 41 items. In the exploratory factor analysis, the cumulative explained variance of the six factors was 61.085%. In the confirmatory factor analysis, the six factors in the EFA were well validated, indicating that the model fits well. The correlation coefficient between CHD Children's Family Stressor Scale and SAS was r = 0.504 (p < 0.001), which indicated that the criterion-related validity of the scale was good. In the reliability test, Cronbach's α coefficients of six sub-scales were 0.774-0.940, and the scale-level Cronbach's α coefficient value was 0.945. Conclusion: The study indicates that the CHD Children's Family Stressor Scale is valid and reliable, and it is recommended for use in clinical practice to assess CHD children's family stressors.

Financial Stressors for Parents of Children and Emerging Adults With Congenital Heart Disease: A Qualitative Study.

INTRODUCTION: Congenital heart disease (CHD) is the most prevalent congenital disability globally. This study aimed to describe parents' perspectives on financial stressors related to having a child with CHD using a descriptive qualitative approach. METHOD: Qualitative data were obtained from parents of children with CHD in a cross-sectional web-based survey study. Iterative data analysis was used to develop essential themes that enabled a rich description of 147 parents' perspectives. RESULTS: Parents identified five financial stressors: perpetual worries about health insurance, facing the dilemma of "making too much money," struggling to balance work, worrying over having an emerging adult with CHD, and constant constraints because of financial needs. DISCUSSION: As experts in pediatric care, pediatric advanced practice providers need to work with policymakers to provide further financial assistance and sufficient insurance coverage for families that struggle to balance finances for the whole family and children with CHD.

Subjective experience of parent-child relationship in adolescents with congenital heart disease: A qualitative study.

PURPOSE: To explore the parent-child relationship through the subjective experience of adolescents with congenital heart disease (CHD). DESIGN AND METHODS: A descriptive phenomenology approach was adopted. Twelve adolescents aged from 12 to 18 years with CHD were recruited from the pediatric cardiology clinics at two medical centers in Taiwan. Data were collected through in-depth interviews. Data were analyzed using Colaizzi's phenomenological analysis method, and results were reported in accordance with the Consolidated Criteria for Reporting Qualitative Research guidelines. RESULTS: The experiences of the adolescents with CHD revealed five themes: 1. the enhancement of self-worth through parents' love; 2. the importance of parental support in desperate situations; 3. the development of a sense of security through mutual understanding; 4. growth under parental expectations; and 5. parental overcontrol disguised as love. CONCLUSIONS: The parent-child relationship encompasses both positive and negative experiences. Adolescents prioritize their relationship with parents over that with peers. PRACTICE IMPLICATIONS: Nurses caring for adolescents with CHD can improve care by recognizing the influence of parental love, support in challenges, mutual understanding, parental expectations, and potential negative consequences of overcontrol. This insight guides effective guidance for adolescents, enhancing parent-child interactions and overall well-being.

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.

Salivary Diurnal Cortisol Predicts Post-Traumatic Stress Symptoms in Parents of Infants With Congenital Heart Disease.

BACKGROUND: Parents of infants born with congenital heart disease (CHD) who require open heart surgery after birth are at risk for prolonged psychological distress. Even after their infants are discharged, parents may experience anxiety, depressive, and post-traumatic stress (PTS) symptoms; yet, it is unclear which parents are at greater risk for ongoing symptoms. The purpose of this study was to explore whether measures of the biomarker cortisol in parents during their infants' postoperative period were associated with subsequent psychological distress symptoms at three-month post discharge. METHODS: This was a prospective, longitudinal exploratory study of 40 parents of infants with CHD after open heart surgery using consecutive enrollment. Parents provided diurnal saliva samples for two consecutive days in the postoperative period. Six predictors were summarized and generated including waking cortisol, bedtime cortisol, cortisol awaking response, area under curve with respect to the ground (AUCg), cortisol index, and cortisol slope. Self-report outcome measures on anxiety, depressive, and PTS symptoms were collected three-months post-discharge. Linear mixed models examined the associations between each predictor and each outcome while accounting for within-dyad variance using an unstructured covariance matrix. RESULTS: Cortisol AUCg was a predictor of PTS at three-months post-discharge (ß = .34, p = .03, Cohen's d = 2.05). No significant relationships were found with the other cortisol measures. CONCLUSIONS & IMPLICATIONS: Findings suggest that cortisol area under curve may help to identify parents at risk for increased PTS in the months following their infants' hospitalization for cardiac surgery, serving as a foundation for future study in this area.

