Evaluating Long-Term Outcomes of Children Undergoing Surgical Treatment for Congenital Heart Disease for National Audit in England and Wales.

BACKGROUND: There is strong interest in the evaluation of longer-term outcome metrics for congenital heart diseases (CHDs); however, registries focus on postoperative metrics. METHODS AND RESULTS: Informed by user online discussion forums and scoping of national data, we selected sentinel CHDs and long-term outcome metrics suitable for routine monitoring. We then developed sentinel CHD phenotypes and algorithms for identifying treatment pathway procedures using clinical codes. Finally, we calculated the metrics within a retrospective national cohort analysis. The 9 selected sentinel CHDs had a higher-than-average prevalence, typically involved surgery in infancy, and were associated with an increased risk of late mortality. The selected metrics of survival and reinterventions at 1, 5, and 10 years were both important and feasible. The cohort included 29 319 (41.3% of all operated CHD births) English and Welsh children born with sentinel CHDs in 2000 to 2022. Example metrics at age 10 years included: survival-hypoplastic left heart syndrome: 57.6% (95% CI, 54.9%-60.4%), functionally univentricular heart: 86.7% (95% CI, 84.6%-88.9%), transposition of the great arteries: 93.1% (95% CI, 92.2%-93.9%), pulmonary atresia: 81.0% (95% CI, 79.1%-82.9%), atrioventricular septal defect: 88.5% (95% CI, 87.5%-89.5%), tetralogy of Fallot: 95.1% (95% CI, 94.4%-95.8%), aortic stenosis: 94.4% (95% CI, 93.3%-95.6%), coarctation: 96.7% (95% CI, 96.2%-97.3%), and ventricular septal defect: 96.9% 95% CI, (96.4%-97.3%); and (2) cumulative incidence of reintervention-hypoplastic left heart syndrome : 54.5% (95% CI, 51.5%-57.3%), functionally univentricular heart: 57.3% (95% CI, 53.9%-60.5%), transposition of the great arteries: 20.9% (95% CI, 19.5%-22.3%), pulmonary atresia: 66.8% (95% CI, 64.2%-69.1%), atrioventricular septal defect: 21.6% (20.3%-23.0%), tetralogy of Fallot: 26.6% (95% CI, 25.2%-28.0%), aortic stenosis: 31.2% (95% CI, 28.8%-33.6%), coarctation: 19.8% (95% CI, 18.6%-21.1%), and ventricular septal defect: 6.1% (95% CI, 5.5%-6.8%). CONCLUSIONS: It is feasible to report important long-term outcomes of survival and reintervention for sentinel CHDs using routinely collected procedure records, adding value to national audit.

Risk factors and early outcomes associated with prolonged pleural effusion/chylothorax after paediatric cardiac surgery.

OBJECTIVES: Prolonged pleural effusion/chylothorax (PPE/C) is a less investigated complication following paediatric cardiac surgery, and its true incidence, risk factors and impact on postoperative outcomes are not well described. We aim to address these gaps in knowledge using data from a prospective, multicentre study. METHODS: Data on 9 post-operative morbidities (unplanned reinterventions, extracorporeal life support, necrotising enterocolitis, PPE/C, renal replacement therapy, major adverse events, acute neurological events, feeding issues and postsurgical infection) were prospectively collected at 5 UK centres between 2015 and 2017, following paediatric cardiac surgery. Incidence of PPE/C, associations with procedure types, and risk factors were described. Mortality (30-day and 6-month) and hospital length of stay (HLoS) were compared between those with isolated PPE/C, single non-PPE/C morbidity, no morbidity, multimorbidity PPE/C and non-PPE/C multimorbidity. RESULTS: A total of 3090 procedures (2861 patients) were included (median age, 228 days). There were 202 PPE/C (incidence of 6.5%), occurring at a median of 6 days postoperatively (interquartile range: 3-10). PPE/C was associated with excess early mortality only when complicating scenarios where at least 2 other post-operative morbidities occurred. On average PPE/C is associated with 8 more HLoS days, but the relative impact is greatest when comparing isolated PPE/C with no morbidity (P < 0.001), whereas in multimorbidity scenarios, PPE/C does not significantly contribute to an increase of HLoS. CONCLUSIONS: Addition of PPE/C increases mortality but not HLoS in multimorbidity and HLoS only in single morbidity scenarios. This reinforces the important role of prevention, early detection and management of PPE/C in complex situations.

