Psychometric evaluation of the traditional chinese version of PedsQL™ 3.0 cardiac module scale in adolescents with congenital heart disease: reliability, validity, measurement invariance, and adolescent-parent agreement.

BACKGROUND: In recent decades, 95% of children with congenital heart disease (CHD) can survive to adolescence and adulthood. However, adolescents with CHD are prone to poorer health-related quality of life (HRQoL). It is imperative to develop a reliable and valid instrument for health professionals to monitor the HRQoL. This study aims to: (1) evaluate the psychometric properties of the traditional Chinese version of Pediatric Quality of Life™ 3.0 Cardiac Module (PedsQL-CM) and measurement invariance across adolescents with CHD and their parents; and (2) investigate the adolescent-parent agreement in HRQoL. METHODS: A total of 162 adolescents and 162 parents were recruited. Internal consistency was examined using Cronbach's alpha and McDonald's Omega. The criterion-related validity was evaluated with intercorrelations between the PedsQL-CM and PedsQL™ 4.0 Generic Core (PedsQL-GC) Scale. The construct validity was examined by second-order confirmatory factor analysis (CFA). Measurement invariance was evaluated using the multi-group CFA. The adolescent-parent agreement was analyzed with the intraclass correlation (ICC), paired t-tests, and Bland-Altman plots. RESULTS: PedsQL-CM showed acceptable internal consistency (self-reports 0.88, proxy-reports 0.91). The intercorrelations were medium to large effect size (self-reports 0.34-0.77, proxy-reports 0.46-0.68). The CFA supported the construct validity (CFI = 0.967, TLI = 0.963, RMSEA = 0.036, 90% CI = 0.026-0.046, SRMR = 0.065). The multi-group CFA proved scalar invariance between self and parent proxy-reports. Parents significantly underestimated their adolescents' HRQoL in cognitive problems (Cohen's d = 0.21) and communication (Cohen's d = 0.23) subscales, while there was a negligible difference in total HRQoL (Cohen's d = 0.16). ICCs were poor to moderate effect size with the highest and lowest agreement in heart problems and treatment subscale (ICC = 0.70) and communication subscale (ICC = 0.27), respectively. The Bland-Altman plots showed lesser variability in the heart problem and treatment subscale and the total scale. CONCLUSION: The traditional Chinese version of PedsQL-CM has acceptable psychometric properties to measure disease-specific HRQoL in adolescents with CHD. Parents may be proxies for adolescents with CHD to rate total HRQoL. When the patient-reported score is the primary outcome, the proxy-reported score could serve as a secondary outcome for research and clinical evaluation.

Transition to adulthood and transfer to adult care of adolescents with congenital heart disease: a global consensus statement of the ESC Association of Cardiovascular Nursing and Allied Professions (ACNAP), the ESC Working Group on Adult Congenital Heart Disease (WG ACHD), the Association for European Paediatric and Congenital Cardiology (AEPC), the Pan-African Society of Cardiology (PASCAR), the Asia-Pacific Pediatric Cardiac Society (APPCS), the Inter-American Society of Cardiology (IASC), the Cardiac Society of Australia and New Zealand (CSANZ), the International Society for Adult Congenital Heart Disease (ISACHD), the World Heart Federation (WHF), the European Congenital Heart Disease Organisation (ECHDO), and the Global Alliance for Rheumatic and Congenital Hearts (Global ARCH).

The vast majority of children with congenital heart disease (CHD) in high-income countries survive into adulthood. Further, paediatric cardiac services have expanded in middle-income countries. Both evolutions have resulted in an increasing number of CHD survivors. Expert care across the life span is necessitated. In adolescence, patients transition from being a dependent child to an independent adult. They are also advised to transfer from paediatrics to adult care. There is no universal consensus regarding how transitional care should be provided and how the transfer should be organized. This is even more challenging in countries with low resources. This consensus document describes issues and practices of transition and transfer of adolescents with CHD, accounting for different possibilities in high-, middle-, and low-income countries. Transitional care ought to be provided to all adolescents with CHD, taking into consideration the available resources. When reaching adulthood, patients ought to be transferred to adult care facilities/providers capable of managing their needs, and systems have to be in place to make sure that continuity of high-quality care is ensured after leaving paediatric cardiology.

[Associations Between Daytime Physical Activity and Sleep Patterns in Children With Epilepsy].