Adolescentes y jóvenes portadores de cardiopatías congénitas en etapa de transferencia a la atención médica de adultos / Adolescents and young adults with congenital heart defects in transfer to adult care

Resumen: Introducción: Las cardiopatías congénitas (CC), la malformación congénita más frecuente, han experimentado un aumento de sobrevida y crecimiento exponencial de la población de adolescentes y adultos portado res de CC. Para el éxito a largo plazo urgen intervenciones que optimicen la transición de cuidados de salud desde los servicios pediátricos a los de adulto. Objetivo: Describir el conocimiento y manejo de la enfermedad, autoeficacia y calidad de vida en adolescentes y jóvenes con CC en periodo de transfe rencia en dos hospitales en Santiago de Chile. Pacientes y Método: Estudio no experimental, descrip tivo, de corte transversal. Se aplicó: a) Encuesta de datos sociodemográficos, conocimiento y manejo de su enfermedad y uso de servicios de salud; b) Escala Con-Qol de Calidad de Vida Relacionada con Salud (CVRS) en pacientes con CC y c) Escala de Autoeficacia Generalizada (EAG). Resultados: Se obtuvo una muestra de 51 pacientes, 53% hombres, edad promedio de 17 ± 2,49 años. El 22% de las CC fue de complejidad simple, 29% moderada y 49% alta. Presentaron alta autoeficacia y buenos niveles de calidad de vida, sin embargo, mostraron escaso conocimiento y manejo de su enfermedad cardiaca. Conclusiones: Destaca la poca preparación para lograr una transición exitosa a servicios de adultos y jóvenes portadores de CC, siendo fundamental implementar programas de transición centrados en educación, autocuidado y automanejo de la enfermedad.

Abstract: Introduction: Congenital heart defects (CHD), the most frequent congenital malformations, have shown an in creased survival and exponential growth of the adolescent and adult population living with CHD. Interventions that optimize the transition of patients from pediatric to adult health care services are essential for ensuring positive long-term outcomes. Objective: To describe the knowledge and management of this disease, self-efficacy, and quality of life of young people with CHD during the transition period in two hospitals in Santiago, Chile. Patients and Method: Non-experimental, des criptive, cross-sectional study. Patients completed: a) a survey of socio-demographic data, knowledge and management of their condition, and use of health services; b) the Health-Related Quality of Life (Con-HRQoL) Scale in patients with CHD; and c) the Generalized Self-Efficacy (GSE) Scale. Re sults: We obtained a sample of 51 patients, 53% of them were men, and the mean age was 17 ± 2.49 years. The complexity of the CHD was mild in 22%, moderate in 29%, and high in 49%. Although patients reported high self-efficacy and good levels of quality of life, there was a lack of knowledge and self-management of their heart disease. Conclusions: The study showed that adolescents and young people with CHD are not prepared to achieve a successful transition to adult health care services, and there is a need for the implementation of transition programs focused on education, self-care, and self-management of the disease.

Functional capacity in children and adolescents with congenital heart disease / Capacidade funcional em crianças e adolescentes com cardiopatia congênita

ABSTRACT Objective: To evaluate the physical activity level and functional capacity of children and adolescents with congenital heart disease and to describe correlations between functionality, surgical and echocardiographic findings, metabolic and inflammatory profile and differences between acyanotic and cyanotic heart defects. Methods: A cross-sectional study including children and adolescents with congenital heart disease between six and 18 years old that were evaluated with the 6-minute walk test (6MWT) to assess functional capacity. The short version form of the International Physical Activity Questionnaire (IPAQ) was performed to evaluate physical activity levels. Also, echocardiography and blood collection, to evaluate the metabolic (blood glucose, lipids, insulin) and inflammatory markers (C-reactive protein), were assessed. Results: Twenty-five individuals were evaluated. Of them, 14 had acyanotic heart defects and 11 cyanotic heart defects. Mean age was 12.0±3.7 years, and 20 (80%) were male. IPAQ showed that six (24%) individuals were very active, eight (32%) were active, nine (36%) had irregular physical activity, and two (8%) were sedentary. The mean distance walked in the 6MWT, considering all studied individuals, was 464.7±100.4 m, which was 181.4±42.0 m less than the predicted (p=0.005). There was a positive correlation between Z score 6MWT and the number of surgical procedures (r=-0.455; p=0.022). Conclusions: Children and adolescents with congenital heart disease have low functional capacity, but they are not completely sedentary.