Retrospective Cohort Study of Additional Procedures and Transplant-Free Survival for Patients With Functionally Single Ventricle Disease Undergoing Staged Palliation in England and Wales.

BACKGROUND: Reinterventions may influence the outcomes of children with functionally single-ventricle (f-SV) congenital heart disease. METHODS AND RESULTS: We undertook a retrospective cohort study of children starting treatment for f-SV between 2000 and 2018 in England, using the national procedure registry. Patients were categorized based on whether they survived free of transplant beyond 1 year of age. Among patients who had transplant-free survival beyond 1 year of age, we explored the relationship between reinterventions in infancy and the outcomes of survival and Fontan completion, adjusting for complexity. Of 3307 patients with f-SV, 909 (27.5%), had no follow-up beyond 1 year of age, among whom 323 (35.3%) had ≥1 reinterventions in infancy. A total of 2398 (72.5%) patients with f-SV had transplant-free survival beyond 1 year of age, among whom 756 (31.5%) had ≥1 reinterventions in infancy. The 5-year transplant-free survival and cumulative incidence of Fontan, among those who survived infancy, were 93.4% (95% CI, 92.4%-94.4%) and 79.3% (95% CI, 77.4%-81.2%), respectively. Both survival and Fontan completion were similar for those with a single reintervention and those who had no reinterventions. Patients who had >1 additional surgery (adjusted hazard ratio, 3.93 [95% CI, 1.87-8.27] P<0.001) had higher adjusted risk of mortality. Patients who had >1 additional interventional catheter (adjusted subdistribution hazard ratio, 0.71 [95% CI, 0.52-0.96] P=0.03) had a lower likelihood of achieving Fontan. CONCLUSIONS: Among children with f-SV, the occurrence of >1 reintervention in the first year of life, especially surgical reinterventions, was associated with poorer prognosis later in childhood.

The Impact of Morbidities Following Pediatric Cardiac Surgery on Family Functioning and Parent Quality of Life.

We previously selected and defined nine important post-operative morbidities linked to paediatric cardiac surgery, and prospectively measured their incidence following 3090 consecutive operations. Our aim was to study the impact of these morbidities on family functioning and parental quality of life over 6 months in a subset of cases. As part of a prospective case matched study in five of the ten children's cardiac centers in the UK, we compared outcomes for parents of children who had a 'single morbidity', 'multiple morbidities', 'extracorporeal life support (ECLS)' or 'no morbidity'. Outcomes were evaluated using the PedsQL Family impact module (FIM) at 6 weeks and 6 months post-surgery. Outcomes were modelled using mixed effects regression, with adjustment for case mix and clustering within centers. We recruited 340 patients with morbidity (60% of eligible patients) and 326 with no morbidity over 21 months. In comparison to the reference group of 'no morbidity', after adjustment for case mix, at 6 weeks parent health-related quality of life (HRQoL) and total FIM sores were lower (worse) only for ECLS (p < 0.005), although a higher proportion of parents in both the ECLS and multi-morbidity groups had low/very low scores (p < .05). At 6 months, parent outcomes had improved for all groups but parent HRQoL and total score for ECLS remained lower than the 'no morbidity' group (p < .05) and a higher proportion of families had low or very low scores in the ECLS (70%) group (p < .01). Post-operative morbidities impact parent HRQoL and aspects of family functioning early after surgery, with this impact lessening by 6 months. Families of children who experience post-operative morbidities should be offered timely psychological support.