BACKGROUND: Epilepsy is one of the most common chronic neurological diseases in children. Sleep disorders tend to increase the risk of seizures, and research has found that moderate physical activity may improve the quantity and quality of sleep in adults. However, the link between physical activity level and sleep patterns in toddlers and preschool-age children with epilepsy remains unclear. PURPOSE: To explore the association between level of physical activity and sleep patterns in toddlers and preschool-age children with epilepsy. METHODS: A cross-sectional, descriptive, correlational study was conducted. Ninety-eight children with epilepsy (1.5-6 years old) wore an actigraph for seven days. Additional data were collected using a health information datasheet, Children's Sleep Habits Questionnaire (CSHQ), and sleep diary, all of which were completed by the parents of each child. RESULTS: The results showed that the mean amount of time spent in moderate-to-vigorous physical activity per day was 36.00 ± 49.20 minutes and that only 23 children (23.5%) had a nighttime sleep efficiency greater than 85%. The overall CSHQ score (56.00 ± 5.69) indicated the presence of moderate to severe sleep disturbances. Multiple regression analysis showed the hours and percentage of moderate-to-vigorous physical activity to be positively associated with night sleep efficiency (ß = .54, p < .01; ß = .51, p < .01) and negatively associated with nighttime sleep hours (ß = -.55, p < .01; ß = -.52, p < .01), even after controlling for potential confounders. CONCLUSIONS / IMPLICATIONS FOR PRACTICE: Based on the findings, the sleep patterns and physical activity of children with epilepsy should be regularly assessed. Furthermore, appropriately increasing the duration of moderate-to-vigorous physical activity may improve sleep efficiency and prevent reductions in the duration of night sleep.

Screen Time Exposure and Altered Sleep in Young Children With Epilepsy.

PURPOSE: To examine the association between daily screen time exposure and both sleep patterns (sleep onset, sleep offset, and nighttime, and daily sleep durations) and sleep disturbances among a clinical sample of children with epilepsy. DESIGN: A cross-sectional actigraphic sleep study. METHODS: A convenience sample of 141 children with epilepsy (1.5-6 years of age) was recruited from an outpatient pediatric neurology clinic of a university-affiliated children's hospital in northern Taiwan. Participating families completed questionnaires and reported children's screen time use, with children wearing an actigraphy monitor for 7 days to assess sleep patterns. Multivariable linear regression analyses were conducted to examine the association of screen time exposure with the child's sleep patterns and sleep disturbance scores. FINDINGS: Mean minutes per day of screen time exposure was 89.79 ± 83.94 min, with 62 parents (44.0%) reporting their child having >1 hr of screen time daily. Mean daily sleep duration was 9.26 ± 1.01 hr, with 106 children (93.0%) sleeping <10 hr in a 24-hr period. In multivariate regression models, daily screen time exposure of >1 hour was associated with 23.4-min later sleep onset (b = 0.39, p = .02), 20.4-min later sleep offset (b = 0.34, p = .04), and more severe sleep disturbances (b = 2.42, p = .04). CONCLUSIONS: In toddlers and preschool-age children with epilepsy, daily screen time exposure is greater and sleep duration is shorter than the recommended amount, with increased screen time exposure associated with disturbed sleep. CLINICAL RELEVANCE: Parents need to be informed about the possible adverse impact of screen time exposure on children's sleep and health as well as the importance of limiting screen time exposure to <1 hr per day for their toddlers and preschool-age children with epilepsy.

Energy Balance-Related Behaviors and Body Mass Index in Asian School-Aged Children With Congenital Heart Disease.

BACKGROUND: Overweight/obesity is a substantial global public health concern, which can be caused by genetic factors and energy balance-related behaviors (EBRBs). If it occurs in children with congenital heart disease (CCHD), it can yield an extra burden on their health. Most studies on CCHD have taken place in Western societies, leaving Asian populations understudied, especially children. OBJECTIVE: We sought (1) to determine body mass index distribution among school-aged CCHD in Taiwan, (2) to ascertain whether the body mass index of CCHD differs from that of the general population, (3) to describe EBRBs in CCHD, and (4) to identify factors associated with underweight and overweight/obesity among CCHD. METHODS: In this cross-sectional study, 97 child-parent dyads (53.6% boys; mean age, 9.73 years; 25.8% moderate-to-severe heart conditions) were enrolled. Self-administered questionnaires were used to collect demographics, medical factors, food frequency, physical activity, and sedentary behaviors. Anthropometric measurements were taken in the hospital. Independent predictors of EBRBs and health conditions were calculated through logistic regression analysis. RESULTS: Among Taiwanese CCHD, 19.6% were underweight and 14.4% were overweight/obese. Children with moderate-to-severe heart defects were more often underweight. Body mass index did not differ between CCHD and children in the general population. More complex heart defects and asthma were associated with being underweight, whereas sedentary behaviors, cardiomegaly, and the New York Heart Association classification II to IV were associated with being overweight/obese. CONCLUSIONS: Sedentary lifestyle is seemingly the only EBRB correlated with being overweight. Physical activity programs for children may help prevent and treat overweight or obesity in Asian CCHD, similar to Western countries.

