Issues facing families of infants discharged after cardiac surgery: the perceptions of charity helpline staff.

AIM: To elicit the perceptions of helpline staff who talk to parents of children discharged after cardiac surgery in infancy about parents' key concerns. METHODS: A qualitative study involving semistructured interviews with 10 staff at four heart charities. Interviews were recorded, transcribed and analysed using Framework analysis. RESULTS: Staff identified the knowledge, communication and support needs of parents which they described in terms of the impact of patient and family factors, sources of support and systems. Staff perceptions of helplines, in terms of the function of a helpline and the roles of its staff, together with staff's personal views based on their experience of multiple encounters with many families, influenced how they viewed families' needs and responded to their requests. CONCLUSION: Helpline staff provided important, previously uncaptured evidence about the challenges faced by parents of children discharged after cardiac surgery in infancy. Staff have an important role in supporting communication, in terms of speaking to families about how to talk to professionals and talking to professionals directly to get or give information when parents are unable to do so. Capturing the perspective of helpline staff about communication issues has highlighted the need for interventions with professionals as well as parents.

Parents' Experiences of Caring for Their Child at the Time of Discharge After Cardiac Surgery and During the Postdischarge Period: Qualitative Study Using an Online Forum.

BACKGROUND: Congenital heart disease (CHD) is the most common class of birth defects, which encompasses a broad spectrum of severity ranging from relatively minor to extremely complex. Improvements in surgery and intensive care have resulted in an increasing number of infants with the most complex lesions surviving after surgery until the time of discharge from the hospital, but there remain concerns about out-of-hospital mortality, variability in how services are provided at the time of discharge and beyond, and difficulties experienced by some families in accessing care. OBJECTIVE: As part of a mixed-methods program of research, this study aimed to elicit parental experiences of caring for a child with CHD after hospital discharge following a cardiac surgery and collect information to inform interviews for a subsequent stage of the project. METHODS: A closed online discussion group was set up via the main Facebook page of the Children's Heart Federation (CHF), a national charity offering support to children with heart disease and their families. The discussion group was advertised through the charity's webpage, and interested participants were directed to the charity's Facebook page from where they could access the closed Facebook group and respond to questions posted. The CHF moderated the forum, and the research team provided questions to be posted on the forum. Responses were collated into a single transcript and subjected to thematic analysis. RESULTS: The forum was open for 4 months, and 91 participants (mean age 35 years, range 23-58 years, 89 females, 89 parents, and 2 grandparents) submitted demographic information and were given access to the closed forum group. A common experience of isolation emerged from the data, with descriptions of how that isolation was experienced (physical, social, knowledge) and its psychological impact, together with the factors that made it worse or better. Woven through this theme was the notion that parents developed expertise over time. CONCLUSIONS: The use of an online forum provided a means for eliciting data from a large number of parents regarding their experiences of caring for their child after hospital discharge following cardiac surgery. Parents engaged with the forum and were able to articulate what went well and what went less well, together with sharing their stories and supporting each other through doing so. Some parents clearly found participating in the forum a positive experience in itself, demonstrating the potential of social media as a mechanism for providing support and reducing isolation. Information gained from the forum was used to shape questions for interviews with parents in a subsequent phase of the study. Furthermore, the themes identified in the online forum have contributed to identifying ways of improving the provision of care and support for parents of high-risk babies following discharge after cardiac surgery.

"I was so worried about every drop of milk" - feeding problems at home are a significant concern for parents after major heart surgery in infancy.

Increasing numbers of operations in small infants with complex congenital heart disease are being carried out in the UK year on year, with more surviving the initial operation. However, even after successful surgery some of these infants remain fragile when they are discharged home. The aim of the study was to elicit parents' experiences of caring for a child with complex needs after major congenital heart surgery. We conducted a qualitative study involving semi-structured interviews with parents of 20 children (aged <1-5 months at hospital discharge), who had undergone open heart surgery and subsequently died or been readmitted unexpectedly to intensive care following their initial discharge home. Feeding difficulties following discharge from the specialist surgical centre emerged as one of the most significant parental concerns spontaneously raised in interviews. For some parents the impact of feeding difficulties overshadowed any other cardiac concerns. Key themes centred around feeding management (particularly the practical challenges of feeding their baby), the emotional impact of feeding for parents and the support parents received or needed after discharge with respect to feeding. Caring for a child with congenital heart disease following surgery is demanding, with feeding difficulties being one of the most significant parent stressors. Local health professionals can be a good source of support for parents provided that they are well informed about the needs of a cardiac baby and have realistic expectations of weight gain. Specialist surgical centres should consider addressing issues of parental stress around feeding and weight gain prior to hospital discharge. © 2016 Blackwell Publishing Ltd.