Long-term survival and center volume for functionally single-ventricle congenital heart disease in England and Wales.

OBJECTIVES: Long-term survival is an important metric for health care evaluation, especially in functionally single-ventricle (f-SV) congenital heart disease (CHD). This study's aim was to evaluate the relationship between center volume and long-term survival in f-SV CHD within the centralized health care service of England and Wales. METHODS: This was a retrospective cohort study of children born with f-SV CHD between 2000 and 2018, using the national CHD procedure registry, with survival ascertained in 2020. RESULTS: Of 56,039 patients, 3293 (5.9%) had f-SV CHD. Median age at first intervention was 7 days (interquartile range [IQR], 4, 27), and median follow-up time was 7.6 years (IQR, 1.0, 13.3). The largest diagnostic subcategories were hypoplastic left heart syndrome, 1276 (38.8%); tricuspid atresia, 440 (13.4%); and double-inlet left ventricle, 322 (9.8%). The survival rate at 1 year and 5 years was 76.8% (95% confidence interval [CI], 75.3%-78.2%) and 72.1% (95% CI, 70.6%-73.7%), respectively. The unadjusted hazard ratio for each 5 additional patients with f-SV starting treatment per center per year was 1.04 (95% CI, 1.02-1.06), P < .001. However, after adjustment for significant risk factors (diagnostic subcategory; antenatal diagnosis; younger age, low weight, acquired comorbidity, increased severity of illness at first procedure), the hazard ratio for f-SV center volume was 1.01 (95% CI, 0.99-1.04) P = .28. There was strong evidence that patients with more complex f-SV (hypoplastic left heart syndrome, Norwood pathway) were treated at centers with greater f-SV case volume (P < .001). CONCLUSIONS: After adjustment for case mix, there was no evidence that f-SV center volume was linked to longer-term survival in the centralized health service provided by the 10 children's cardiac centers in England and Wales.

Linkage of National Congenital Heart Disease Audit data to hospital, critical care and mortality national data sets to enable research focused on quality improvement.

OBJECTIVES: To link five national data sets (three registries, two administrative) and create longitudinal healthcare trajectories for patients with congenital heart disease (CHD), describing the quality and the summary statistics of the linked data set. DESIGN: Bespoke linkage of record-level patient identifiers across five national data sets. Generation of spells of care defined as periods of time-overlapping events across the data sets. SETTING: National Congenital Heart Disease Audit (NCHDA) procedures in public (National Health Service; NHS) hospitals in England and Wales, paediatric and adult intensive care data sets (Paediatric Intensive Care Audit Network; PICANet and the Case Mix Programme from the Intensive Care National Audit & Research Centre; ICNARC-CMP), administrative hospital episodes (hospital episode statistics; HES inpatient, outpatient, accident and emergency; A&E) and mortality registry data. PARTICIPANTS: Patients with any CHD procedure recorded in NCHDA between April 2000 and March 2017 from public hospitals. PRIMARY AND SECONDARY OUTCOME MEASURES: Primary: number of linked records, number of unique patients and number of generated spells of care. Secondary: quality and completeness of linkage. RESULTS: There were 143 862 records in NCHDA relating to 96 041 unique patients. We identified 65 797 linked PICANet patient admissions, 4664 linked ICNARC-CMP admissions and over 6 million linked HES episodes of care (1.1M inpatient, 4.7M outpatient). The linked data set had 4 908 153 spells of care after quality checks, with a median (IQR) of 3.4 (1.8-6.3) spells per patient-year. Where linkage was feasible (in terms of year and centre), 95.6% surgical procedure records were linked to a corresponding HES record, 93.9% paediatric (cardiac) surgery procedure records to a corresponding PICANet admission and 76.8% adult surgery procedure records to a corresponding ICNARC-CMP record. CONCLUSIONS: We successfully linked four national data sets to the core data set of all CHD procedures performed between 2000 and 2017. This will enable a much richer analysis of longitudinal patient journeys and outcomes. We hope that our detailed description of the linkage process will be useful to others looking to link national data sets to address important research priorities.