[Using the FOCUS Family Intervention for Family-Centered Care in a Premature Infant With Grade IV Intraventricular Hemorrhage].

Preterm infants face increased rates of mortality and developmental complications, which are a burden on children's parents (and caregivers), who suffer from exhaustion and situational uncertainty. This case focused on an extremely-low-birth-weight (908 gm) premature infant with initial unstable vital signs complicated by a grade 4 intraventricular hemorrhage (IVH) that led to partial brain atrophy and enlarged brain ventricles. A poor neurological outcome was expected due to the high risk of cerebral palsy and impaired cognitive abilities. Long-term healthcare for this critical infant was causing tremendous physical, emotional, and financial strains on the family. The parents suffered from worries over the poor prognosis, resulting in stress, sleep disorders, and relationship difficulties with the healthcare professionals. Considering the poor prognosis of the infant, the parents faced a medical dilemma between choosing aggressive treatment and withdrawal of treatment, which led to stress and sleep disorders. Differences between the parents and health professionals regarding disease severity perception and treatment opinions further strained their mutual relationship. To ameliorate this issue, the author implemented family-centered care (the FOCUS family intervention) to help the patient and his family. This intervention is designed to increase family involvement, foster an optimistic attitude and effective stress coping techniques, and reduce uncertainty and negative emotions. For the patient, we provided symptom-relief management to improve abnormal muscle tone and development delay. Our intervention ameliorated the negative emotions, insomnia symptoms, and imbalanced family relationships and improved the life quality of the caregivers. Furthermore, the intervention enhanced the patient's autoregulatory ability, and both physical and neurological development. This case study is expected to provide experience in critical care for premature infants with a poor prognosis and their family using a FOCUS family intervention as well as to improve the quality of healthcare delivery in intensive clinical settings.

Frailty and Cognitive Function in Middle-Aged and Older Adults With Congenital Heart Disease.

BACKGROUND: Life expectancy of patients with congenital heart disease (CHD) has increased rapidly, resulting in a growing and aging population. Recent studies have shown that older people with CHD have higher morbidity, health care use, and mortality. To maintain longevity and quality of life, understanding their evolving medical and psychosocial challenges is essential. OBJECTIVES: The authors describe the frailty and cognitive profile of middle-aged and older adults with CHD to identify predictor variables and to explore the relationship with hospital admissions and outpatient visits. METHODS: Using a cross-sectional, multicentric design, we included 814 patients aged ≥40 years from 11 countries. Frailty phenotype was determined using the Fried method. Cognitive function was assessed by the Montreal Cognitive Assessment. RESULTS: In this sample, 52.3% of patients were assessed as robust, 41.9% as prefrail, and 5.8% as frail; 38.8% had cognitive dysfunction. Multinomial regression showed that frailty was associated with older age, female sex, higher physiologic class, and comorbidities. Counterintuitively, patients with mild heart defects were more likely than those with complex lesions to be prefrail. Patients from middle-income countries displayed more prefrailty than those from higher-income countries. Logistic regression demonstrated that cognitive dysfunction was related to older age, comorbidities, and lower country-level income. CONCLUSIONS: Approximately one-half of included patients were (pre-)frail, and more than one-third experienced cognitive impairment. Frailty and cognitive dysfunction were identified in patients with mild CHD, indicating that these concerns extend beyond severe CHD. Assessing frailty and cognition routinely could offer valuable insights into this aging population.

An evaluation of disease knowledge in dyads of parents and their adolescent children with congenital heart disease.

