Health anxiety and associated constructs in school-age children and adolescents with congenital heart disease and their parents: A children's healthy-heart activity monitoring program in Saskatchewan cohort study.

Youth with congenital heart disease (CHD) have been found to experience higher levels of health anxiety and associated constructs than typically developing peers. The association between youth and parent health anxiety has been explored in typically developing youth but this association remains unknown in youth with CHD. This association was explored using a prospective, cross-sectional study that included 36 school-age children and adolescents with CHD (median age =10.5 years, IQR = 4) and 35 parents (median age = 44 years, IQR = 10.5). Participants completed a demographic form and measures of health anxiety, anxiety sensitivity, intolerance of uncertainty, and anxiety disorder symptom categories (youth) or general anxiety (parent). Associations were observed between child and adolescent panic/agoraphobia symptoms and parent state anxiety (r = .41), child and adolescent intolerance of uncertainty and parent state and trait anxiety (r = .37; r = .46, respectively), and child and adolescent anxiety sensitivity and parent state anxiety (r = .40). No association was observed between health anxiety in children and adolescents and parents nor between child and adolescent health anxiety and parent associated constructs. For parents, associations between health anxiety and all measures of associated constructs of interest were observed. Study findings will facilitate improved understanding of the psychological needs of school-age children and adolescents with CHD.

Barriers to Care in ACHD: A Study of Young Adults in Saskatchewan.

BACKGROUND: Adults with congenital heart disease (CHD) are living longer with more complex disease. Maintaining lifelong care prevents morbidity and mortality, but many patients remain lost to follow-up or experience care gaps. We sought to assess barriers to care for patients with adult CHD (ACHD) in Saskatchewan, a Canadian province with no local congenital cardiac surgical support and no clear framework for ACHD care. METHODS: We performed a telephone survey of patients with CHD transferred from pediatric to adult cardiology from 2007 to 2014. Our primary outcome was loss to follow-up > 2 years from last recommended cardiology appointment and/or multiple missed cardiology appointments. Secondary outcomes were guideline-based care (specialist training, adherence to appropriate endocarditis prophylaxis, pre-pregnancy counselling for women), presence or absence of previously described barriers to care in ACHD, and health care autonomy using the Krantz Health Opinion Survey. RESULTS: We interviewed 32 patients (30% response rate). One-quarter met the primary outcome: lost to follow-up > 2 years from last recommended cardiology appointment and/or self-report of missed cardiology appointments. Only 69% of young adults in Saskatchewan were receiving guideline-based care for their CHD (appropriate level of specialist expertise and frequency of follow-up). Only 72% of patients were adhering to endocarditis prophylaxis recommendations and 61% of women surveyed received counselling regarding pregnancy. Patients indicated a low preference for participating in decision making regarding their care on the Krantz Health Opinion Survey. CONCLUSIONS: With our survey, we have created a novel snapshot of CHD care in Saskatchewan and have identified significant deficits.

CONTEXTE: Les adultes souffrant d'une cardiopathie congénitale vivent de plus en plus longtemps avec une maladie complexe. Les soins qui leur sont prodigués toute leur vie aident à prévenir la morbidité et la mortalité, mais nombreux sont les patients qui sont perdus de vue en cours de route ou qui vivent des périodes sans recevoir de soins. Nous avons tenté d'évaluer les obstacles aux soins des patients adultes atteints d'une cardiopathie congénitale en Saskatchewan, une province canadienne où il n'existe aucun programme local de soutien en matière de chirurgie cardiaque pour les troubles congénitaux ni de cadre définissant clairement les soins à prodiguer aux adultes atteints d'une cardiopathie congénitale. MÉTHODOLOGIE: Nous avons interviewé par téléphone des patients atteints d'une cardiopathie congénitale qui sont passés d'un suivi en cardiologie en soins pédiatriques aux soins aux adultes entre 2007 et 2014. Le critère d'évaluation principal était la perte de vue pendant plus de 2 ans après le dernier rendez-vous de suivi en cardiologie recommandé et/ou plusieurs rendez-vous en cardiologie manqués. Les critères d'évaluation secondaires étaient les soins recommandés dans les lignes directrices (formation spécialisée, observance d'une prophylaxie endocardite appropriée, counseling préalable à la grossesse chez les femmes), la présence ou l'absence d'obstacles aux soins relatifs à la cardiopathie congénitale chez l'adulte cités antérieurement et l'autonomie en matière de soins de santé selon le sondage d'opinion sur la santé de Krantz. RÉSULTATS: Nous avons interviewé 32 patients (taux de réponse de 30 %). Le quart des patients interrogés répondaient au critère d'évaluation principal, soit la perte de vue pendant plus de 2 ans après le dernier rendez-vous de suivi en cardiologie recommandé et/ou plusieurs rendez-vous en cardiologie manqués autodéclarés. Seulement 69 % des jeunes patients adultes de la Saskatchewan recevaient les soins recommandés dans les lignes directrices en matière de cardiopathie congénitale (degré approprié d'expertise spécialisée du médecin et fréquence des consultations de suivi). Seulement 72 % des patients observaient les recommandations en matière de prophylaxie endocardite, et 61 % des femmes interrogées avaient reçu des services de counseling concernant la grossesse. Selon les résultats au sondage d'opinion sur la santé de Krantz, les patients étaient généralement peu disposés à participer aux décisions concernant leur programme de soins. CONCLUSIONS: Grâce à notre enquête, nous disposons maintenant d'un portrait à jour des soins aux patients atteints de cardiopathie congénitale en Saskatchewan; l'exercice nous a par ailleurs permis de cerner d'importantes lacunes à cet égard.

