This study explored the prospective use of the Ages and Stages Questionnaires-3 in follow-up after cardiac surgery.

For children undergoing cardiac surgery at 5 United Kingdom centres, the Ages and Stages Questionnaires-3 were administered 6 months and 2 years later, with an outcome based on pre-defined cut-points: Red = 1 or more domain scores >2 standard deviations below the normative mean, Amber = 1 or more domain scores 1–2 standard deviations below the normal range based on the manual, Green = scores within the normal range based on the manual.

From a cohort of 554 children <60 months old at surgery, 306 participated in the postoperative assessment: 117 (38.3%) were scored as Green, 57 (18.6%) as Amber, and 132 (43.1%) as Red. Children aged 6 months at first assessment (neonatal surgery) were likely to score Red (113/124, 85.6%) compared to older age groups (n = 32/182, 17.6%). Considering risk factors of congenital heart complexity, univentricular status, congenital comorbidity, and child age in a logistic regression model for the outcome of Ages and Stages score Red, only younger age was significant (p < 0.001). 87 children had surgery in infancy and were reassessed as toddlers. Of these, 43 (49.2%) improved, 30 (34.5%) stayed the same, and 13 (16.1%) worsened. Improved scores were predominantly in those who had a first assessment at 6 months old.

The Ages and Stages Questionnaires results are most challenging to interpret in young babies of 6 months old who are affected by complex CHD.

An early warning tool, the Congenital Heart Assessment Tool (CHAT), was designed in 2012 to support parental preparation before discharge, enhancing understanding of their infant’s complex CHD, the signs of deterioration to look out for and to support decision-making at home. Acceptability and feasibility of the tool were tested during 2013–2015 in a single centre.

To evaluate the wider implementation across four children’s cardiac centres of the CHAT for infants with complex CHD in the community setting.

A four-centre collaborative mixed-methods quality improvement project funded by The Health Foundation, during 2016–2018. A plan, do, study, act cycle of improvement was employed. This article reports on the planning phases, creation of a modified tool (CHATm); and the implementation phases, including retrospective case note review using CHATm; tabletop simulation exercise using CHATm and clinical implementation of CHATm.

Key findings included the benefit of using CHATm simulation for practitioners; the effectiveness of CHATm in predicting amber and red triggers, indicating parental actions and escalation of concerns to professionals. Parents using CHATm found it enhanced knowledge in preparation for going home, supported decision-making, and discussions with health care professionals.

Using the CHATm clinically identified the need to develop a structured model of assessment of parental suitability for home-monitoring programmes. A robust and nationally agreed training programme for all staff using CHATm was recommended to ensure successful and complete implementation in practice.

CHD was the most prevalent congenital anomaly (60.9 per 10,000, 95% CI 59.0–62.8) in England in 2018, with 1767 babies born with severe cardiac defects. The 30-day survival rates for complex procedures continue to improve; however despite care advances, the early post-operative period and first year of life remain a critical time for these infants. The Congenital Heart Assessment Tool was developed to support parental decision-making, standardise care provision, improve communication, and the safety and quality of care.

To further evaluate the Congenital Heart Assessment Tool.

A four centre collaborative mixed-methods quality improvement project funded by The Health Foundation, involving eight phases conducted during 2016–2018. Phases six to eight (clinical simulation exercise, parent workshop, and updated tool) are reported in this paper.

Four themes emerged from the clinical simulation exercise (phase six) including: improving documentation; preparation of parents; preparation of health care professionals; and communication. One main theme emerged from the parent workshop (phase seven): “what parents know versus what professionals know [about CHD]”.

These phases further validated the effectiveness of the CHATm in terms of triggering amber and red indicators and demonstrated parents’ ability to identify deterioration in their infant’s clinical condition. Recommendations arising from the quality improvement project enabled the project team to create an updated version of the Congenital Heart Assessment Tool, CHAT2.

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.

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.

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.

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.

Neurodevelopmental abnormalities are common in children with CHD and are the highest-priority concerns for parents and professionals following cardiac surgery in childhood. There is no additional routine monitoring of development for children with CHD in the United Kingdom; hence, neurodevelopmental concerns may be detected late, precluding early referral and intervention.