RESUMO Objetivo: Avaliar o nível de atividade física e a capacidade funcional de crianças e adolescentes com cardiopatia congênita, além de descrever correlações entre funcionalidade, achados cirúrgicos e ecocardiográficos, perfil metabólico e inflamatório e diferenças entre cardiopatias congênitas acianótica e cianótica. Métodos: Estudo transversal com crianças e adolescentes com cardiopatia congênita entre seis e 18 anos de idade. Foi realizado o teste de caminhada de 6 minutos para avaliar a capacidade funcional, e aplicou-se a versão curta do Questionário Internacional de Atividade Física (IPAQ) para avaliar os níveis de atividade física. Foram feitos também: exame ecocardiográfico, coleta de sangue para avaliação de perfil metabólico e inflamatório (glicemia, triglicerídeos, colesterol total, lipoproteína de alta densidade - HDL-colesterol, lipoproteína de baixa densidade - LDL-colesterol, hemograma completo, proteína C reativa, insulina). Resultados: Foram avaliados 25 indivíduos, dos quais 14 tinham cardiopatia congênita acianótica e 11 cianótica. A média de idade foi de 12,0±3,7 anos, e 20 (80%) eram do sexo masculino. O IPAQ mostrou que seis (24%) indivíduos eram muito ativos, oito (32%) eram ativos, nove (36%) tinham atividade física irregular e dois (8%) eram sedentários. A média de distância percorrida no teste de caminhada dos 6 minutos, considerando todos os indivíduos estudados, foi de 464,7±100,4 m, sendo 181,4±42,0 m menor do que o previsto (p=0,005). Encontrou-se correlação entre o escore Z do teste de caminhada de 6 minutos e o número de procedimentos cirúrgicos realizados (r=-0,455; p=0,022). Conclusões: Crianças e adolescentes com cardiopatia congênita têm baixa capacidade funcional, mas não são completamente sedentários.

Atuação do psicólogo no acompanhamento de pais de neonatos com malformação fetal / The psychologist's work assisting parents of newborns with fetal malformation

A gravidez é um momento importante na vida da mulher. Notam-se os desejos e as expectativas da família. A mãe imagina o bebê, relaciona-se com o feto, projeta como ele será, enfim, sonha um lugar no mundo para aquele que paradoxalmente não nasceu embora já esteja vivo e presente. O período de gestação é acompanhado por ansiedades específicas, assim sendo, o impacto da notícia de uma cardiopatia congênita no bebê pode tornar-se avassaladora. O bebê real tem um problema cardíaco o que desperta desamparo e regressão materna. Notam-se profundos desejos e expectativas ameaçando a dinâmica familiar. Objetivos: Refletir sobre a atuação do psicólogo junto à família de neonatos com malformação fetal. Métodos: Revisão narrativa da literatura na base de dados da Biblioteca Virtual, no período de 2009 a 2014. Conclusão: A atuação do psicólogo consiste em acompanhar a mãe, o bebê e a família desde o impacto da notícia da malformação. Trabalhar as emoções e fantasias, o luto simbólico do bebê imaginário e a possibilidade de morte. Reconstruir a história do bebê, o lugar que ele ocupará na família e detectar possíveis quadros psicopatológicos. A constituição de uma equipe multidisciplinar é fundamental para oferecer espaço de troca e rede de sustentação