A cardiovascular disease risk factor in children with congenital heart disease: unmasking elevated waist circumference - a CHAMPS* study *CHAMPS: Children's Healthy-Heart Activity Monitoring Program in Saskatchewan.

BACKGROUND: Children with congenital heart disease (CHD) have an elevated risk of future cardiovascular disease but the underlying mechanisms are unclear. Abdominal obesity (measured as waist circumference) is a risk factor for adult onset of cardiovascular diseases and is correlated with low physical activity levels, commonly found in children with congenital heart disease. Elevated waist circumference may be a mechanism by which cardiovascular disease risk is elevated in children with CHD. The purpose of this study was to compare waist circumference between children with and without CHD, while considering potential confounders. We hypothesized that children with CHD would have higher measures of waist circumference when controlling for differences in birthweight, lean mass, and physical activity. METHODS: Thirty-two children with CHD (10.9 ± 2.6 years; 12 female) from the Children's Healthy-Heart Activity Monitoring Program in Saskatchewan, and 23 healthy controls (11.7 ± 2.5 years; 10 female) were studied. Waist circumference, physical activity (physical activity questionnaire), body composition (lean mass; dual x-ray absorptiometry), and birthweight were assessed. Analysis of covariance, Mann-Whitney U, and independent sample t-tests were used to assess group differences (p < 0.05). RESULTS: Children with CHD had greater waist circumference than controls, controlling for lean mass, physical activity, birthweight, and sex (F (1, 49) = 4.488, p = 0.039). Physical activity, lean mass, and birthweight were not significantly different between groups (p > 0.05). CONCLUSION: Our findings generate a novel hypothesis-higher waist circumferences in children with CHD compared to age-matched controls, may contribute to an elevated risk of cardiovascular disease.

Health anxiety and associated constructs in children and adolescents with congenital heart disease: A CHAMPS cohort study.

This study explored health anxiety and associated constructs in children and adolescents with congenital heart disease and typically developing children and adolescents. A total of 84 participants (7-16 years) completed measures of health anxiety, intolerance of uncertainty, anxiety sensitivity, and DSM-IV anxiety disorder symptom categories. Results demonstrated that children and adolescents with congenital heart disease experienced significantly higher levels of health anxiety and associated constructs compared to typically developing children and adolescents. Our findings highlight a specific chronic physical health population who may be at risk of clinical levels of health anxiety and related psychopathology and require appropriate intervention.

Physical activity modulates arterial stiffness in children with congenital heart disease: A CHAMPS cohort study.

Children with congenital heart disease are at risk for developing increased arterial stiffness and this may be modulated by physical activity. OBJECTIVE: To compare arterial stiffness in high- and low-physically active children with congenital heart disease and healthy age- and sex-matched controls. PATIENTS: Seventeen children with congenital heart disease (12 ± 2 years; females = 9), grouped by low- and high-physical activity levels from accelerometry step count values, and 20 matched controls (11 ± 3 years; females = 9) were studied. OUTCOME MEASURES: Carotid-radial pulse wave velocity was assessed with applanation tonometry to determine arterial stiffness. Body composition and 6-min walk test measures were performed. Data were analyzed using analysis of variance and multiple regression. Significance was P < .05. RESULTS: Arterial stiffness was increased in low-physically active children with congenital heart disease (9.79 ± 0.97 m/s) compared to high-physically active children with congenital heart disease (7.88 ± 0.71 m/s; P = .002) and healthy-matched controls (8.67 ± 1.28 m/s; P = .015). There were no differences in body composition measures between groups (all P > .05), but 6-min walk test distance was less in both congenital heart disease groups (high-physically active: 514 ± 40 m; low-physically active: 539 ± 49 m) versus controls (605 ± 79 m; all P < .05). Average daily step count significantly predicted arterial stiffness in children with congenital heart disease (R2 = 0.358) with a negative correlation (R = -0.599, P = .011), while % fat mass (P = .519) and % lean mass (P = .290) did not predict arterial stiffness. CONCLUSIONS: Low-physically active children with congenital heart disease have increased arterial stiffness compared to high-physically active children with congenital heart disease and healthy-matched controls. Regular physical activity in children with congenital heart disease may modulate arterial stiffness.

