"Quality of life" analysis in the long-term follow-up after "Fontan" palliation for CHDs-a single-centre experience.

BACKGROUND: Complex CHDs are life threatening, and surgical treatment is needed for survival. Fontan palliation led to a significant increase in survival rates during the last decades. Consequently, quality of life became more essential. While a reduced quality of life compared to healthy children has been reported, detailed knowledge about individual quality of life and particular areas is lacking. Furthermore, the effect of different risk factors on quality of life is only rarely evaluated. METHOD AND RESULTS: Database of the department for pediatric cardiology, Heart Center Leipzig, was screened for children after total cavopulmonary connection palliation. n = 39 patients were included in the study, the outcome after total cavopulmonary connection was analysed in detail and quality of life data were collected and analysed using the standardised questionnaire "Pediatric quality of life inventory", version 4.0. We compared the total health score of our patients to the mean score of healthy children in the literature. The mean follow-up time was 6.4 ± 3.2 years, the overall survival was 100% after maximal follow-up time of 11.1 years. We could not find any age or gender dependence, nor an influence of age at total cavopulmonary connection on the later quality of life. Yet, patients with three-staged surgery exhibited a worse quality of life than patients with two-staged palliation. Late complications might influence quality of life, but patient number is too small, to find universal results. CONCLUSION: The total cavopulmonary connection palliation affects physical and psychological quality of life as well as cardiac health independently from age and gender. More patients and longer observation should be examined to confirm the results.

Open field stress testing: finally an optimal method in young children? Reference values for mobile cardiopulmonary exercise testing in healthy children aged 4-8 years.

INTRODUCTION: Cardiopulmonary exercise testing represents the diagnostic tool for determining cardiopulmonary function. Especially in small children, exercise testing is extremely challenging. To address this problem, field testing has been implemented using small mobile devices. This study aims at using this protocol for developing normal values for cardiopulmonary exercise testing in very young children. MATERIAL AND METHODS: Healthy children aged 4-8 years were recruited. All children were tested according to an outdoor protocol, in which they were instructed to walk, then run slowly, then a little harder and at last run at full speed. Each step lasted for 2 minutes, except the last step, in which the children were instructed to maintain as long as possible. RESULTS: A total of 104 children (64 female/35 male, mean age 6.6 years) performed outdoor cardiopulmonary exercise testing using a mobile device. Almost all tests were completed successfully (95%). Despite a predominance of female study subjects, anthropometric values did not differ between boys and girls. V̇O2peak/kg, respiratory exchange ratio, VT1, heart rate at VT1, and time of exercise were also comparable between sexes. Generally, a tendency of higher maximal oxygen uptake could be observed in older children. CONCLUSION: Open field mobile cardiopulmonary exercise testing represents a novel approach in very young children. In this study, we were able to determine normal values of maximal oxygen uptake and OUES/kg for 4-8-year-old children. The method is easy to achieve and safe.

X-ray micro-computed-tomography in pediatric surgery: a new tool for studying embryos.

PURPOSE: The embryology of common congenital malformations is discussed controversially. Studies are hampered by a shortage of study material and techniques which require partial or complete preparation and therewith destruction of embryos. X-ray micro-computed-tomography (µCT) is a technical opportunity keeping the embryos intact. Thus, the aim of this study was to assess the applicability of µCT in embryonic research compared to the anatomical information obtained by scanning electron microscopy (SEM). METHODS: Chicken, rat, mouse and sheep embryos, processed either for SEM studies or as whole embryos, were imaged in three-dimensional (3D) using µCT. The obtained two-dimensional (2D) digital datasets were volume rendered by tomographic reconstruction software and studied using analysis software. RESULTS: All embryos were µCT scanned without technical problems. The quality of the µCT images (image contrast, anatomical details) was excellent, but varied depending on age and species studied. µCT imaging allowed a more comprehensive anatomical/morphological analysis but showed less surface details compared to SEM. CONCLUSION: µCT is a technique suitable and innovative for pediatric surgical research, which allows detailed evaluation of entire embryos without time- and specimen-consuming micro-dissection. Samples prepared for SEM can be used for µCT and vice versa.