Neurodevelopmental status and follow-up in preschool children with heart disease in London, UK.

OBJECTIVE: To describe neurodevelopment and follow-up services in preschool children with heart disease (HD). DESIGN: Secondary analysis of a prospectively collected multicentre dataset. SETTING: Three London tertiary cardiac centres. PATIENTS: Preschool children<5 years of age: both inpatients and outpatients. METHODS: We analysed results of Mullen Scales of Early Learning (MSEL) and parental report of follow-up services in a representative convenience sample evaluated between January 2014 and July 2015 within a previous study. RESULTS: Of 971 preschool children: 577 (59.4%) had ≥1 heart operation, 236 (24.3%) had a known diagnosis linked to developmental delay (DD) ('known group') and 130 (13.4%) had history of clinical event linked to DD. On MSEL assessment, 643 (66.2%) had normal development, 181 (18.6%) had borderline scores and 147 (15.1%) had scores indicative of DD. Of 971 children, 609 (62.7%) were not receiving follow-up linked to child development and were more likely to be under these services with a known group diagnosis, history of clinical event linked to DD and DD (defined by MSEL). Of 236 in known group, parents of 77 (32.6%) and of 48 children not in a known group but with DD 29 (60.4%), reported no child development related follow-up. DD defined by MSEL assessment was more likely with a known group and older age at assessment. CONCLUSIONS: Our findings indicate that a 'structured neurodevelopmental follow-up pathway' in preschool children with HD should be considered for development and evaluation as children get older, with particular focus on those at higher risk.

Childhood Extracorporeal Membrane Oxygenation Survivors: Parents Highlight Need for Structured Follow-Up and Support After Hospital Discharge.

OBJECTIVES: To examine parental experiences of childhood extracorporeal membrane oxygenation survivors to understand: 1) the problems children faced and 2) the support received following hospital discharge. DESIGN: Single-center descriptive study. SETTING: Nationally commissioned center for neonatal and pediatric extracorporeal membrane oxygenation. PATIENTS: All traceable survivors less than 18 years old who received extracorporeal membrane oxygenation from January 1998 to April 2013. INTERVENTION: Anonymized postal questionnaire completed by parents of extracorporeal membrane oxygenation survivors. MEASUREMENTS AND MAIN RESULTS: Parent-reported developmental problems, follow-up, and the degree of satisfaction with any follow-up experience. Parents of 89 of 366 extracorporeal membrane oxygenation survivors (24%) responded. Sixty-six (74%) reported having developmental concerns about their child, including speech and language (n = 32; 36%), concentration (n = 28; 31%), movement/physical difficulties (n = 26; 29%), and educational difficulties (n = 22; 25%); 46 (52%) indicated that their child had difficulties across multiple domains. Twenty-one (34%) of those with one or more reported developmental concerns were not receiving any follow-up. However, 57 (64%) attended our 1-year follow-up extracorporeal membrane oxygenation clinic and 54 of 57 (95%) found it very useful. Three themes related to perceived need were identified from parents' free-text comments: the need for an expert point of contact and follow-up at the extracorporeal membrane oxygenation center; more information on extracorporeal membrane oxygenation and any long-term effects; and more support from, and easier access to, community specialist services. CONCLUSIONS: A proportion of children who have undergone extracorporeal membrane oxygenation treatment have needs that are not being met, with variable access to service provision. Structured follow-up after discharge would enable early identification of developmental concerns, permit early referral or intervention, and provide support to families. Education and sharing of information about extracorporeal membrane oxygenation with general practitioners/family physicians, community professionals, and schools are essential.

Further investigations of the IgE response to tetanus and diphtheria following covaccination with acellular rather than cellular Bordetella pertussis.

BACKGROUND: It has previously been shown in an uncontrolled study that the IgE response to vaccine antigens is downregulated by co-vaccination with cellular Bordetella pertussis vaccine. METHODS: In the present study, we compared in a controlled trial the humoral immune response to diphtheria toxoid (D) and tetanus toxoid (T) in relation to co-vaccinated cellular or acellular B pertussis vaccine. IgE, IgG4, and IgG to D and T were analyzed at 2, 7, and 12 months of age in sera of children vaccinated with D and T (DT, N = 68), cellular (DTPw, N = 68), 2- or 5-component acellular B pertussis vaccine (DTPa2, N = 64; DTPa5, N = 65). RESULTS: One month after vaccination, D-IgE was detected in 10% sera of DTPw-vaccinated children, whereas vaccination in the absence of whole-cell pertussis resulted in 50%-60% IgE positivity. Six months after vaccination, the IgE antibody levels were found to be more persistent than the IgG antibodies. These diphtheria findings were mirrored by those for tetanus. Only minor differences between vaccine groups were found with regard to D-IgG and T-IgG. No immediate-type allergic reactions were observed. CONCLUSION: Cellular (but not acellular) B pertussis vaccine downregulates IgE to co-vaccinated antigens in infants. We assume that the absence of immediate-type allergic reactions is due to the high levels of IgG antibodies competing with IgE antibodies.

Validation of the Brief Developmental Assessment in pre-school children with heart disease.