Pulmonary vein stenosis of ex-premature infants with pulmonary hypertension and bronchopulmonary dysplasia, epidemiology, and survival from a multicenter cohort.

BACKGROUND: Pulmonary vein stenosis is emerging as an important clinical problem in ex-premature infants. METHODS: We sought to describe the epidemiology of pulmonary vein stenosis affecting ex-premature infants by a multicenter retrospective cohort study of patients from seven children's hospitals diagnosed between 2000-2014. RESULTS: We identified 39 ex-premature patients (26 males, median gestational age 28 weeks range 22-36 weeks, birth weight 1.1 kg range 433-2645-g) with pulmonary vein stenosis. Median age at diagnosis was 6.5 months (1 month-6 years). Presentation with pulmonary hypertension occurred in 26/39 (67%) and 29/39 (74%) had bronchopulmonary dysplasia, 15 (39%) were born of twin pregnancies with unaffected twin siblings. A median of 5 (range 1-25) echocardiograms was performed prior to diagnosis. The diagnosis was made using echocardiography in 22/39 (56%), by multi-detector contrast computed tomography scan (CT) in 8/39 (21%), cardiac catheterization in 6/39 (15%) patients, magnetic resonance imaging in 3/39 (8%). Freedom from death or re-stenosis was 73% at 1-year, 55% at 2, 5, and 10 years. Factors associated with shorter survival or re-stenosis were stenosis of ≥3 pulmonary veins (P < 0.01), bilateral pulmonary vein stenosis (P < 0.01) small for gestational age (P = 0.05), aged <6 months at diagnosis (P < 0.01). CONCLUSION: Pulmonary vein stenosis of ex-premature infants is a complex problem with poor survival, delayed diagnosis, and unsatisfactory treatment. The lack of concordance in twins suggests epigenetic or environmental factors may play a role in the development of pulmonary vein stenosis. In ex-premature infants with pulmonary hypertension and bronchopulmonary dysplasia a focused echocardiographic assessment of the pulmonary veins is required with further imaging if the echocardiogram is inconclusive.

Comparison of two surgical techniques for complete atrioventricular septal defect repair using two- and three-dimensional echocardiography.

Different surgical techniques for complete atrioventricular septal defect (CAVSD) repair have been described, with the double-patch technique being most frequently employed. More recently a newer technique using a modified single-patch repair has been advocated. We hypothesized that the modified single-patch technique would result in an increased incidence of the two major post-repair comorbidities, namely, distortion of the left AV valve (LAVV) leaflets and narrowing of the left-ventricular outflow tract (LVOT). We studied 14 patients with CAVSD who underwent either traditional double-patch technique [group 1 (n = 7)] or modified single-patch technique [group 2 (n = 7)]. Preoperative and immediate postoperative two-dimensional (2D) echocardiograms, as well as follow-up 2D and three-dimensional (3D) studies, were reviewed. For group 1, the median age at repair was 4.1 months with a median duration from surgical repair and last echocardiogram of 44 months. For group 2, the median age at repair was 3 months with a median duration from surgical repair and last echocardiogram of 28 months. The two groups had similar demographics and ventricular septal defect size before surgery. For the LAVV, no significant difference was observed with respect to LAVV annulus size, tenting height, and the size of the vena contracta. Furthermore, there was no significant difference in the 2D echocardiographic areas and volumes of the LVOT between pre-repair and immediate post-repair studies for both groups. At the last evaluation, although there had been growth of the LVOT in both groups, no significant difference between areas and volumes were observed. Areas of the LVOT measured by 3D echocardiography on the final study showed no significant statistical difference between both groups. There was good correlation of the areas measured by 2D and 3D echocardiography within each group. In this small group, modified single-patch technique does not appear to tether the LAVV or promote an increase in regurgitation. In the short term, LVOT growth is unaffected, and the repair does not promote LVOT obstruction. 3D echocardiography is useful for area measurements of the LVOT and showed good correlation with areas measured by assumption of the LVOT shape as determined using 2D techniques.

Effect of fresh red blood cell transfusions on clinical outcomes in premature, very low-birth-weight infants: the ARIPI randomized trial.