Unexpected deaths and unplanned re-admissions in infants discharged home after cardiac surgery: a systematic review of potential risk factors.

BACKGROUND: Babies with CHDs are a particularly vulnerable population with significant mortality in their 1st year. Although most deaths occur in the hospital within the early postoperative period, around one-fifth of postoperative deaths in the 1st year of life may occur after hospital discharge in infants who have undergone apparently successful cardiac surgery. Aim To systematically review the published literature and identify risk factors for adverse outcomes, specifically deaths and unplanned re-admissions, following hospital discharge after infant surgery for life-threatening CHDs. METHODS: A systematic search was conducted in MEDLINE, EMBASE, CINAHL, Cochrane Library, Web of Knowledge, and PsycINFO electronic databases, supplemented by manual searching of conference abstracts. RESULTS: A total of 15 studies were eligible for inclusion. Almost exclusively, studies were conducted in single US centres and focussed on children with complex single ventricle diagnoses. A wide range of risk factors were evaluated, and those more frequently identified as having a significant association with higher mortality or unplanned re-admission risk were non-Caucasian ethnicity, lower socio-economic status, co-morbid conditions, age at surgery, operative complexity and procedure type, and post-operative feeding difficulties. CONCLUSIONS: Studies investigating risk factors for adverse outcomes post-discharge following diverse congenital heart operations in infants are lacking. Further research is needed to systematically identify higher risk groups, and to develop interventions targeted at supporting the most vulnerable infants within an integrated primary and secondary care pathway.

A multimodal approach to emotion recognition ability in autism spectrum disorders.

BACKGROUND: Autism spectrum disorders (ASD) are characterised by social and communication difficulties in day-to-day life, including problems in recognising emotions. However, experimental investigations of emotion recognition ability in ASD have been equivocal, hampered by small sample sizes, narrow IQ range and over-focus on the visual modality. METHODS: We tested 99 adolescents (mean age 15;6 years, mean IQ 85) with an ASD and 57 adolescents without an ASD (mean age 15;6 years, mean IQ 88) on a facial emotion recognition task and two vocal emotion recognition tasks (one verbal; one non-verbal). Recognition of happiness, sadness, fear, anger, surprise and disgust were tested. Using structural equation modelling, we conceptualised emotion recognition ability as a multimodal construct, measured by the three tasks. We examined how the mean levels of recognition of the six emotions differed by group (ASD vs. non-ASD) and IQ (≥ 80 vs. < 80). RESULTS: We found no evidence of a fundamental emotion recognition deficit in the ASD group and analysis of error patterns suggested that the ASD group were vulnerable to the same pattern of confusions between emotions as the non-ASD group. However, recognition ability was significantly impaired in the ASD group for surprise. IQ had a strong and significant effect on performance for the recognition of all six emotions, with higher IQ adolescents outperforming lower IQ adolescents. CONCLUSIONS: The findings do not suggest a fundamental difficulty with the recognition of basic emotions in adolescents with ASD.

Childhood rituals and executive functions.

Repetitive and ritualistic behaviours (RRBs) are a feature of both typical and atypical development. While the cognitive correlates of these behaviours have been investigated in some neurodevelopmental conditions these links remain largely unexplored in typical development. The current study examined the relationship between RRBs and executive functions (EF) in a sample of typically developing children aged between 37 and 107 months. Results showed that cognitive flexibility, and not response inhibition or generativity, was most strongly associated with the frequency of RRBs in this sample. In younger children (<67.5 months) cognitive flexibility was significantly associated with 'Repetitive Behaviours' but in older children (>67.5 months) cognitive flexibility was associated with both 'Just Right' and 'Repetitive Behaviour', suggesting that the association between EF and RRBs may become stronger with age in typically developing children.

