Sonya Crowe

BACKGROUND: Pulmonary atresia with intact ventricular septum management has evolved, with increased use of transcatheter right ventricular decompression, but the impact on contemporary outcomes remains unknown. This study evaluates contemporary national outcomes in patients aged 0 to 18 years. METHODS: Patients with pulmonary atresia with intact ventricular septum undergoing cardiac intervention (2000–2022) in England and Wales were linked to multiple national data sets. Mortality (early and late), reintervention, and hospital stay were described overall and by treatment pathway. RESULTS: Among 488 included patients with pulmonary atresia with intact ventricular septum, 58.8% underwent right ventricular decompression as the first procedure, predominantly transcatheter (84.8%), while 32.4% received stage 1 palliation (arteriopulmonary shunt or ductal stenting), and 8.8% died or were lost to follow-up before either intervention. Overall, 54.5% achieved biventricular (BiV) repair, 26.7% underwent functionally single-ventricle palliation, and 18.8% were noncommitted. Overall group in-hospital mortality was 11.7%. The median follow-up was 8.9 years, with 1- and 5-year mortality of 19.8% (95% CI, 16.2%–23.2%) and 20.8% (95% CI, 17.1%–24.4%), respectively. Stage 1 palliation as the initial therapy had higher 1-year mortality than right ventricular decompression (37.2% [29.0%–44.5%] versus 7.7% [4.5%–10.7%]). Cardiac reintervention was 62.6% (56.1%–68.4%) at 10 years after BiV repair and 32.8% (21.5%–44.4%) at 10 years after palliative stage 3. Median hospital stay was greatest in the first year (35 days), declining to 2 days annually by 10 years, with functionally single-ventricle patients requiring more days in hospital than BiV patients (52 versus 28). CONCLUSIONS: In a contemporary pulmonary atresia with intact ventricular septum cohort, transcatheter right ventricular decompression was the predominant initial intervention. This approach had lower in-hospital mortality compared with older multicenter series, with BiV repair achieved in more than half of the patients. Early mortality after nonantegrade flow augmentation remains high, likely due to nonmodifiable risk factors in severe cases. Long-term survival and hospital stay remain better for those amenable to BiV circulation, reflecting more favorable anatomy compared with those on the single-ventricle pathway, although reintervention is lower in the latter.

Objectives: We wanted, as part of a wider study, to elicit perspectives of patients with congenital heart disease (CHD) and/or their parents/carers about which outcomes of CHD and/or cardiac surgery matter and what should be measured and reported. Design: Qualitative, closed, asynchronous, online discussion forums underpinned by an interpretivist framework. Setting and participants: Three patient charities in the UK set up and moderated separate online forums for adult patients with CHD/carers, adolescent patients with CHD and parents/carers of children and young people with CHD. Analysis: Thematic analysis using a codebook approach. Results: There were five forums in total; 343 participants signed up, two-thirds of whom were adult patients (n=235; 69%). Four themes reflecting different types of outcomes were identified: clinical outcomes related to surgery (eg, diagnosis-based survival, complications, readmission and reintervention rates); other clinical outcomes (eg, long-term survival, use of medications, pregnancy risks and outcomes); outcomes related to service provision (eg, availability of support services, cancellations, delayed follow-up); and patient-reported outcomes (eg, quality of life, mental health outcomes). Conclusions: Participants identified a range of outcomes about which they wanted more information but within the context of an individualised approach to how much, when and what information is provided. The importance of achieving a balance between knowing so much that it has a detrimental impact on mental health and not knowing enough so that patients/carers are unable to make informed decisions was emphasised.

1. Implementation is the process through which proposed changes are adopted in real-life settings. In the domain of operational research (OR), analysts use systematic analysis, model building, and ...

IntroductionData linkage studies generate opportunities to answer important health questions, but are also notoriously time consuming, with delays accessing data lasting years. Previously we described our experience of accessing and linking five national datasets for our study 'LAUNCHES QI'. However, the delays and challenges we encountered did not end once data were received - maintaining access and research amendments also required significant time and resources. ObjectiveTo outline the costs, time, and workload associated with LAUNCHES, including set-up, annual requirements, and amendments. We also describe the experience of re-using the linked dataset for a second study, CHAMPION, which we wrongly assumed would be simpler. We highlight new delays and challenges, offer advice to researchers, and suggest improvements to the system. ResultsLAUNCHES set-up involved 39 documents and data access took 2.5 years. Subsequent amendments/renewals added 75 more documents. Repurposing the data for CHAMPION required restarting the entire process from scratch. It took nearly two years to gain initial approvals and another 18 months to receive the datasets, with 44 documents submitted for set-up and 92 more for amendments and annual processes. New challenges and delays included changes of data controller, data extract errors, and the repetitive amendment process. ConclusionsWith growing recognition of the challenges in using health data for research and our own increasing experience navigating the system, we had hoped for positive change. Instead, there are more forms, and processes have become harder, not easier. We believe simple changes could streamline the system, yet with each new study, the document set grows, and the forms get longer. Research with linked datasets is incredibly painful, and the system discourages amendments, stifling innovation. Whilst we've realised the value of linked data, delays to research are significant, and researchers may give up without simpler processes to link and update data.