An early recognition tool – the “Brief Developmental Assessment” – was developed using quality improvement methodology involving several iterations and rounds of pilot testing. Our requirements were for a tool covering important developmental domains and practicable for use within inpatient and outpatient settings by paediatric cardiac health professionals who are non-developmental specialists, without specialised equipment and which involved direct observation, as well as parental report.

Items were included in the tool based on existing developmental measures, covering the domains of gross and fine motor skills, daily living skills, communication, socialisation, and general understanding. Items were developed for five age bands – 0–16 weeks, 17–34 weeks, 35–60 weeks, 15 months–2.9 years, and 3–4.9 years – and the final versions included a traffic light scoring system for identifying children with possible delay in any or all domains. Preliminary testing indicated excellent inter-rater reliability, an ability to detect children with a diagnosis known to be associated with developmental delay, and largely acceptable internal reliability.

We report the evolution and preliminary testing of an early recognition tool for assessing the development of children with heart disease; this was encouraging and sufficiently good to support further validation in a larger study.

Morbidity is defined as a state of being unhealthy or of experiencing an aspect of health that is “generally bad for you”, and postoperative morbidity linked to paediatric cardiac surgery encompasses a range of conditions that may impact the patient and are potential targets for quality assurance.

As part of a wider study, a multi-disciplinary group of professionals aimed to define a list of morbidities linked to paediatric cardiac surgery that was prioritised by a panel reflecting the views of both professionals from a range of disciplines and settings as well as parents and patients.

We present a set of definitions of morbidity for use in routine audit after paediatric cardiac surgery. These morbidities are ranked in priority order as acute neurological event, unplanned re-operation, feeding problems, the need for renal support, major adverse cardiac events or never events, extracorporeal life support, necrotising enterocolitis, surgical site of blood stream infection, and prolonged pleural effusion or chylothorax. It is recognised that more than one such morbidity may arise in the same patient and these are referred to as multiple morbidities, except in the case of extracorporeal life support, which is a stand-alone constellation of morbidity.

It is feasible to define a range of paediatric cardiac surgical morbidities for use in routine audit that reflects the priorities of both professionals and parents. The impact of these morbidities on the patient and family will be explored prospectively as part of a wider ongoing, multi-centre study.

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.

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.

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.

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.

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.

To demonstrate the generalisability of the Pediatric Cardiac Quality of Life Inventory in the United Kingdom.

Children and adolescents with heart disease were recruited from three tertiary paediatric cardiac centres in the United Kingdom and completed the Pediatric Cardiac Quality of Life Inventory. Item response option variability, total and subscale scores, patterns of correlation, and internal consistency were compared between the three sites.

A total of 1537 participants – 768 children/adolescents and 769 parents – were evaluated from the three sites. Patterns of item response option variability were similar and acceptable for all samples – child, adolescent, parent of child, and parent of adolescent. Internal consistency was high (0.82–0.96) for all samples from each site, and item–subscale, subscale–subscale, subscale–total, and item–total correlations were moderate to excellent for each centre. Comparisons of patterns of subscale and total score correlations between the three sites revealed no significant differences.

Scores on the Pediatric Cardiac Quality of Life Inventory are generalisable in the United Kingdom, supporting the use of this measure for multi-centre studies of health-related quality of life of children and adolescents with heart disease.

Patients with repaired tetralogy of Fallot have good long-term survival but less is known about the subjectively assessed quality of life or objectively measured functional status of those who have not required subsequent pulmonary valve replacement. We assessed these parameters in a group of children and adults free from pulmonary valve replacement after tetralogy of Fallot repair.

A random sample of 50 subjects – 16 children and 34 adults, aged 4.1–56.7 years – who had undergone tetralogy of Fallot repair and were free from subsequent pulmonary valve replacement underwent cardiopulmonary exercise testing and completed standardised questionnaires assessing health-related quality of life and resilience. Patients were generally asymptomatic (median New York Heart Association class = I). Exercise capacity was within two standard deviations of normal for most children and adults (mean z VO2max: 0.20 ± 1.5; mean z VE/VCO2: −0.9 ± 1.3). Children reported a total health-related quality of life score similar to healthy norms (78 ± 10 versus 84 ± 1, p = 0.73). Adult survivors also reported quality of life scores comparable to healthy norms. Resilience was highly correlated with all domains of health-related quality of life (r = 0.713, p < 0.0001).