CONTEXT: Even though red blood cells (RBCs) are lifesaving in neonatal intensive care, transfusing older RBCs may result in higher rates of organ dysfunction, nosocomial infection, and length of hospital stay. OBJECTIVE: To determine if RBCs stored for 7 days or less compared with usual standards decreased rates of major nosocomial infection and organ dysfunction in neonatal intensive care unit patients requiring at least 1 RBC transfusion. DESIGN, SETTING, AND PARTICIPANTS: Double-blind, randomized controlled trial in 377 premature infants with birth weights less than 1250 g admitted to 6 Canadian tertiary neonatal intensive care units between May 2006 and June 2011. INTERVENTION: Patients were randomly assigned to receive transfusion of RBCs stored 7 days or less (n = 188) vs standard-issue RBCs in accordance with standard blood bank practice (n = 189). MAIN OUTCOME MEASURES: The primary outcome was a composite measure of major neonatal morbidities, including necrotizing enterocolitis, retinopathy of prematurity, bronchopulmonary dysplasia, and intraventricular hemorrhage, as well as death. The primary outcome was measured within the entire period of neonatal intensive care unit stay up to 90 days after randomization. The rate of nosocomial infection was a secondary outcome. RESULTS: The mean age of transfused blood was 5.1 (SD, 2.0) days in the fresh RBC group and 14.6 (SD, 8.3) days in the standard group. Among neonates in the fresh RBC group, 99 (52.7%) had the primary outcome compared with 100 (52.9%) in the standard RBC group (relative risk, 1.00; 95% CI, 0.82-1.21). The rate of clinically suspected infection in the fresh RBC group was 77.7% (n = 146) compared with 77.2% (n = 146) in the standard RBC group (relative risk, 1.01; 95% CI, 0.90-1.12), and the rate of positive cultures was 67.5% (n = 127) in the fresh RBC group compared with 64.0% (n = 121) in the standard RBC group (relative risk, 1.06; 95% CI, 0.91-1.22). CONCLUSION: In this trial, the use of fresh RBCs compared with standard blood bank practice did not improve outcomes in premature, very low-birth-weight infants requiring a transfusion. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT00326924; Current Controlled Trials Identifier: ISRCTN65939658.

The registry and follow-up of complex pediatric therapies program of Western Canada: a mechanism for service, audit, and research after life-saving therapies for young children.

Newly emerging health technologies are being developed to care for children with complex cardiac defects. Neurodevelopmental and childhood school-related outcomes are of great interest to parents of children receiving this care, care providers, and healthcare administrators. Since the 1970s, neonatal follow-up clinics have provided service, audit, and research for preterm infants as care for these at-risk children evolved. We have chosen to present for this issue the mechanism for longitudinal follow-up of survivors that we have developed for western Canada patterned after neonatal follow-up. Our program provides registration for young children receiving complex cardiac surgery, heart transplantation, ventricular assist device support, and extracorporeal life support among others. The program includes multidisciplinary assessments with appropriate neurodevelopmental intervention, active quality improvement evaluations, and outcomes research. Through this mechanism, consistently high (96%) follow-up over two years is maintained.

The age of red blood cells in premature infants (ARIPI) randomized controlled trial: study design.

Despite recent trends in decreasing transfusion thresholds and the development of technologies designed to avoid allogeneic exposure, allogeneic red blood cell (RBC) transfusions remain an important supportive and life-saving measure for neonatal intensive care patients experiencing illness and anemia. Reluctantly, a number of laboratory and observational studies have indicated that the amount of time RBCs are stored can affect oxygen delivery to tissues. Consequently, older RBCs may result in higher rates of organ dysfunction, nosocomial infection, and lengths of stay. Because of such harmful effects, an evaluation of the association between age of blood and nosocomial infection and organ dysfunction is warranted. The aim of the study was to determine if RBCs stored for 7 days or less (fresh RBCs) compared to current standard transfusion practice decreases major nosocomial infection and organ dysfunction in neonates admitted to the neonatal intensive care unit and requiring at least one RBC transfusion. This study is a double-blind, multicenter, randomized controlled trial design. The trial will be an effectiveness study evaluating the effectiveness of stored vs fresh RBCs in neonates requiring transfusion. Neonatal patients requiring at least one unit of RBCs will be randomized to receive either (1) RBCs stored no longer than 7 days or (2) standard practice. The study was conducted in Canadian university-affiliated level III (tertiary) neonatal intensive care units. The primary outcome for this study will be a composite measure of major neonatal morbidities (necrotizing enterocolitis, retinopathy of prematurity, bronchopulmonary dysplasia, intraventricular hemorrhage, and mortality). Secondary outcomes include individual items of the composite measure and nosocomial infection (bacteremia, septic shock, and pneumonia). The sample size calculations have been estimated based on the formula for 2 independent proportions using an alpha of .05, a (1-beta) of .80, and a 10% noncompliance factor. The baseline rate for our composite measure is estimated to be 65% as indicated by the literature. Assuming a 15% absolute risk reduction with the use of RBCs stored 7 days or less, our estimated total sample size required will be 450 (225 patients per treatment arm). The Age of Red Blood Cells in Premature Infants (ARIPI) trial is registered at the US National Institutes of Health (ClinicalTrials.gov) no. NCT00326924 and current controlled trials ISRCTN65939658.