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.

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.

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.

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.

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.

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.

[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.

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.

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.

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.

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.

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.

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.

A multinational observational investigation of illness perceptions and quality of life among patients with a Fontan circulation.

OBJECTIVE: First, to compare QOL and illness perceptions between patients with a Fontan circulation and patients with anatomically simple defects (ie, atrial septal defects [ASD] or ventricular septal defects [VSD]). Second, to explore illness perceptions as a mediator of the association between congenital heart disease (CHD) diagnosis and QOL. DESIGN: Cross-sectional observational study. SETTING: Twenty-four cardiology centers from 15 countries across five continents. PATIENTS: Four hundred thirty-five adult patients with congenital heart disease (177 Fontan and 258 ASD/VSD) ages 18-83 years. OUTCOME MEASURES: QOL and illness perceptions were assessed by the Satisfaction With Life Scale and the Brief Illness Perceptions Questionnaire, respectively. RESULTS: Patients with a Fontan circulation reported lower QOL (Wald Z = -3.59, p = <.001) and more negative perceptions of their CHD (Wald Z = -7.66, p < .001) compared with patients with ASD/VSD. After controlling for demographics, anxiety, depressive symptoms, and New York Heart Association functional class, path analyses revealed a significant mediation model, αß = 0.15, p = .002, 95% CI = 0.06-0.25, such that CHD diagnosis was indirectly related to QOL through illness perceptions. CONCLUSIONS: The Fontan sample's more negative perceptions of CHD were likely a reflection of life with a more complex defect. Illness perceptions appear to account for unique differences in QOL between groups of varying CHD complexity. Psychosocial screening and interventions may be important treatment components for patients with CHD, particularly those with Fontan circulations.

Meta-Prediction of MTHFR Gene Polymorphisms and Air Pollution on the Risk of Hypertensive Disorders in Pregnancy Worldwide.

Hypertensive disorders in pregnancy (HDP) are devastating health hazards for both women and children. Both methylenetetrahydrofolate reductase (MTHFR) gene polymorphisms and air pollution can affect health status and result in increased risk of HDP for women. The major objective of this study was to investigate the effect of MTHFR polymorphisms, air pollution, and their interaction on the risk of HDP by using meta-predictive analytics. We searched various databases comprehensively to access all available studies conducted for various ethnic populations from countries worldwide, from 1997 to 2017. Seventy-one studies with 8064 cases and 13,232 controls for MTHFR C677T and 11 studies with 1425 cases and 1859 controls for MTHFR A1298C were included. MTHFR C677T homozygous TT (risk ratio (RR) = 1.28, p < 0.0001) and CT plus TT (RR = 1.07, p = 0.0002) were the risk genotypes, while wild-type CC played a protective role (RR = 0.94, p = 0.0017) for HDP. The meta-predictive analysis found that the percentage of MTHFR C677T TT plus CT (p = 0.044) and CT (p = 0.043) genotypes in the HDP case group were significantly increased with elevated levels of air pollution worldwide. Additionally, in countries with higher air pollution levels, the pregnant women with wild-type CC MTHFR 677 had a protection effect against HDP (p = 0.014), whereas, the homozygous TT of MTHFR C677T polymorphism was a risk genotype for developing HDP. Air pollution level is an environmental factor interacting with increased MTHFR C677T polymorphisms, impacting the susceptibility of HDP for women.

A positive perspective of knowledge, attitude, and practices for health-promoting behaviors of adolescents with congenital heart disease.

BACKGROUND: Health-promoting behaviors could serve as a major strategy to optimize long-term outcomes for adolescents with congenital heart disease. The associations assessed from a positive perspective of knowledge, attitudes, and practice model would potentially cultivate health-promoting behaviors during adolescence. AIM: The purpose of this study was to examine the relationships between disease knowledge, resilience, family functioning, and health-promoting behaviors in adolescents with congenital heart disease. METHODS: A total of 320 adolescents with congenital heart disease who were aged 12-18 years were recruited from pediatric cardiology outpatient departments, and participated in a cross-sectional survey. The participants completed the Leuven Knowledge Questionnaire for Congenital Heart Disease; Haase Adolescent Resilience in Illness Scale; Family Adaptability, Partnership, Growth, Affection, and Resolve; and Adolescent Health Promotion scales. The collected data were analyzed using descriptive statistics and three multiple regression models. RESULTS: Greater knowledge of prevention of complications and higher resilience had a more powerful effect in enhancing health-promoting behaviors. Having symptoms and moderate or severe family dysfunction were significantly more negatively predictive of health-promoting behaviors than not having symptoms and positive family function. The third model explained 40% of the variance in engaging in health-promoting behaviors among adolescents with congenital heart disease. CONCLUSION: The findings of this study provide new insights into the role of disease knowledge, resilience, and family functioning in the health-promoting behavior of adolescents with congenital heart disease. Continued efforts are required to plan family care programs that promote the acquisition of sufficient disease knowledge and the development of resilience for adolescents with congenital heart disease.