Patients who have undergone tetralogy of Fallot repair in childhood and have not required pulmonary valve replacement have a good long-term health-related quality of life. The finding that patients with greater resilience had better health-related quality of life suggests that it may be beneficial to implement interventions to foster resilience.

The provision of emotional and psychological support for all family members who need it is an essential element of holistic palliative care. Within East Anglia's Children's Hospice, teams of professionally trained and experienced workers offer psychosocial support to all family members at all times during the child's and family's journey. However, the effectiveness and appropriateness of current psychosocial provision is unclear, as is the requirement for any additional psychological services.

The purpose of this study was to elicit perceptions about current psychological support within the hospice from a group of stakeholders (parents, hospice staff, and external professionals).

Forty-five parents participated in family focus groups, telephone interviews, individual interviews in their home, or a web-based survey. Ninety-five hospice staff (including nurses, carers, play specialists, therapists, and family support practitioners) and 28 external staff (including physicians, nurses, and commissioning managers) were seen using a mixture of focus group and individual meetings. Focus groups and meetings were held at the hospice building or at an external venue. Interviews were recorded and transcribed verbatim and analyzed using thematic coding.

Two main themes addressing perceptions of current psychological provision emerged: “understanding psychological support” and “unmet psychological need.” Subthemes linked to support included choice, staff roles and labels, communication, and flexibility, whereas the themes within unmet need had a stronger focus on people and problems.

Understanding different user perspectives is an important first step in enhancing current psychological provision; operationalizing the findings will be challenging.

To anglicise an American – that is, English language – disease-specific health-related quality of life measure, using the Paediatric Cardiac Quality of Life Inventory, for children in the age group of 8–12 years and adolescents in the age group of 13–18 years with cardiac disease, and to assess conceptual equivalence of the American and British versions.

A process of forward and backward translation of the measure was undertaken before focus groups and individual interviews with 40 participants – that is, 20 children/adolescents with cardiac disease and 20 parents of children/adolescents with cardiac disease – to determine their understanding of the meaning of the questions.

Interviews established that participants understood the meaning of the questions, although some found it difficult to explain the meaning of questions in which the language was explicit and wanted instead to answer the individual questions as they applied to them/their child. There was agreement that all versions of the questionnaire were relevant and comprehensive, and that the length of the questionnaires was acceptable and practical.

The anglicised version of the Paediatric Cardiac Quality of Life Inventory appears to be a linguistically valid measure of health-related quality of life for children and adolescents with cardiac disease. The psychometric properties of the anglicised Paediatric Cardiac Quality of Life Inventory are now being tested in a multi-centre study in the United Kingdom.

With improvements in the surgical and medical management of children with congenital heart disease, the majority are now expected to transition into adult services. Whilst the importance of successful transition is increasingly acknowledged, previous research has identified a number of areas of concern. We therefore wished to elicit the views of young people themselves concerning their requirements from a specialist cardiac service.

Using an exploratory approach our aims were to increase awareness of the service needs and expectations of young people, identify good practice and areas where communication processes and facilities could be improved, and enhance understanding of transition between paediatric and adult services. Postal questionnaires were sent to 63 young people with congenital cardiac disease aged 17 to 20 years. Open ended questions asked for views and experiences about facilities in the paediatric and adult areas, perceptions of transition between services and suggestions for improvements. Qualitative data were transferred verbatim into electronic format to facilitate coding and theming.

Thirty-eight (60.3 percent) completed questionnaires were received. A number of themes, including communication, care processes/caring interactions and environment, emerged regarding areas of satisfaction and dissatisfaction within the paediatric and adult services, together with areas for improvement and important aspects of transition.

Young people’s views need to be taken into account at all stages of the patient journey. They want an individualised approach to their care which recognises that they have specific emotional and practical needs. Such findings need to be incorporated into an ongoing model of care.