Ethnic-specific mortality of infants undergoing congenital heart surgery in England and Wales.

PURPOSE: To investigate ethnic differences in mortality for infants with congenital heart defects (CHDs) undergoing cardiac surgery or interventional catheterisation. DESIGN: Observational study of survival to age 1 year using linked records from routine national paediatric cardiac surgery and intensive care audits. Mortality risk was investigated using multivariable Poisson models with multiple imputation. Predictors included sex, ethnicity, preterm birth, deprivation, comorbidities, prenatal diagnosis, age and weight at surgery, preprocedure deterioration and cardiac diagnosis. SETTING: All paediatric cardiac surgery centres in England and Wales. PATIENTS: 5350 infants with CHDs born from 2006 to 2009. MAIN OUTCOME MEASURE: Survival at age 1 year. RESULTS: Mortality was 83.9 (95% CI 76.3 to 92.1) per 1000 infants, with variation by ethnic group. Compared with those of white ethnicity, infants in British Asian (Indian, Pakistani and Bangladeshi) and 'all other' (Chinese, mixed and other) categories experienced significantly higher mortality by age 1 year (relative risk [RR] 1.52[95% CI 1.19 to 1.95]; 1.62[95% CI 1.20 to 2.20], respectively), specifically during index hospital admission (RR 1.55 [95% CI 1.07 to 2.26]; 1.64 [95% CI 1.05 to 2.57], respectively). Further predictors of mortality included non-cardiac comorbidities, prenatal diagnosis, older age at surgery, preprocedure deterioration and cardiac diagnosis. British Asian infants had higher mortality risk during elective hospital readmission (RR 1.86 [95% CI 1.02 to 3.39]). CONCLUSIONS: Infants of British Asian and 'all other' non-white ethnicity experienced higher postoperative mortality risk, which was only partly explained by socioeconomic deprivation and access to care. Further investigation of case-mix and timing of risk may provide important insights into potential mechanisms underlying ethnic disparities.

The association between theory of mind, executive function, and the symptoms of autism spectrum disorder.

It has been strongly argued that atypical cognitive processes in autism spectrum disorder (ASD) contribute to the expression of behavioural symptoms. Comprehensive investigation of these claims has been limited by small and unrepresentative sample sizes and the absence of wide-ranging task batteries. The current study investigated the cognitive abilities of 100 adolescents with ASD (mean age = 15 years 6 months), using 10 tasks to measure the domains of theory of mind (ToM) and executive function (EF). We used structural equation modelling as a statistically robust way of exploring the associations between cognition and parent-reported measures of social communication and restricted and repetitive behaviours (RRBs). We found that ToM ability was associated with both social communication symptoms and RRBs. EF was a correlate of ToM but had no direct association with parent-reported symptom expression. Our data suggest that in adolescence ToM ability, but not EF, is directly related to autistic symptom expression. Autism Res 2018, 11: 95-109. © 2017 International Society for Autism Research, Wiley Periodicals, Inc. LAY SUMMARY: The behaviours that are common to autism spectrum disorder (ASD) have been linked to differences in thinking ability. We assessed autistic adolescents and found that social communication difficulties and the presence of restricted and repetitive behaviours related to difficulties in understanding other peoples' minds (theory of mind). In contrast, these behaviours were not associated with the general thinking abilities involved in planning and executing tasks (executive function).

Combining qualitative and quantitative operational research methods to inform quality improvement in pathways that span multiple settings.

