Isolated CHDs and neurodevelopmental follow-up using the Bayley Scales of Infant and Toddler Development and the Ages and Stages Questionnaire at 18 and 36 months.

OBJECTIVES: To compare early neurocognitive development in children born with and without isolated CHD using the Bayley Scales of Infant and Toddler Development (3rd edition) and the Ages and Stages Questionnaire (3rd edition). METHODS: Recruitment took place before birth. Women expecting fetuses with and without CHD causing disturbances in the flow of oxygenated blood to the fetal brain were included in a prospective cohort study comprising fetal MRI (previously published) and neurodevelopmental follow-up. We now present the 18- and 36-month neurodevelopmental follow-up using the Bayley Scales according to age and the 6-month-above-age Ages and Stages Questionnaire in 15 children with and 27 children without CHD. RESULTS: Children with CHD had, compared with the children without CHD, an increased risk of scoring ≤ 100 in the Bayley Scales cognition category at 18 and 36 -months; relative risk 1.7 (95% confidence interval (CI): 1.0-2.8) and 3.1 (CI: 1.2-7.5), respectively. They also achieved lower scores in the 6-month-above-age Ages and Stages Questionnaires (24 and 42 months) communication; mean z-score difference -0.72 (CI: -1.4; -0.1) and -1.06 (CI: -1.8; -0.3) and gross motor; mean z-score difference: -0.87 (CI: -1.7; -0.1) and -1.22 (CI: -2.4; -0.02) categories. CONCLUSIONS: The children with CHD achieved lower scores in the Bayley Scales cognition category and the Ages and Stages Questionnaire communication and gross motor categories possibly indicative of early neurodevelopmental deficiencies. We recommend early screening and monitoring for neurodevelopmental delays in children with CHD in order to improve further neurodevelopment and educational achievements.

Chronic pain in children after cardiac surgery via sternotomy.

INTRODUCTION: Chronic pain is common after sternotomy in adults with reported prevalence rates of 20-50%. So far, no studies have examined whether children develop chronic pain after sternotomy. MATERIAL AND METHODS: Postal questionnaires were sent to 171 children 10-60 months after undergoing cardiac surgery via sternotomy at the age of 0-12 years. The children were asked to recall the intensity and duration of their post-operative pain, if necessary with the help from their parents, and to describe the intensity and character of any present pain. Another group of 13 children underwent quantitative sensory testing of the scar area 3 months after sternotomy. RESULTS: A total of 121 children, median (range) age 7.7 (4.2-16.9) years, answered the questionnaire. Their age at the time of surgery was median (range) 3.8 (0-12.9) years, and the follow-up period was median (range) 4 (0.8-5.1) years. In all, 26 children (21%) reported present pain and/or pain within the last week located in the scar area; in 12 (46%) out of the 26 children, the intensity was ≥4 on a numeric rating scale (0-10). Quantitative sensory testing of the scar area revealed sensory abnormalities--pinprick hyperalgesia and brush and cold allodynia--in 10 out of 13 children. CONCLUSION: Chronic pain after cardiac surgery via sternotomy in children is a problem that should not be neglected. The pain is likely to have a neuropathic component as suggested by the sensory abnormalities demonstrated by quantitative sensory testing.

Transposition of the great arteries - a phenotype associated with 16p11.2 duplications?

Genetic analyses of patients with transposition of the great arteries have identified rare copy number variations, suggesting that they may be significant to the aetiology of the disease. This paper reports the identification of a 16p11.2 microduplication, a variation that has yet to be reported in association with transposition of the great arteries. The 16p11.2 microduplication is associated with autism spectrum disorder and developmental delay, but with highly variable phenotypic effects. Autism and attention deficit disorders are observed more frequently in children with congenital heart disease than in the general population. Neonatal surgery is proposed as a risk factor, but as yet unidentified genetic abnormalities should also be taken into account. Thus, congenital heart abnormalities may constitute a part of the phenotypic spectrum associated with duplications at 16p11.2. We suggest chromosomal microarray be considered part of the diagnostic work-up in patients with transposition of the great arteries.

Cerebral Oxygenation Measurements by Magnetic Resonance Imaging in Fetuses With and Without Heart Defects.

BACKGROUND: Children with major congenital heart defects are risking impaired cerebral growth, delayed cerebral maturation, and neurodevelopmental disorders. We aimed to compare the cerebral tissue oxygenation of fetuses with major heart defects to that of fetuses without heart defects as estimated by the magnetic resonance imaging modality T2*. T2* is low in areas with high concentrations of deoxyhemoglobin. METHODS AND RESULTS: At gestational age mean 32 weeks (early) and mean 37 weeks (late), we compared the fetal cerebral T2* in 28 fetuses without heart defects to that of 15 fetuses with major heart defects: transposition of the great arteries (n=7), coarctation of the aorta/hypoplastic aortic arch (n=5), tetralogy of Fallot (n=1), hypoplastic right heart (n=1), and common arterial trunk (n=1). The women were scanned with a 1.5 T Philips scanner using a breath-hold multiecho gradient echo sequence. Among fetuses without heart defects, the mean T2* value was 157 ms (95% confidence interval [CI], 152-163) early and 125 ms (95% CI, 120-130) late. These figures were significantly lower (mean 14 ms; 95% CI, 6-22; P<0.001) among fetuses with heart defects 143 ms (95% CI, 136-150) early and 111 ms (95% CI, 104-118) late. CONCLUSIONS: Our findings indicate that fetal cerebral T2* is measurable and that fetal cerebral tissue oxygenation measured by T2* is lower in fetuses with heart defects compared with fetuses without heart defects. This corroborates the hypothesis that tissue hypoxia may be a potential pathogenic factor that possibly affects brain development in fetuses with heart defects.

[The effect of Christmas joy on the mood among medical doctors - a randomized, blinded intervention study].

INTRODUCTION: Each December Santa's elves spread Christmas joy (JN). Laughter and humour may influence health and stress level. No other study has investigated the effect of JN on the good spirit (DGH) among healthcare professionals. MATERIAL AND METHODS: We performed a single-centre blinded intervention study with crossover at three hospital departments. JN intervention of three days was randomized. Median ± standard deviation was given. The level of significance was p < 0.05. RESULTS: During a four-week period, we made 24 observations (response rate 67). The laugh index increased from 0.02 in November to 0.03 in December (without JN) and further to 0.05 with JN. At one department, the rise was significant. At a department without morning coffee, the DGH level raised after JN intervention corresponding to the level at the departments with morning coffee before JN intervention. CONCLUSION: Christmas atmosphere tended to increase DGH at the morning conferences. JN tended to have an additive effect. JN exposure may be beneficial. FUNDING: The study did not receive any funding. TRIAL REGISTRATION: The trial was not registered and was kept secret for the participants in accordance with the tradition of Santa's elves.