Pregnancy is an important time in a woman's life. It is a time of desires and the family expectations; the mother imagines the baby, connects with the fetus, predicts what the baby will be like, and dreams about a place in the world for the one who, paradoxically, has not yet been born yet is already alive and present. The gestation period is accompanied by specific anxieties, therefore, the impact of the news of a congenital heart disease in the baby can be devastating. The real baby has a heart problem that provokes abandonment and maternal regression. Deep desires and expectations are observed that threaten the family's dynamics. Objective: To reflect on the psychologist's work with the family of the newborn with fetal malformation. Methods: A narrative review of the literature in the Biblioteca Virtual database from 2009 to 2014. Conclusion: The psychologist's work consists in assisting the mother, the baby and the family, from the time they receive the news of the malformation; working with emotions and fantasies, the symbolic mourning of the baby of their imaginations, and the possibility of death; reconstructing the history of the baby and the place he/she will occupy in the family; and detecting possible psychopathological conditions. The formation of a multidisciplinary team, which provides space for exchange of experiences and a support network, is crucial

Congenital Heart Disease and Impacts on Child Development

Abstract Objective: To evaluate the child development and evaluate a possible association with the commitment by biopsychosocial factors of children with and without congenital heart disease. Methods: Observational study of case-control with three groups: Group 1 - children with congenital heart disease without surgical correction; Group 2 - children with congenital heart disease who underwent surgery; and Group 3 - healthy children. Children were assessed by socio-demographic and clinical questionnaire and the Denver II Screening Test. Results: One hundred and twenty eight children were evaluated, 29 in Group 1, 43 in Group 2 and 56 in Group 3. Of the total, 51.56% are girls and ages ranged from two months to six years (median 24.5 months). Regarding the Denver II, the children with heart disease had more "suspicious" and "suspect/abnormal" ratings and in the group of healthy children 53.6% were considered with "normal" development (P≤0.0001). The biopsychosocial variables that were related to a possible developmental delay were gender (P=0.042), child's age (P=0.001) and income per capita (P=0.019). Conclusion: The results suggest that children with congenital heart disease are likely to have a developmental delay with significant difference between children who have undergone surgery and those awaiting surgery under clinical follow-up.

Qualidade de Vida e Cardiopatia Congenita na Infancia e Adolescencia / Quality of Life and Congenital Heart Disease in Childhood and Adolescenc

Os avanços nas técnicas de cirurgia cardíaca e o diagnóstico precoce têm possibilitado maior sobrevida de indivíduos com cardiopatias congênitas. A investigação da qualidade de vida em crianças e adolescentes com cardiopatias congênitas fornece informações complementares aos dados clínicos que podem auxiliar na tomada de decisão dos profissionais de saúde. Embora muitos estudos tenham sido realizados para investigar a qualidade de vida de crianças e adolescentes com cardiopatias congênitas, os resultados mostram-se contraditórios. Enquanto alguns estudos revelam que as cardiopatias podem impactar a qualidade de vida, outros descrevem melhor percepção da qualidade de vida entre crianças e adolescentes cardiopatas quando comparados com controles saudáveis. O objetivo deste estudo é revisar a literatura sobre a avaliação da qualidade de vida relacionada à saúde em crianças e adolescentes com cardiopatias congênitas, de forma a sistematizar o conhecimento existente sobre esse tema na atualidade. Observa-se que as pesquisas procuram investigar aspectos relacionados à personalidade do paciente cardiopata, às estratégias de enfrentamento utilizadas por ele e ao suporte social percebido, visando à melhor compreensão da associação de tais variáveis com o nível de qualidade de vida nessa população.

Advances in cardiac surgery techniques and early diagnosis have enabled the increased survival of individuals with congenital heart disease. The investigation of the quality of life in children and adolescents with congenital heart disease provides complementary information to clinical data that can assist in decision making on the part of health professionals. Although many studies have been conducted to investigate the quality of life of children and adolescents with congenital heart disease, the results prove to be contradictory; while some studies show that congenital heart disease can impact the quality of life, others describe a better perception of quality of life among children and adolescents who suffer from the disease when compared with healthy control subjects. The purpose of this study is to review the literature on the assessment of health related quality of life in children and adolescents with congenital heart disease, in order to systematize the existing knowledge on this topic today. It is observed that research seeks to investigate aspects of personality in cardiac patients, their coping strategies used and perceived social support, aiming at better understanding the association of these variables with the level of quality of life in this population.