Cohort study of intervened functionally univentricular heart in England and Wales (2000-2018).

OBJECTIVE: Given the paucity of long-term outcome data for complex congenital heart disease (CHD), we aimed to describe the treatment pathways and survival for patients who started interventions for functionally univentricular heart (FUH) conditions, excluding hypoplastic left heart syndrome. METHODS: We performed a retrospective cohort study using all procedure records from the National Congenital Heart Diseases Audit for children born in 2000-2018. The primary outcome was mortality, ascertained from the Office for National Statistics in 2020. RESULTS: Of 53 615 patients, 1557 had FUH: 55.9% were boys and 67.4% were of White ethnic groups. The largest diagnostic categories were tricuspid atresia (28.9%), double inlet left ventricle (21.0%) and unbalanced atrioventricular septal defect (AVSD) (15.2%). The ages at staged surgery were: initial palliation 11.5 (IQR 5.5-43.5) days, cavopulmonary shunt 9.2 (IQR 6.0-17.1) months and Fontan 56.2 (IQR 45.5-70.3) months. The median follow-up time was 10.8 (IQR 7.0-14.9) years and the 1, 5 and 10-year survival rates after initial palliation were 83.6% (95% CI 81.7% to 85.4%), 79.4% (95% CI 77.3% to 81.4%) and 77.2% (95% CI 75.0% to 79.2%), respectively. Higher hazards were present for unbalanced AVSD HR 2.75 (95% CI 1.82 to 4.17), atrial isomerism HR 1.75 (95% CI 1.14 to 2.70) and low weight HR 1.65 (95% CI 1.13 to 2.41), critical illness HR 2.30 (95% CI 1.67 to 3.18) or acquired comorbidities HR 2.71 (95% CI 1.82 to 4.04) at initial palliation. CONCLUSION: Although treatment pathways for FUH are complex and variable, nearly 8 out of 10 children survived to 10 years. Longer-term analyses of outcome based on diagnosis (rather than procedure) can inform parents, patients and clinicians, driving practice improvements for complex CHD.

Factors associated with unplanned reinterventions and their relation to early mortality after pediatric cardiac surgery.

OBJECTIVE: Unplanned reintervention (uRE) is used as an indicator of patient morbidity and quality of care in pediatric cardiac surgery. We investigated associated factors and early mortality after uREs. METHODS: Morbidity data were prospectively collected in 5 UK centers between 2015 and 2017; uRE included surgical cardiac, interventional transcatheter cardiac, permanent pacemaker, and diaphragm plication procedures. Mortality (30-day and 6-month) in uRE/no-uRE patients was reported before and after matching. Predicted 30-day mortality was calculated using the Partial Risk Adjustment in Surgery score. RESULTS: A total of 3090 procedures (2861 patients) were included (median age, 228 days). There were 146 uREs, resulting in an uRE rate of 4.7%. Partial Risk Adjustment in Surgery score, 30-day mortality and 6-month mortality in uRE and no-uRE groups were 2.4% versus 1.3%, 8.9% versus 1%, and 17.1% versus 2.4%, respectively. After matching, mortality at 6 months remained higher in uRE compared with no-uRE (12.2% vs 1.4%; P = .02; 74 pairs). In the uRE group, 21 out of 25 deaths at 6 months occurred when at least 1 additional postoperative complication was present. In multivariable analysis, neonatal age (P = .002), low weight (P = .009), univentricular heart (P < .001), and arterial shunt (P < .001) were associated with increased risk of uRE, but Partial Risk Adjustment in Surgery score was not (only in univariable analysis). CONCLUSIONS: uREs are a relatively frequent complication after pediatric cardiac surgery and are associated with some patient characteristics, but not the Partial Risk Adjustment in Surgery risk score. Early mortality was higher after uRE, independent of preoperative factors, but linked to other postoperative complications.