BACKGROUND: Congenital heart disease (CGHD) can be considered a chronic disease for many patients. To adopt a healthy lifestyle and to avoid complications, patients with CGHD and their parents need to have good knowledge of the heart defect and its consequences. OBJECTIVE: The aims of this study were to evaluate patient and parental knowledge of CGHD and to explore the related factors of their respective disease knowledge. METHODS: This study included 116 dyads of adolescents with CGHD (43.1% male adolescents; aged 12-18 years) and one of their parents (79.3% mothers; median age, 46 years). All participants completed the Leuven Knowledge Questionnaire for Congenital Heart Disease, and then we calculated a correct rate score to determine the overall disease knowledge of the respondents. RESULTS: The correct rate score was 38.8% for adolescents with CGHD and 51.4% for parents (t = 7.69; P < .001). The only determinant of knowledge in parents was their educational level (standardized estimate = 6.160, P < .001). In adolescents, knowledge was determined by age (standardized estimate = 2.242, P = .002) and parental knowledge (standardized estimate = 0.311, P < .001). CONCLUSIONS: Although parents have significantly greater disease knowledge than their children do, the level of knowledge in both parents and adolescents is suboptimal. Because parents' knowledge influenced their adolescents' knowledge, educational interventions should target both adolescent patients and parents. Transition programs can play a pivotal role in this respect.

Patient-reported outcomes in the aging population of adults with congenital heart disease: results from APPROACH-IS.

The congenital heart disease (CHD) population now comprises an increasing number of older persons in their 6th decade of life and beyond. We cross-sectionally evaluated patient-reported outcomes (PROs) in persons with CHD aged 60 years or older, and contrasted these with PROs of younger patients aged 40-59 years and 18-39 years. Adjusted for demographic and medical characteristics, patients ≥60 years had a lower Physical Component Summary, higher Mental Component Summary, and lower anxiety (Hospital Anxiety and Depression Scale-Anxiety) scores than patients in the two younger categories. For satisfaction with life, older persons had a higher score than patients aged 40-59 years. Registration: ClinicalTrials.gov NCT02150603.

The use of complementary and alternative medicine for patients with traumatic brain injury in Taiwan.

BACKGROUND: The use of complementary and alternative medicine (CAM) continues to increase in Taiwan. This study examined the use of CAM and beliefs about CAM as expressed by patients with traumatic brain injury (TBI) in Taiwan. METHODS: TBI patients and their accompanying relatives were interviewed by using a structured questionnaire at an outpatient clinic in a medical center in northern Taiwan. RESULTS: A total of 101 patients with TBI participated in the study. Sixty-four (63%) patients had used at least one form of CAM after sustaining TBI. CAM users had used an average of 2.72 forms of CAM after sustaining TBI. The most frequently used CAM category was traditional Chinese medicine (37; 57.8%), followed by folk and religious therapies (30; 46.9%), and dietary supplements (30; 46.9%). The majority of the patients (45; 70.3%) did not report CAM use because they felt it was unnecessary to do so. Patients who used CAM had a significantly stronger positive belief in CAM than those who did not (t = -2.72; P = .008). After using CAM, most of the patients (54; 85%) perceived moderate satisfaction (2.89 ± 0.44), according to a 4-point Likert scale. CONCLUSION: Although the use of CAM is common for TBI patients receiving conventional medical health care in Taiwan, most patients did not inform health care personnel about their CAM use. TBI patients perceive combined use of CAM and conventional medicine as beneficial for their overall health.

Validating Accuracy of an Internet-Based Application against USDA Computerized Nutrition Data System for Research on Essential Nutrients among Social-Ethnic Diets for the E-Health Era.

Internet-based applications (apps) are rapidly developing in the e-Health era to assess the dietary intake of essential macro-and micro-nutrients for precision nutrition. We, therefore, validated the accuracy of an internet-based app against the Nutrition Data System for Research (NDSR), assessing these essential nutrients among various social-ethnic diet types. The agreement between the two measures using intraclass correlation coefficients was good (0.85) for total calories, but moderate for caloric ranges outside of <1000 (0.75) and >2000 (0.57); and good (>0.75) for most macro- (average: 0.85) and micro-nutrients (average: 0.83) except cobalamin (0.73) and calcium (0.51). The app underestimated nutrients that are associated with protein and fat (protein: −5.82%, fat: −12.78%, vitamin B12: −13.59%, methionine: −8.76%, zinc: −12.49%), while overestimated nutrients that are associated with carbohydrate (fiber: 6.7%, B9: 9.06%). Using artificial intelligence analytics, we confirmed the factors that could contribute to the differences between the two measures for various essential nutrients, and they included caloric ranges; the differences between the two measures for carbohydrates, protein, and fat; and diet types. For total calories, as an example, the source factors that contributed to the differences between the two measures included caloric range (<1000 versus others), fat, and protein; for cobalamin: protein, American, and Japanese diets; and for folate: caloric range (<1000 versus others), carbohydrate, and Italian diet. In the e-Health era, the internet-based app has the capacity to enhance precision nutrition. By identifying and integrating the effects of potential contributing factors in the algorithm of output readings, the accuracy of new app measures could be improved.