BACKGROUND: Improving integration and continuity of care across sectors within resource constraints is a priority in many health systems. Qualitative operational research methods of problem structuring have been used to address quality improvement in services involving multiple sectors but not in combination with quantitative operational research methods that enable targeting of interventions according to patient risk. We aimed to combine these methods to augment and inform an improvement initiative concerning infants with congenital heart disease (CHD) whose complex care pathway spans multiple sectors. METHODS: Soft systems methodology was used to consider systematically changes to services from the perspectives of community, primary, secondary and tertiary care professionals and a patient group, incorporating relevant evidence. Classification and regression tree (CART) analysis of national audit datasets was conducted along with data visualisation designed to inform service improvement within the context of limited resources. RESULTS: A 'Rich Picture' was developed capturing the main features of services for infants with CHD pertinent to service improvement. This was used, along with a graphical summary of the CART analysis, to guide discussions about targeting interventions at specific patient risk groups. Agreement was reached across representatives of relevant health professions and patients on a coherent set of targeted recommendations for quality improvement. These fed into national decisions about service provision and commissioning. CONCLUSIONS: When tackling complex problems in service provision across multiple settings, it is important to acknowledge and work with multiple perspectives systematically and to consider targeting service improvements in response to confined resources. Our research demonstrates that applying a combination of qualitative and quantitative operational research methods is one approach to doing so that warrants further consideration.

Ethnic and socioeconomic variation in incidence of congenital heart defects.

INTRODUCTION: Ethnic differences in the birth prevalence of congenital heart defects (CHDs) have been reported; however, studies of the contemporary UK population are lacking. We investigated ethnic variations in incidence of serious CHDs requiring cardiac intervention before 1 year of age. METHODS: All infants who had a cardiac intervention in England and Wales between 1 January 2005 and 31 December 2010 were identified in the national congenital heart disease surgical audit and matched with paediatric intensive care admission records to create linked individual child records. Agreement in reporting of ethnic group by each audit was evaluated. For infants born 1 January 2006 to 31 December 2009, we calculated incidence rate ratios (IRRs) for CHDs by ethnicity and investigated age at intervention, antenatal diagnosis and area deprivation. RESULTS: We identified 5350 infants (2940 (55.0%) boys). Overall CHD incidence was significantly higher in Asian and Black ethnic groups compared with the White reference population (incidence rate ratios (IRR) (95% CIs): Asian 1.5 (1.4 to 1.7); Black 1.4 (1.3 to 1.6)); incidence of specific CHDs varied by ethnicity. No significant differences in age at intervention or antenatal diagnosis rates were identified but affected children from non-White ethnic groups were more likely to be living in deprived areas than White children. CONCLUSIONS: Significant ethnic variations exist in the incidence of CHDs, including for specific defects with high infant mortality. It is essential that healthcare provision mitigates ethnic disparity, including through timely identification of CHDs at screening, supporting parental choice and effective interventions. Future research should explore the factors underlying ethnic variation and impact on longer-term outcomes.

Signs of deterioration in infants discharged home following congenital heart surgery in the first year of life: a qualitative study.

AIMS: To describe the ways in which parents recognise and make decisions about their child's symptoms following discharge home after congenital heart interventions in the first year of life and their experiences of seeking help. METHODS: This was a qualitative study involving semistructured interviews with parents. Twenty-one parents were recruited to the study. Parents all had a child who had congenital heart surgery in their first year of life between September 2009 and October 2013 at one of three UK cardiac centres; the children had either died or were readmitted as an emergency following initial discharge. RESULTS: Some parents were unable to identify any early warning signs. Others described symptoms of deterioration including changes in feeding and appearance, respiratory distress and subtle behavioural changes that may not be routinely highlighted to parents at discharge. Several barriers to accessing prompt medical assistance were identified including parents feeling that their concerns were not taken seriously, long wait times and lack of protocols at A&E. CONCLUSIONS: Our study highlights behavioural symptoms as being a potentially underemphasised sign of deterioration and identifies a number of barriers to parents accessing support when they are concerned. It is important that parents are encouraged to seek advice at the earliest opportunity and that those health professionals at the front line have access to the information they need in order to respond in an appropriate and timely way. A role for home monitoring was also noted as potentially useful in identifying at risk children who appear clinically well.

Identifying improvements to complex pathways: evidence synthesis and stakeholder engagement in infant congenital heart disease.