Morbidities After Cardiac Surgery: Impact on Children's Quality of Life and Parents' Mental Health.

BACKGROUND: Most children now survive cardiac surgery, and the focus of quality improvement initiatives has shifted toward more complex outcome measures. The aim of this investigation was to study the impact of early postoperative morbidities on parent-reported patient quality of life and parental anxiety or depression over 6 months. METHODS: This prospective case-matched cohort study was conducted in 5 UK children's cardiac centers. Measures of impact for patient categories of "single morbidity," "multiple morbidities," and "extracorporeal life support (ECLS)" were compared with "no morbidity." The measures used were the Pediatric Quality of Life Inventory (PedsQL) and the 4-item Patient Health Questionnaire (PHQ-4) at 6 weeks and 6 months postoperatively. The study modeled the outcomes using mixed effects regression, adjusting for case mix and clustering within centers. RESULTS: The study included 666 patients who underwent operation at a median age of 81 days (interquartile range, 10 to 325 days). At 6-week follow-up, significant adjusted differences to the reference group with no morbidity were found for total PedsQL scores, which were lower in patients with ECLS (P = .01), multiple morbidities (P < .001), and a single morbidity (P = .04), as well as the proportion of parents with anxiety and depression, which were higher in the group with multiple morbidities (P = .04 and P = .01, respectively). At 6 months, measures had improved in all morbidity groups. The only significant adjusted difference in the reference group was for physical PedsQL scores in ECLS (P = .04) and multiple morbidities (P < .01). CONCLUSIONS: Patient and parent well-being are strongly influenced by postoperative morbidities early after surgery, with improvement by 6 months. Family psychological support and holistic rehabilitation are vital for children who experience postoperative morbidities.

Costs of postoperative morbidity following paediatric cardiac surgery: observational study.

OBJECTIVE: Early mortality rates for paediatric cardiac surgery have fallen due to advancements in care. Alternative indicators of care quality are needed. Postoperative morbidities are of particular interest. However, while health impacts have been reported, associated costs are unknown. Our objective was to calculate the costs of postoperative morbidities following paediatric cardiac surgery. DESIGN: Two methods of data collection were integrated into the main study: (1) case-matched cohort study of children with and without predetermined morbidities; (2) incidence rates of morbidity, measured prospectively. SETTING: Five specialist paediatric cardiac surgery centres, accounting for half of UK patients. PATIENTS: Cohort study included 666 children (340 with morbidities). Incidence rates were measured in 3090 consecutive procedures. METHODS: Risk-adjusted regression modelling to determine marginal effects of morbidities on per-patient costs. Calculation of costs for hospital providers according to incidence rates. Extrapolation using mandatory audit data to report annual financial burden for the health service. OUTCOME MEASURES: Impact of postoperative morbidities on per-patient costs, hospital costs and UK health service costs. RESULTS: Seven of the 10 morbidity categories resulted in significant costs, with mean (95% CI) additional costs ranging from £7483 (£3-£17 289) to £66 784 (£40 609-£103 539) per patient. On average all morbidities combined increased hospital costs by 22.3%. Total burden to the UK health service exceeded £21 million each year. CONCLUSION: Postoperative morbidities are associated with a significant financial burden. Our findings could aid clinical teams and hospital providers to account for costs and contextualise quality improvement initiatives.

Parental understanding of our communication of morbidity associated with paediatric cardiac surgery: a qualitative study.