Validating Accuracy of a Mobile Application against Food Frequency Questionnaire on Key Nutrients with Modern Diets for mHealth Era.

In preparation for personalized nutrition, an accurate assessment of dietary intakes on key essential nutrients using smartphones can help promote health and reduce health risks across vulnerable populations. We, therefore, validated the accuracy of a mobile application (app) against Food Frequency Questionnaire (FFQ) using artificial intelligence (AI) machine-learning-based analytics, assessing key macro- and micro-nutrients across various modern diets. We first used Bland and Altman analysis to identify and visualize the differences between the two measures. We then applied AI-based analytics to enhance prediction accuracy, including generalized regression to identify factors that contributed to the differences between the two measures. The mobile app underestimated most macro- and micro-nutrients compared to FFQ (ranges: -5% for total calories, -19% for cobalamin, -33% for vitamin E). The average correlations between the two measures were 0.87 for macro-nutrients and 0.84 for micro-nutrients. Factors that contributed to the differences between the two measures using total calories as an example, included caloric range (1000-2000 versus others), carbohydrate, and protein; for cobalamin, included caloric range, protein, and Chinese diet. Future studies are needed to validate actual intakes and reporting of various diets, and to examine the accuracy of mobile App. Thus, a mobile app can be used to support personalized nutrition in the mHealth era, considering adjustments with sources that could contribute to the inaccurate estimates of nutrients.

Heart Failure and Patient-Reported Outcomes in Adults With Congenital Heart Disease from 15 Countries.

Background Heart failure (HF) is the leading cause of mortality and associated with significant morbidity in adults with congenital heart disease. We sought to assess the association between HF and patient-report outcomes in adults with congenital heart disease. Methods and Results As part of the APPROACH-IS (Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease-International Study), we collected data on HF status and patient-reported outcomes in 3959 patients from 15 countries across 5 continents. Patient-report outcomes were: perceived health status (12-item Short Form Health Survey), quality of life (Linear Analogue Scale and Satisfaction with Life Scale), sense of coherence-13, psychological distress (Hospital Anxiety and Depression Scale), and illness perception (Brief Illness Perception Questionnaire). In this sample, 137 (3.5%) had HF at the time of investigation, 298 (7.5%) had a history of HF, and 3524 (89.0%) had no current or past episode of HF. Patients with current or past HF were older and had a higher prevalence of complex congenital heart disease, arrhythmias, implantable cardioverter-defibrillators, other clinical comorbidities, and mood disorders than those who never had HF. Patients with HF had worse physical functioning, mental functioning, quality of life, satisfaction with life, sense of coherence, depressive symptoms, and illness perception scores. Magnitudes of differences were large for physical functioning and illness perception and moderate for mental functioning, quality of life, and depressive symptoms. Conclusions HF in adults with congenital heart disease is associated with poorer patient-reported outcomes, with large effect sizes for physical functioning and illness perception. Registration URL: https://clinicaltrials.gov; Unique identifier: NCT02150603.

Comorbidity as a mediator of depression in adults with congenital heart disease: A population-based cohort study.