OBJECTIVES: Many infants die in the year following discharge from hospital after surgical or catheter intervention for congenital heart disease (3-5% of discharged infants). There is considerable variability in the provision of care and support in this period, and some families experience barriers to care. We aimed to identify ways to improve discharge and postdischarge care for this patient group. DESIGN: A systematic evidence synthesis aligned with a process of eliciting the perspectives of families and professionals from community, primary, secondary and tertiary care. SETTING: UK. RESULTS: A set of evidence-informed recommendations for improving the discharge and postdischarge care of infants following intervention for congenital heart disease was produced. These address known challenges with current care processes and, recognising current resource constraints, are targeted at patient groups based on the number of patients affected and the level and nature of their risk of adverse 1-year outcome. The recommendations include: structured discharge documentation, discharging certain high-risk patients via their local hospital, enhanced surveillance for patients with certain (high-risk) cardiac diagnoses and an early warning tool for parents and community health professionals. CONCLUSIONS: Our recommendations set out a comprehensive, system-wide approach for improving discharge and postdischarge services. This approach could be used to address challenges in delivering care for other patient populations that can fall through gaps between sectors and organisations.

Going home after infant cardiac surgery: a UK qualitative study.

OBJECTIVE: To qualitatively assess the discharge processes and postdischarge care in the community for infants discharged after congenital heart interventions in the first year of life. DESIGN: Qualitative study using semistructured interviews and Framework Analysis. SETTING: UK specialist cardiac centres and the services their patients are discharged to. SUBJECTS: Twenty-five cardiologists and nurses from tertiary centres, 11 primary and secondary health professionals and 20 parents of children who had either died after discharge or had needed emergency readmission. RESULTS: Participants indicated that going home with an infant after cardiac intervention represents a major challenge for parents and professionals. Although there were reported examples of good care, difficulties are exacerbated by inconsistent pathways and potential loss of information between the multiple teams involved. Written documentation from tertiary centres frequently lacks crucial contact information and contains too many specialist terms. Non-tertiary professionals and parents may not hold the information required to respond appropriately when an infant deteriorates, this contributing to the stressful experience of managing these infants at home. Where they exist, the content of formal 'home monitoring pathways' varies nationally, and families can find this onerous. CONCLUSIONS: Service improvements are needed for infants going home after cardiac intervention in the UK, focusing especially on enhancing mechanisms for effective transfer of information outside the tertiary centre and processes to assist with monitoring and triage of vulnerable infants in the community by primary and secondary care professionals. At present there is no routine audit for this stage of the patient journey.

Death and Emergency Readmission of Infants Discharged After Interventions for Congenital Heart Disease: A National Study of 7643 Infants to Inform Service Improvement.

BACKGROUND: Improvements in hospital-based care have reduced early mortality in congenital heart disease. Later adverse outcomes may be reducible by focusing on care at or after discharge. We aimed to identify risk factors for such events within 1 year of discharge after intervention in infancy and, separately, to identify subgroups that might benefit from different forms of intervention. METHODS AND RESULTS: Cardiac procedures performed in infants between 2005 and 2010 in England and Wales from the UK National Congenital Heart Disease Audit were linked to intensive care records. Among 7976 infants, 333 (4.2%) died before discharge. Of 7643 infants discharged alive, 246 (3.2%) died outside the hospital or after an unplanned readmission to intensive care (risk factors were age, weight-for-age, cardiac procedure, cardiac diagnosis, congenital anomaly, preprocedural clinical deterioration, prematurity, ethnicity, and duration of initial admission; c-statistic 0.78 [0.75-0.82]). Of the 7643, 514 (6.7%) died outside the hospital or had an unplanned intensive care readmission (same risk factors but with neurodevelopmental condition and acquired cardiac diagnosis and without preprocedural deterioration; c-statistic 0.78 [0.75-0.80]). Classification and regression tree analysis were used to identify 6 subgroups stratified by the level (3-24%) and nature of risk for death outside the hospital or unplanned intensive care readmission based on neurodevelopmental condition, cardiac diagnosis, congenital anomaly, and duration of initial admission. An additional 115 patients died after planned intensive care admission (typically following elective surgery). CONCLUSIONS: Adverse outcomes in the year after discharge are of similar magnitude to in-hospital mortality, warrant service improvements, and are not confined to diagnostic groups currently targeted with enhanced monitoring.

No evidence for a fundamental visual motion processing deficit in adolescents with autism spectrum disorders.