BACKGROUND: Following paediatric cardiac surgery, quality of life may be significantly impacted by morbidities associated with cardiac surgery. Parental understanding of the potential for postoperative morbidity is important for informed decision making. As part of a broader research study, we aimed to elicit parental understanding and experience of the communication of morbidities following their child's cardiac surgery, using traditional focus groups together with an online forum. METHODS: The Children's Heart Federation set up and moderated a closed, anonymous online discussion group via their Facebook page, focusing on complications, information needs and methods of providing families with information. Additionally, we ran three focus groups with parents/carers, moderated by an experienced independent professional. Focus groups were recorded and transcribed and a single transcript was generated from the online forum. All transcripts were thematically analysed. RESULTS: All data were collected in 2014. The forum ran over 3 months in 2014 and involved 72 participants. Focus groups involved 13 participants. Three broad themes were identified: (1) clinicians' use of language, (2) feeling unprepared for complications and (3) information needs of families. CONCLUSIONS: Clinicians' language is often misunderstood, with wide variability in the way morbidities are described, and between differing teams looking after the same child. Information may not be easily absorbed or retained by families, who often felt unprepared for morbidities that arose after their child's heart surgery. Here, we propose key principles of good communication tailored to the individual receiving it.

A tool for routine monitoring and feedback of morbidities following paediatric cardiac surgery.

Short-term survival after paediatric cardiac surgery has improved significantly over the past 20 years and increasing attention is being given to measuring and reducing incidence of morbidities following surgery. How to best use routinely collected data to share morbidity information constitutes a challenge for clinical teams interested in analysing their outcomes for quality improvement. We aimed to develop a tool facilitating this process in the context of monitoring morbidities following paediatric cardiac surgery, as part of a prospective multi-centre research study in the United Kingdom.We developed a prototype software tool to analyse and present data about morbidities associated with cardiac surgery in children. We used an iterative process, involving engagement with potential users, tool design and implementation, and feedback collection. Graphical data displays were based on the use of icons and graphs designed in collaboration with clinicians.Our tool enables automatic creation of graphical summaries, displayed as a Microsoft PowerPoint presentation, from a spreadsheet containing patient-level data about specified cardiac surgery morbidities. Data summaries include numbers/percentages of cases with morbidities reported, co-occurrences of different morbidities, and time series of each complication over a time window.Our work was characterised by a very high level of interaction with potential users of the tool, enabling us to promptly account for feedback and suggestions from clinicians and data managers. The United Kingdom centres involved in the project received the tool positively, and several expressed their interest in using it as part of their routine practice.

What are the important morbidities associated with paediatric cardiac surgery? A mixed methods study.

OBJECTIVES: Given the current excellent early mortality rates for paediatric cardiac surgery, stakeholders believe that this important safety outcome should be supplemented by a wider range of measures. Our objectives were to prospectively measure the incidence of morbidities following paediatric cardiac surgery and to evaluate their clinical and health-economic impact over 6 months. DESIGN: The design was a prospective, multicentre, multidisciplinary mixed methods study. SETTING: The setting was 5 of the 10 paediatric cardiac surgery centres in the UK with 21 months recruitment. PARTICIPANTS: Included were 3090 paediatric cardiac surgeries, of which 666 patients were recruited to an impact substudy. RESULTS: Families and clinicians prioritised:Acute neurological event, unplanned re-intervention, feeding problems, renal replacement therapy, major adverse events, extracorporeal life support, necrotising enterocolitis, postsurgical infection and prolonged pleural effusion or chylothorax.Among 3090 consecutive surgeries, there were 675 (21.8%) with at least one of these morbidities. Independent risk factors for morbidity included neonatal age, complex heart disease and prolonged cardiopulmonary bypass (p<0.001). Among patients with morbidity, 6-month survival was 88.2% (95% CI 85.4 to 90.6) compared with 99.3% (95% CI 98.9 to 99.6) with none of the morbidities (p<0.001). The impact substudy in 340 children with morbidity and 326 control children with no morbidity indicated that morbidity-related impairment in quality of life improved between 6 weeks and 6 months. When compared with children with no morbidities, those with morbidity experienced a median of 13 (95% CI 10.2 to 15.8, p<0.001) fewer days at home by 6 months, and an adjusted incremental cost of £21 292 (95% CI £17 694 to £32 423, p<0.001). CONCLUSIONS: Evaluation of postoperative morbidity is more complicated than measuring early mortality. However, tracking morbidity after paediatric cardiac surgery over 6 months offers stakeholders important data that are of value to parents and will be useful in driving future quality improvement.