Validación del cuestionario de calidad de vida ConQol, en niños chilenos portadores de cardiopatías congénitas / Validation of quality of life questionnaire ConQol for Chilean children with congenital heart diseases

Background: ConQoL questionnaire assesses health related quality of life among children with congenital heart diseases. It has a version for children aged 8 to 11 years and anotherfor children aged 12 to 16years. Aim: To validate ConQol questionnaire for Chilean children with a congenital heart disease. Material and Methods: Using a multicentric cross sectional design, 334 children from four hospitals (54% males), were surveyed. Among them 45% were aged 8 to 11 years and 55%, 12 to 16 years. The study involved three stages: cross cultural adjustment of the original questionnaire, pre-test study, and estimation of its psychometric properties. Content, construct and criterion validity and internal consistency with Cronbach's alpha, were assessed. Results: The version for children aged 8 to 11 years and comprised by three domains (symptoms, activity and relationships), obtained and α ≥ 0.60. In the questionnaire for children aged 12 to 16years, there is one more domain called coping, which obtained an α of 0.53, that was different to the other three domains that obtained an α > 0.70. The correlation between Health Quality of Life and Perception of Health Quality of Life was statistically significant for both groups. The association between Health Quality ofLife and health capability was only significant among children aged 12 to 16years (p < 0.01). Conclusions: The adapted ConQol questionnaire matched properly with the original one. The adapted questionnaire is valid and reliable to assess Health Quality ofLife among Chilean children with congenital heart diseases.

A cirurgia cardíaca pediátrica sob o olhar dos pais: um estudo qualitativo / Pediatric cardiac surgery under the parents sight: a qualitative study

INTRODUÇÃO: As cardiopatias congênitas podem muitas vezes ser corrigidas por meio de cirurgia, assegurando para os pais a expectativa de uma vida normal, entretanto, a vivência da hospitalização, muitas vezes precoce, ocasiona maior sofrimento, sendo a operação o pior momento. OBJETIVO: O objetivo deste estudo foi analisar a vivência de famílias de crianças submetidas à cirurgia cardíaca, identificando os recursos de enfrentamento utilizados pelos familiares. MÉTODOS: A abordagem qualitativa foi a opção metodológica deste estudo, onde realizou-se seis entrevistas semi-estruturadas e 100 horas de observação participante. Foi utilizada a análise temática para a compreensão dos dados. RESULTADOS: Os resultados foram categorizados em quatro núcleos temáticos: sentimentos e emoções frente ao adoecimento do filho; a doença do coração sob o olhar materno; mãe e filho na dinâmica da unidade de terapia intensiva e recursos de enfrentamento. A fala das mães demonstrou a importância do coração devido a seu simbolismo que, por sua vez, potencializa sua fragilidade emocional diante do adoecimento. A religiosidade e uma consistente rede social de apoio foram fatores contribuintes para a manutenção de comportamentos adaptativos. A presença da mãe em todas as etapas do tratamento da criança contribuiu para a minimização do sofrimento gerado pela internação. CONCLUSÕES: A vivência das famílias foi caracterizada por sentimentos ambivalentes, como medo da morte, culpa e impotência frente às diferentes etapas do tratamento. A angústia e a ansiedade prevaleceram diante de situações desconhecidas, necessidade de informações frente às condutas terapêuticas, rotinas hospitalares e da própria situação de vida das famílias entrevistadas.

INTRODUCTION: Congenital heart defects can often be corrected through surgery, providing for parents to expect a normal life, but the hospitalization experience often early, causes more pain, for which surgery is the worst moment. OBJECTIVE: The aim of this study was to analyze the experience of families of children undergoing cardiac surgery and to identify the coping resources used by the families. METHODS: A qualitative approach was the metodology of choice for this study, which took place with six semi-structured interviews and 100 hours of observation. Thematic analysis was used to understand the data. RESULTS: The results were categorized into four themes: feelings and emotions facing the illness of the child; heart disease under the watchful mother, mother and child on the ICU and coping resources. The speech of mothers demonstrated the importance of the heart due to its symbolism that enhances their emotional fragility in the face of illness. Religiosity and a solid social network of support were contributing factors for the maintenance of the adaptive behaviors. The presence of mothers in all stages of the child's treatment contributed to minimizing the suffering generated by hospitalization. CONCLUSION: The experience of families was characterized by ambivalent feelings such as fear of death, guilt and helplessness against the different stages of treatment. The anguish and anxiety prevailed in the face of unknown situations when information were required before therapeutic procedures, hospital routines and the actual life situation of the families.