It has been suggested that atypicalities in low-level visual processing contribute to the expression and development of the unusual cognitive and behavioral profile seen in autism spectrum disorders (ASD). However, previous investigations have yielded mixed results. In the largest study of its kind (ASD n = 89; non-ASD = 52; mean age 15 years 6 months) and testing across the spectrum of IQ (range 52-133), we investigated performance on three measures of basic visual processing: motion coherence, form-from-motion and biological motion (BM). At the group level, we found no evidence of differences between the two groups on any of the tasks, suggesting that there is no fundamental visual motion processing deficit in individuals with an ASD, at least by adolescence. However, we identified a tail of individuals with ASD (18% of the sample) who had exceptionally poor BM processing abilities compared to the non-ASD group, and who were characterized by low IQ. For the entire sample of those both with and without ASD, performance on the BM task uniquely correlated with performance on the Frith-Happé animations, a higher-level task that demands the interpretation of moving, interacting agents in order to understand mental states. We hypothesize that this association reflects the shared social-cognitive characteristics of the two tasks, which have a common neural underpinning in the superior temporal sulcus.

'Everyday memory' impairments in autism spectrum disorders.

'Everyday memory' is conceptualised as memory within the context of day-to-day life and, despite its functional relevance, has been little studied in individuals with autism spectrum disorders (ASDs). In the first study of its kind, 94 adolescents with an ASD and 55 without an ASD completed measures of everyday memory from the Rivermead Behavioural Memory Test (RBMT) and a standard word recall task (Children's Auditory Verbal Learning Test-2: CAVLT-2). The ASD group showed significant impairments on the RBMT, including in prospective memory, alongside impaired performance on the CAVLT-2. Social and communication ability was significantly associated with prospective remembering in an everyday memory context but not with the CAVLT-2. The complex nature of everyday memory and its relevance to ASD is discussed.

Reading and arithmetic in adolescents with autism spectrum disorders: peaks and dips in attainment.

In describing academic attainment in autism spectrum disorders (ASD), results are typically reported at the group mean level. This may mask subgroups of individuals for whom academic achievement is incommensurate with intellectual ability. The authors tested the IQ, literacy, and mathematical abilities of a large group (N = 100) of adolescents (14-16 years old) with ASD. Seventy-three percent of the sample had at least one area of literacy or mathematical achievement that was highly discrepant (approximately 14 standard score points) from full-scale IQ (FSIQ). The authors focused on four subgroups with either word reading ("Reading Peak" and "Reading Dip") or arithmetic ("Arithmetic Peak" and "Arithmetic Dip") higher or lower than FSIQ. These subgroups were largely mutually exclusive and were characterized by distinct intellectual profiles. The largest was the "Arithmetic Peak" subgroup of participants, who presented with average intellectual ability alongside superior arithmetic skills and who were predominantly in a mainstream educational setting. Overall, the most pervasive profile was discrepantly poor reading comprehension, which associated with severity of social and communication difficulties. The high rate of uneven academic attainment in ASD has implications for educational practice.

Auditory discrimination and auditory sensory behaviours in autism spectrum disorders.

It has been hypothesised that auditory processing may be enhanced in autism spectrum disorders (ASD). We tested auditory discrimination ability in 72 adolescents with ASD (39 childhood autism; 33 other ASD) and 57 IQ and age-matched controls, assessing their capacity for successful discrimination of the frequency, intensity and duration differences in pairs of sounds. At the group level, auditory discrimination ability did not differ between the adolescents with and without ASD. However, we found a subgroup of 20% of individuals in the ASD group who showed 'exceptional' frequency discrimination skills (defined as 1.65 SDs above the control mean) and who were characterised by average intellectual ability and delayed language onset. Auditory sensory behaviours (i.e. behaviours in response to auditory sensory input) are common in ASD and we hypothesised that these would relate to auditory discrimination ability. For the ASD group, poor performers on the intensity discrimination task reported more auditory sensory behaviours associated with coping with loudness levels. Conversely, those who performed well on the duration discrimination task reported more auditory sensory behaviours across the full range measured. Frequency discrimination ability did not associate with auditory sensory behaviours. We therefore conclude that (i) enhanced frequency discrimination is present in around 1 in 5 individuals with ASD and may represent a specific phenotype; and (ii) individual differences in auditory discrimination ability in ASD may influence the expression of auditory sensory behaviours by modulating the degree to which sounds are detected or missed in the environment.