BACKGROUND: The population of adults with congenital heart disease (CHD) has increased dramatically with a high prevalence of acquired cardiac and non-cardiac comorbidities. However, the relationship among congenital heart disease, physical comorbidities, and psychological health in this population is not well studied. AIMS: The purpose of this study was to investigate (a) the association between adult congenital heart disease and the occurrence of depression and (b) whether physical comorbidities mediated the association between congenital heart disease and the occurrence of depression. METHODS: This retrospective cohort study was followed from 1 January 2010-31 December 2013, based on the data from the National Health Insurance Research Database 2010 in Taiwan. We used mediation analysis in survival data to assess the mediated effect. The hazard ratios were adjusted by age, sex, area of residence, and estimated propensity scores. RESULTS: We recruited 2122 adult congenital heart disease patients and 8488 matched controls. Nearly half of patients diagnosed with simple congenital heart disease, 39.0% had complex congenital heart disease, and 11.2% had unclassified congenital heart disease. Adult congenital heart disease patients had a significantly higher risk of depression than matched controls (adjusted hazard ratio = 1.43 and 1.48, for all and complex congenital heart disease, respectively, p<0.05). Coronary artery disease and chronic obstructive pulmonary disease were the significant comorbidities mediating the relationship between adult congenital heart disease and depression, the proportions mediated by coronary artery disease or chronic obstructive pulmonary disease were 35.5% and 12.9%, respectively. CONCLUSIONS: Helping patients to prevent psychological and physical acquired disease is imperative. Coronary artery disease is a potent mediator between congenital heart disease and depression, especially for patients with complex congenital heart disease.

Trend analysis of nursing intervention studies published by Taiwan nurses association between 1954 and 2006.

This study reviewed historical nursing intervention studies in Taiwan and analyzed results based on sample characteristics, methodology characteristics, and content characteristics. Historical studies were split into four groups, G1970, G1980, G1990, and G2000, based on the decade in which they were published. A comparison was then made to identify significant differences between G2000 and other groups. A total of 103 nursing intervention studies were analyzed in this study. Results included sample characteristics of studies conducted primarily by single-disciplinary research teams, although multidisciplinary studies showed a growth trend across decades, with the number of multidisciplinary studies significantly greater in G2000 than in other decades. Nursing practice was the main area of research, clients were the major participants studied, and hospitals provided the principal research setting. No statistical difference was noted in the above mentioned variables. In terms of methodology characteristics, questionnaires were used most frequently to collect data. A quasi-experimental design was the primary research design employed. Although the number of experimental designs has increased significantly in recent years, most studies specified their inclusion criteria. Reliability and validity of instruments were more clearly specified in recent decade groups than in earlier decade groups. Methodology rigor has improved in recent years, achieving a statistically significant difference between G2000 and other groups in all categories, with the exception of instrument validity. In terms of content characteristics, health education was the leading intervention type. Most interventions were categorized as tertiary prevention, with perceptual outcome used most often as the outcome indicator. However, no statistical differences in content dimension variables were identified between G2000 and other groups. In summary, the number of nursing intervention research cases has risen, and research methodology rigor has improved in recent decades. However, no statistically significant differences in sample characteristics and content characteristics between the most recent and earlier decade groups were found. Suggestions for future domestic nursing intervention studies based on study findings are made at the conclusion of this article.

Education as important predictor for successful employment in adults with congenital heart disease worldwide.

BACKGROUND: Conflicting results have been reported regarding employment status and work ability in adults with congenital heart disease (CHD). Since this is an important determinant for quality of life, we assessed this in a large international adult CHD cohort. METHODS: Data from 4028 adults with CHD (53% women) from 15 different countries were collected by a uniform survey in the cross-sectional APPROACH International Study. Predictors for employment and work limitations were studied using general linear mixed models. RESULTS: Median age was 32 years (IQR 25-42) and 94% of patients had at least a high school degree. Overall employment rate was 69%, but varied substantially among countries. Higher education (OR 1.99-3.69) and having a partner (OR 1.72) were associated with more employment; female sex (OR 0.66, worse NYHA functional class (OR 0.67-0.13), and a history of congestive heart failure (OR 0.74) were associated with less employment. Limitations at work were reported in 34% and were associated with female sex (OR 1.36), increasing age (OR 1.03 per year), more severe CHD (OR 1.31-2.10), and a history of congestive heart failure (OR 1.57) or mental disorders (OR 2.26). Only a university degree was associated with fewer limitations at work (OR 0.62). CONCLUSIONS: There are genuine differences in the impact of CHD on employment status in different countries. Although the majority of adult CHD patients are employed, limitations at work are common. Education appears to be the main predictor for successful employment and should therefore be encouraged in patients with CHD.

Religion and spirituality as predictors of patient-reported outcomes in adults with congenital heart disease around the globe.