Incidence and risk factors for important early morbidities associated with pediatric cardiac surgery in a UK population.

OBJECTIVE: Given excellent 30-day survival for pediatric cardiac surgery, other outcome measures are important. We aimed to study important early postoperative morbidities selected by stakeholders following a rigorous and evidenced-based process, with a view to identifying potential risk factors. METHODS: The incidence of selected morbidities was prospectively measured for 3090 consecutive pediatric cardiac surgical admissions in 5 UK centers between October 2015 and June 2017. The relationship between the candidate risk factors and the incidence of morbidities was explored using multiple regressions. Patient survival, a secondary outcome, was checked at 6 months. RESULTS: A total of 675 (21.8%) procedure episodes led to at least 1 of the following: acute neurologic event, unplanned reoperation, feeding problems, renal replacement therapy, major adverse events, extracorporeal life support, necrotizing enterocolitis, surgical infection, or prolonged pleural effusion. The highest adjusted odds ratio of morbidity was in neonates compared with children, 5.26 (95% confidence interval, 3.90-7.06), and complex heart diseases (eg, hypoplastic left heart), 2.14 (95% confidence interval, 1.41-3.24) compared with low complexity (eg, atrial septal defect, P < .001 for all). Patients with any selected morbidity had a 6-month survival of 88.2% (95% confidence interval, 85.4-90.6) compared with 99.3% (95% confidence interval, 98.9-99.6) with no defined morbidity (P < .001). CONCLUSIONS: Evaluation of postoperative morbidity provides important information over and above 30-day survival and should become a focus for audit and quality improvement. Our results have been used to initiate UK-based audit for 5 of these 9 morbidities, co-develop software for local monitoring of these morbidities, and parent information about these morbidities.

Exploring communication between parents and clinical teams following children's heart surgery: a survey in the UK.

OBJECTIVE: To explore communication between clinicians and families of children undergoing heart surgery. DESIGN: This study was part of a larger study to select, define and measure the incidence of postoperative complications in children undergoing heart surgery. Parents of children recruited to a substudy between October 2015 and December 2017 were asked to complete a questionnaire about communication during their child's inpatient stay. We explored all responses and then disaggregated by the following patient characteristics: presence of a complication, length of stay, hospital site, ethnicity and child's age. This was a descriptive study only. SETTING: Four UK specialist hospitals. RESULTS: We recruited 585 children to the substudy with 385 responses (response rate 66%).81% of parents reported that new members of staff always introduced themselves (18% sometimes, 1% no). Almost all parents said they were encouraged to be involved in decision-making, but often only to some extent (59% 'yes, definitely'; 37% 'to some extent'). Almost two-thirds of parents said they were told different things by different people which left them feeling confused (10% 'a lot'; 53% 'sometimes'). Two-thirds (66%) reported that staff were definitely aware of their child's medical history (31% 'to some extent'). 90% said the operation was definitely explained to them (9% 'to some extent') and 79% that they were definitely told what to do if they were worried after discharge (17% 'to some extent').Parents of children with a complication tended to give less positive responses for involvement in decision-making, consistent communication and staff awareness of their child's medical history. Parents whose children had longer stays in hospital tended to report lower levels of consistent communication and involvement in decision-making. CONCLUSIONS: Our results emphasise the need for consistent communication with families, particularly where complications arise or for children who have longer stays in the hospital.

Interventional treatments and risk factors in patients born with hypoplastic left heart syndrome in England and Wales from 2000 to 2015.