Introduction The objective of this study was to prospectively validate the "Brief Developmental Assessment", which is a new early recognition tool for neurodevelopmental abnormalities in children with heart disease that was developed for use by cardiac teams. METHODS: This was a prospective validation study among a representative sample of 960 pre-school children with heart disease from three United Kingdom tertiary cardiac centres who were analysed grouped into five separate age bands. RESULTS: The "Brief Developmental Assessment" was successfully validated in the older four age bands, but not in the youngest representing infants under the age of 4 months, as pre-set validation thresholds were met - lower 95% confidence limit for the correlation coefficient above 0.75 - in terms of agreement of scores between two raters and with an external measure the "Mullen Scales of Early Learning". On the basis of American Association of Pediatrics Guidelines, which state that the sensitivity and specificity of a developmental screening tool should fall between 70 and 80%, "Brief Developmental Assessment" outcome of Red meets this threshold for detection of Mullen scores >2 standard deviations below the mean. CONCLUSION: The "Brief Developmental Assessment" may be used to improve the quality of assessment of children with heart disease. This will require a training package for users and a guide to action for abnormal results. Further research is needed to determine how best to deploy the "Brief Developmental Assessment" at different time points in children with heart disease and to determine the management strategy in infants younger than 4 months old.

The 1-Year Follow-Up Clinic for Neonates and Children After Respiratory Extracorporeal Membrane Oxygenation Support: A 10-Year Single Institution Experience.

OBJECTIVES: To establish the effectiveness of a "1-year extracorporeal membrane oxygenation follow-up clinic" and to characterize any neurodevelopmental concerns identified. DESIGN: Single-center retrospective cohort of respiratory extracorporeal membrane oxygenation survivors over 10 years. SETTING: Nationally commissioned center for neonatal and pediatric (> 28 d of life) respiratory extracorporeal membrane oxygenation. PATIENTS: Children attending the follow-up clinic 1 year after receiving respiratory extracorporeal membrane oxygenation between 2003 and 2013. INTERVENTIONS: Standardized follow-up 1 year after extracorporeal membrane oxygenation. MEASUREMENTS AND MAIN RESULTS: In 10 years, 290 children received extracorporeal membrane oxygenation, 194 (67%) survived; all were offered 1-year follow-up, and 98 (51%) attended the clinic. Among these, 51 of 98 (52%) had meconium aspiration syndrome, and 74 of 98 (75%) were on veno-arterial extracorporeal membrane oxygenation with a median (interquartile range) duration of 6 days (4-8 d). Neurodevelopmental problems were identified in 30 of 98 (30%). The specific abnormalities noted included neurologic (seizures, motor, or vision abnormalities) (n = 8), hearing with/without language delay (n = 8), and behavioral problems (as reported by parents) (n = 6), with eight of 30 (27%) having difficulties spanning these domains. An acute neurologic event on extracorporeal membrane oxygenation was found to be the only risk factor for neurodevelopmental concerns (p = 0.006 with odds ratio 5.4 [95% CI, 1.63-17.92]). Despite having neither a cardiac arrest nor an acute neurologic event documented, 18 of 74 (24.3%), 95% CI (15.1-35.7), had neurodevelopmental concerns at 1-year follow-up. Among the nonattenders, 30 (15%) had local follow-up, and 66 (34%) were lost to follow-up. CONCLUSIONS: All extracorporeal membrane oxygenation survivors need follow-up either at the extracorporeal membrane oxygenation center or in their community, as evidenced by the 1-year follow-up data. Our 1-year extracorporeal membrane oxygenation follow-up clinic provides an opportunity to engage with families, identify neurodevelopmental concerns, and signpost to appropriate services. Of concern, one third of survivors are lost to follow-up, some with an acute neurologic event on extracorporeal membrane oxygenation, a significant risk factor. A consensus-based standardized national follow-up program is vital.

Reliability of team-based self-monitoring in critical events: a pilot study.

BACKGROUND: Teamwork is a critical component during critical events. Assessment is mandatory for remediation and to target training programmes for observed performance gaps. METHODS: The primary purpose was to test the feasibility of team-based self-monitoring of crisis resource management with a validated teamwork assessment tool. A secondary purpose was to assess item-specific reliability and content validity in order to develop a modified context-optimised assessment tool.We conducted a prospective, single-centre study to assess team-based self-monitoring of teamwork after in-situ inter-professional simulated critical events by comparison with an assessment by observers. The Mayo High Performance Teamwork Scale (MHPTS) was used as the assessment tool with evaluation of internal consistency, item-specific consensus estimates for agreement between participating teams and observers, and content validity. RESULTS: 105 participants and 58 observers completed the MHPTS after a total of 16 simulated critical events over 8 months. Summative internal consistency of the MHPTS calculated as Cronbach's alpha was acceptable with 0.712 for observers and 0.710 for participants. Overall consensus estimates for dichotomous data (agreement/non-agreement) was 0.62 (Cohen's kappa; IQ-range 0.31-0.87). 6/16 items had excellent (kappa > 0.8) and 3/16 good reliability (kappa > 0.6). Short questions concerning easy to observe behaviours were more likely to be reliable. The MHPTS was modified using a threshold for good reliability of kappa > 0.6. The result is a 9 item self-assessment tool (TeamMonitor) with a calculated median kappa of 0.86 (IQ-range: 0.67-1.0) and good content validity. CONCLUSIONS: Team-based self-monitoring with the MHPTS to assess team performance during simulated critical events is feasible. A context-based modification of the tool is achievable with good internal consistency and content validity. Further studies are needed to investigate if team-based self-monitoring may be used as part of a programme of assessment to target training programmes for observed performance gaps.