AIMS: Religion and spirituality can be resources for internal strength and resilience, and may assist with managing life's challenges. Prior studies have been undertaken primarily in countries with high proportions of religious/spiritual people. We investigated (i) whether being religious/spiritual is an independent predictor of patient-reported outcomes (PROs) in a large international sample of adults with congenital heart disease, (ii) whether the individual level of importance of religion/spirituality is an independent predictor for PROs, and (iii) if these relationships are moderated by the degree to which the respective countries are religious or secular. METHODS AND RESULTS: APPROACH-IS was a cross-sectional study, in which 4028 patients from 15 countries were enrolled. Patients completed questionnaires to measure perceived health status; psychological functioning; health behaviors; and quality of life. Religion/spirituality was measured using three questions: Do you consider yourself religious or spiritual?; How important is religion, spirituality, or faith in your life?; and If religious, to what religion do you belong?. The country level of religiosity/secularity was appraised using data from the Gallup Poll 2005-2009. General linear mixed models, adjusting for patient characteristics and country differences were applied. Overall, 49.2% of patients considered themselves to be religious/spiritual. Being religious/spiritual and considering religion/spirituality as important in one's life was positively associated with quality of life, satisfaction with life and health behaviors. However, among patients living in more secular countries, religion/spirituality was negatively associated with physical and mental health. CONCLUSION: Religiosity/spirituality is an independent predictor for some PROs, but has differential impact across countries.

Prevalence and Effects of Cigarette Smoking, Cannabis Consumption, and Co-use in Adults From 15 Countries With Congenital Heart Disease.

BACKGROUND: The prevalence and effects of cigarette smoking and cannabis use in persons with congenital heart disease (CHD) are poorly understood. We (1) described the prevalence of cigarette smoking, cannabis consumption, and co-use in adults with CHD; (2) investigated intercountry differences; (3) tested the relative effects on physical functioning, mental health, and quality of life (QOL); and (4) quantified the differential effect of cigarette smoking, cannabis use, or co-use on those outcomes. METHODS: APPROACH-IS was a cross-sectional study, including 4028 adults with CHD from 15 countries. Patients completed questionnaires to measure physical functioning, mental health, and QOL. Smoking status and cannabis use were assessed by means of the Health Behaviour Scale-Congenital Heart Disease. Linear models with doubly robust estimations were computed after groups were balanced with the use of propensity weighting. RESULTS: Overall, 14% of men and 11% of women smoked cigarettes only; 8% of men and 4% of women consumed cannabis only; and 4% of men and 1% of women used both substances. Large intercountry variations were observed, with Switzerland having the highest prevalence for smoking cigarettes (24% of men, 19% of women) and Canada the highest for cannabis use (19% of men, 4% of women). Cigarette smoking had a small negative effect on patient-reported outcomes, and the effect of cannabis was negligible. The effect of co-use was more prominent, with a moderate negative effect on mental health. CONCLUSIONS: We found significant intercountry variability in cigarette and cannabis use in adults with CHD. Co-use has the most detrimental effects on patient-reported outcomes.

Meta-Prediction of MTHFR Gene Polymorphism and Air Pollution on the Risks of Congenital Heart Defects Worldwide: A Transgenerational Analysis.

Congenital heart disease (CHD) is the leading cause of death in children, and is affected by genetic and environmental factors. To investigate the association of air pollution with methylene-tetrahydrofolate reductase (MTHFR) polymorphisms and the risk of CHD, we included 58 study groups of children and parents, with 12,347 cases and 18,106 controls worldwide. Both MTHFR C677T (rs 1801133) and A1298C (rs 1801131) gene polymorphisms were risks for CHD in children with transgenerational effects from their parents. Countries with greater risks of CHD with a pooled risk ratio (RR) > 2 from MTHFR 677 polymorphisms included Germany, Portugal, China, and Egypt for children; and Brazil, Puerto Rico, Mexico, China, and Egypt for mothers. Whereas, countries with greater risk of CHD with RR > 2 from MTHFR 1298 polymorphisms included Taiwan, Turkey, and Egypt for children; and Brazil, China, and Egypt for mothers. Additionally, meta-prediction analysis revealed that the percentages of MTHFR 677TT and TT plus CT polymorphisms together were increased in countries with higher levels of air pollution, with a trend of increased CHD risks with higher levels of air pollution for children (p = 0.07). Our findings may have significant implications for inflammatory pathways in association with MTHFR polymorphisms and future intervention studies to correct for folate-related enzyme deficits resultied from MTHFR polymorphisms to prevent CHDs for future generations.