OBJECTIVE: To describe the long-term outcomes, treatment pathways and risk factors for patients diagnosed with hypoplastic left heart syndrome (HLHS) in England and Wales. METHODS: The UK's national audit database captures every procedure undertaken for congenital heart disease and updated life status for resident patients in England and Wales. Patients with HLHS born between 2000 and 2015 were identified using codes from the International Paediatric and Congenital Cardiac Code. RESULTS: There were 976 patients with HLHS. Of these, 9.6% had a prepathway intervention, 89.5% underwent a traditional pathway of staged palliation and 6.4% of infants underwent a hybrid pathway. Patients undergoing prepathway procedures or the hybrid pathway were more complex, exhibiting higher rates of prematurity and acquired comorbidity. Prepathway intervention was associated with the highest in-hospital mortality (34.0%).44.6% of patients had an off-pathway procedure after their primary procedure, most frequently stenting or dilation of residual or recoarctation and most commonly occurring between stage 1 and stage 2.The survival rate at 1 year and 5 years was 60.7% (95% CI 57.5 to 63.7) and 56.3% (95% CI 53.0 to 59.5), respectively. Patients with an antenatal diagnosis (multivariable HR (MHR) 1.63 (95% CI 1.12 to 2.38)), low weight (<2.5 kg) (MHR 1.49 (95% CI 1.05 to 2.11)) or the presence of an acquired comorbidity (MHR 2.04 (95% CI 1.30 to 3.19)) were less likely to survive. CONCLUSION: Treatment pathways among patients with HLHS are complex and variable. It is essential that the long-term outcomes of conditions like HLHS that require serial interventions are studied to provide a fuller picture and to inform quality assurance and improvement.

Selection by a panel of clinicians and family representatives of important early morbidities associated with paediatric cardiac surgery suitable for routine monitoring using the nominal group technique and a robust voting process.

OBJECTIVE: With survival following paediatric cardiac surgery improving, the attention of quality assurance and improvement initiatives is shifting to long-term outcomes and early surgical morbidities. We wanted to involve family representatives and a range of clinicians in selecting the morbidities to be measured in a major UK study. SETTING: Paediatric cardiac surgery services in the UK. PARTICIPANTS: We convened a panel comprising family representatives, paediatricians from referring centres, and surgeons and other clinicians from surgical centres. PRIMARY AND SECONDARY OUTCOME MEASURES: Using the nominal group technique augmented by a robust voting process to identify group preferences, suggestions for candidate morbidities were elicited, discussed, ranked and then shortlisted. The shortlist was passed to a clinical group that provided a view on the feasibility of monitoring each shortlisted morbidity in routine practice. The panel then met again to select a prioritised list of morbidities for further study, with the list finalised by the clinical group and chief investigators. RESULTS: At the first panel meeting, 66 initial suggestions were made, with this reduced to a shortlist of 24 after two rounds of discussion, consolidation and voting. At the second meeting, this shortlist was reduced to 10 candidate morbidities. Two were dropped on grounds of feasibility and replaced by another the panel considered important. The final list of nine morbidities included indicators of organ damage, acute events and feeding problems. Family representatives and clinicians from outside tertiary centres brought some issues to greater prominence than if the panel had consisted solely of tertiary clinicians or study investigators. CONCLUSION: The inclusion of patient and family perspectives in identifying metrics for use in monitoring a specialised clinical service is challenging but feasible and can broaden notions of quality and how to measure it.

Understanding Children's Heart Surgery Data: A Cross-Disciplinary Approach to Codevelop a Website.

Risk-adjusted survival statistics after children's heart surgery are published annually in the United Kingdom. Interpreting these statistics is difficult, and better resources about how to interpret survival data are needed. Here we describe how a multidisciplinary team of mathematicians, psychologists, and a charity worked with parents of heart surgery children and other users to codevelop online resources to present survival outcomes. Early and ongoing involvement of users was crucial and considerably changed the content, scope, and look of the website, and the formal psychology experiments provided deeper insight. The website http://childrensheartsurgery.info/ was launched in June 2016 to very positive reviews.