Aim: To understand whether the risk of complications following paediatric cardiac surgery differs according to a child’s ethnicity or the degree of residential deprivation. Methods: We conducted a retrospective cohort study using data from the National Congenital Heart Disease Audit, including children younger than 18 years who underwent cardiac surgery between April 2015 and March 2022 across 10 paediatric cardiac surgical centres in England and Wales. We examined the occurrence of six defined postoperative complications and used previously reported descriptive models to account for case complexity. Multivariable analysis was used to assess the association between complications and individual ethnicity and socioeconomic deprivation. Results: There were 23 423 30-day postoperative episodes. Children of Asian ethnicity were more likely to have a functionally univentricular heart or congenital cardiac risk factors, while children of Black ethnicity were more likely to have Down syndrome and prematurity than children of the remaining ethnicities. Children from the most versus the least deprived areas had higher rates of congenital comorbidity, functionally univentricular heart, high illness severity and urgent operations. After adjusting for case complexity, children from high compared with low deprivation areas had greater odds of prolonged pleural effusion (p=0.05), extracorporeal life support (p=0.001) and unplanned reintervention within 30 days (p=0.04). Conclusions: Greater area deprivation in England is associated with increased preoperative medical complexity and a higher incidence of certain postoperative complications among children with congenital heart disease (CHD). Further research is needed to explore the relationship between ethnic background and perioperative outcomes in CHD and to develop pathways to improvement based on social factors.

The sustainable delivery of Home Health Care (HHC) is increasingly important as ageing populations and rising demands place pressure on health and social care systems. HHC involves complex decision-making across strategic, tactical, and operational levels, often constrained by limited resources such as workforce availability. While Operational Research (OR) has been widely applied to support operational decisions in HHC, recent reviews highlight a lack of focus on strategic and tactical planning, and limited recognition of the hierarchical structure linking decisions across levels. This undermines the potential of OR to inform real-world planning effectively. To address these gaps, we propose a modelling approach enabling consistent analysis of decisions across planning levels. We defined a modular approach for analysing hierarchies of decisions and accounting for cascade effects. Then, we applied those principles by developing a configurable tool in R, comprising a synthetic data generator and a suite of optimisation and heuristic routines. We illustrate the benefits of this approach through a case study. Our results demonstrate the value of this structured modelling approach for informing decisions in HHC. The emphasis we gave on modularity facilitated the development of an analysis tool that can be easily adapted to different hierarchies of decisions and settings.

Background Population studies of congenital heart disease (CHD) often include only children receiving cardiac interventions, underestimating the burden of cases without intervention. We evaluated outcomes for all detected structural CHD cases in England from fetal life to the age of 1 year. Method We linked the National Congenital Anomaly and Rare Disease Registration Service, the National Congenital Heart Disease Audit, and Office for National Statistics mortality records to construct an incident cohort with estimated delivery/birth dates 2018–2020. Outcomes were: termination of pregnancy, fetal loss (miscarriage/stillbirth), live birth with no cardiac intervention in infancy, and live birth with intervention(s) in infancy. Infant mortality at the age of 1 year was assessed. Results Among 11 265 CHD cases, 63.7% were antenatally detected (95% CI 62.8% to 64.6%), rising to 94.2% (92.0% to 96.0%) for hypoplastic left heart syndrome (HLHS). There were 1766 terminations (15.7%, 95% CI 14.7% to 16.7%), 295 fetal losses (2.6%, 95% CI 1.6% to 3.6%), 4538 live births with no infant cardiac intervention (40.3%, 95% CI 39.3% to 41.3%) and 4666 with intervention(s) (41.4%, 95% CI 40.4% to 42.4%). Termination was higher with greater CHD complexity (eg, HLHS 51.1% (95% CI 46.8% to 55.5%) versus isolated ventricular septal defect 6.0% (95% CI 4.3% to 7.7%), pConclusion This national, linked cohort shows that un-intervened cases account for most infant deaths and that antenatal detection exceeds 90% for the most complex lesions. Registries and quality improvement should include all CHD care pathways to inform counselling and equitable service planning.

OBJECTIVES: As post-operative mortality for paediatric cardiac surgery is very low, we aimed to develop methods for monitoring of post-operative complication rates, given their impact upon children’s health and wellbeing. METHODS: We used national registry data to develop and evaluate a suite of risk adjustment models for the outcomes of 6 defined post-operative complications, designed for use in complication monitoring for quality assurance. RESULTS: There were 23,423 30-day post-operative episodes in children under the age of 18-years undergoing cardiac surgery between 2015–2021 in England and Wales, with 361 (1.5%) deaths <30-days. 257 (1.9%) of 13,556 post-operative episodes in infants (0.85. The performance of the models for necrotising enterocolitis, acute neurological event and unplanned reintervention were less good (AUC statistics 0.74–0.79). CONCLUSIONS: Although complications are more complex outcome measures than mortality, national registry data can be used to capture them and to evaluate methods for risk adjustment of these outcomes. These methods may enable future risk-adjusted monitoring of complication metrics for quality assurance.