Predictors of health-related quality of life in children with chronic heart disease.

OBJECTIVE: Chronic paediatric heart disease is often associated with residual symptoms, persisting functional restrictions, and late sequelae for psychosocial development. It is, therefore, increasingly important to evaluate the health-related quality of life of children and adolescents with chronic heart disease. The aim of this study was to determine medical and socio-demographic variables affecting health-related quality of life in school-aged children and adolescents with chronic heart disease. Patients and methods The Pediatric Cardiac Quality of Life Inventory was administered to 375 children and adolescents and 386 parental caregivers. Medical information was obtained from the charts. The socio-demographic information was provided by the patients and caregivers. RESULTS: Greater disease severity, low school attendance, current cardiac medication, current parental employment, uncertain or limited prognosis, history of connection to a heart-lung machine, number of nights spent in a hospital, and need for treatment in a paediatric aftercare clinic independently contributed to lower health-related quality of life (self-report: R2=0.41; proxy-report: R2=0.46). High correlations between self-reports and parent-proxy reports indicated concordance regarding the evaluation of a child's health-related quality of life. CONCLUSIONS: Beyond medical treatment, integration into school is important to increase health-related quality of life in children and adolescents surviving with chronic heart disease. Regular screening of health-related quality of life is recommended to identify patients with special needs.

Overweight prevention implemented by primary school teachers: a randomised controlled trial.

OBJECTIVE: To describe the effects of URMEL-ICE, a German school-based intervention for overweight prevention, on children's BMI and other measures of fat mass. METHODS: A cluster-randomised controlled design was used. The intervention which focused on physical activity, TV time and soft drink consumption was integrated into a second-grade curriculum and was implemented by classroom teachers themselves. It comprised 29 teaching lessons, 2 short exercise blocks per day and 6 family homework lessons. BMI was assessed as primary outcome measure, waist circumference and skinfold thickness as secondary outcomes. Data of 945 children were analysed. RESULTS: Multivariate analyses adjusted for baseline values showed no statistically significant effect of the intervention on BMI, but on waist circumference (-0.85; 95% confidence interval (95% CI) -1.59 to -0.12) and subscapular skinfold thickness (-0.64; 95% CI -1.25 to -0.02). After additional adjustment for individual time lag between baseline and follow-up, these effects were reduced to -0.60 (95% CI -1.25 to 0.05) and -0.61 (95% CI -1.26 to 0.04) and lost their statistical significance. CONCLUSION: This study contributes to the field of randomised school-based studies on overweight prevention and shows that within a 1-year, integrated intervention no effect on BMI, but a tendency towards effects on fat mass can be achieved.

Determinants of obesity in the Ulm Research on Metabolism, Exercise and Lifestyle in Children (URMEL-ICE).

We investigated the prevalence of overweight and obesity in German schoolchildren and analyzed determinants of overweight. In the context of a randomized intervention study, a baseline cross-sectional assessment was carried out in 2006. During a physical examination, height, weight, skin fold thickness, and upper arm and waist circumferences were measured according to a standardized protocol among 1.079 children aged 6-9 years. Overweight and obesity were classified according to the definitions of the International Obesity Task Force. Parents completed a questionnaire on potential determinants of overweight. Logistic regression models were calculated for determinants of overweight and obesity. The prevalence of overweight was 16.5% in boys and 17.3% in girls and of obesity 3.5% and 3.6%, respectively. Migration (29.4 %) was correlated with overweight and obesity. In particular, among boys with migration background, overweight (24.0%) and obesity (6.6%) were highly prevalent. Higher obesity prevalence was associated with maternal smoking during pregnancy, parental overweight, and low parental education. Indicators for physical inactivity such as watching television more than 1 h per weekday, participation in club sports less than once a week, consumption of sweetened drinks (>or=3 times per week), and skipping breakfast before school were associated with childhood obesity. Our results provide further evidence that parental factors such as migration background and education are strongly associated with body mass of the offspring. Physically inactive children with regular consumption of sweetened drinks and no breakfast were prone to be overweight or obese. Changes of these lifestyle factors as targets of interventions are promising to prevent childhood obesity.

A new treatment option for pulmonary valvar insufficiency: first experiences with implantation of a self-expanding stented valve without use of cardiopulmonary bypass.

OBJECTIVE: Pulmonary regurgitation is the predominant problem in the long-term follow-up of tetralogy of Fallot (TOF) patients after primary repair. Apart from standard homograft implantation, a percutaneous valve delivery approach has been described recently. A right ventricular outflow tract (RVOT) diameter of greater than 22mm, however, precludes percutaneous valve delivery. We describe a novel technique with a transventricular implantation of a stented bio-prosthesis without cardiopulmonary bypass that allows for implantation of prosthesis with diameters greater than 22mm. METHODS: All patients (9-27 years of age) had undergone total correction of TOF at a mean age of 4.2+/-4.0 years. The RVOT was enlarged at that time with a transannular patch in all but one patient. All patients presented with severe pulmonary regurgitation without any significant RVOT obstruction. Mean MRI pulmonary regurgitation was 53+/-8%. The mean magnetic resonance imaging (MRI) right ventricular end diastolic volume index (RVEDVI) was 143+/-23ml/m(2), with a mean MRI right ventricular ejection fraction (RVEF) of 46+/-9%. In another two patients indication for treatment was based on reduced exercise capacity with patients being in NYHA Class III. After repeat sternotomy, a porcine valve mounted inside a self-expandable stent, covered with No-React treated porcine pericardium (Shelhigh, Model NR-4000MIS), was introduced just beneath the RVOT without use of cardiopulmonary bypass. External sutures were placed at the proximal and distal site of the valve to ensure fixation. RESULTS: The implantations were uneventful, with the patients hemodynamically stable throughout the procedure. One patient with severely dilated RVOT (up to 31mm) exhibited paravalvular leakage and the valve was replaced by a homograft after 2 days. At 6-12 month follow-up the remaining five patients exhibited no more than mild pulmonary regurgitation. The mean MRI RVEDVI was 94+/-18ml/m(2), with a mean MRI RVEF of 58+/-27%. CONCLUSIONS: Cardiopulmonary bypass for repeat RVOT interventions can be avoided in selected patients with this newly available device. In combination with a wide range of prosthesis sizes it offers yet another important treatment option.

Familial polycythemia vera with Budd-Chiari syndrome in childhood.

Polycythemia vera is a myeloproliferative disorder that, in most cases, occurs sporadically with a median age at presentation of 60 years. Familial cases are very rare and usually manifest in elderly family members. The Budd-Chiari syndrome, characterized by the obstruction and occlusion of the suprahepatic veins, is a rare typical complication in polycythemia vera patients. To date, only two children or adolescents with polycythemia vera and Budd-Chiari syndrome have been described. Here, we report an 11-year-old girl with Budd-Chiari syndrome as the initial symptom of familial polycythemia vera, which was also found in the girl's grandmother. Details of the diagnostic procedures used and the clinical course are reported. The patient underwent orthotopic liver transplantation and is being treated with hydroxyurea. The available literature on familial polycythemia vera and polycythemia vera in childhood with and without Budd-Chiari syndrome is reviewed.