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

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

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.

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

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

Improving Risk Adjustment for Mortality After Pediatric Cardiac Surgery: The UK PRAiS2 Model.

BACKGROUND: Partial Risk Adjustment in Surgery (PRAiS), a risk model for 30-day mortality after children's heart surgery, has been used by the UK National Congenital Heart Disease Audit to report expected risk-adjusted survival since 2013. This study aimed to improve the model by incorporating additional comorbidity and diagnostic information. METHODS: The model development dataset was all procedures performed between 2009 and 2014 in all UK and Ireland congenital cardiac centers. The outcome measure was death within each 30-day surgical episode. Model development followed an iterative process of clinical discussion and development and assessment of models using logistic regression under 25 × 5 cross-validation. Performance was measured using Akaike information criterion, the area under the receiver-operating characteristic curve (AUC), and calibration. The final model was assessed in an external 2014 to 2015 validation dataset. RESULTS: The development dataset comprised 21,838 30-day surgical episodes, with 539 deaths (mortality, 2.5%). The validation dataset comprised 4,207 episodes, with 97 deaths (mortality, 2.3%). The updated risk model included 15 procedural, 11 diagnostic, and 4 comorbidity groupings, and nonlinear functions of age and weight. Performance under cross-validation was: median AUC of 0.83 (range, 0.82 to 0.83), median calibration slope and intercept of 0.92 (range, 0.64 to 1.25) and -0.23 (range, -1.08 to 0.85) respectively. In the validation dataset, the AUC was 0.86 (95% confidence interval [CI], 0.82 to 0.89), and the calibration slope and intercept were 1.01 (95% CI, 0.83 to 1.18) and 0.11 (95% CI, -0.45 to 0.67), respectively, showing excellent performance. CONCLUSIONS: A more sophisticated PRAiS2 risk model for UK use was developed with additional comorbidity and diagnostic information, alongside age and weight as nonlinear variables.

Incorporating Comorbidity Within Risk Adjustment for UK Pediatric Cardiac Surgery.

BACKGROUND: When considering early survival rates after pediatric cardiac surgery it is essential to adjust for risk linked to case complexity. An important but previously less well understood component of case mix complexity is comorbidity. METHODS: The National Congenital Heart Disease Audit data representing all pediatric cardiac surgery procedures undertaken in the United Kingdom and Ireland between 2009 and 2014 was used to develop and test groupings for comorbidity and additional non-procedure-based risk factors within a risk adjustment model for 30-day mortality. A mixture of expert consensus based opinion and empiric statistical analyses were used to define and test the new comorbidity groups. RESULTS: The study dataset consisted of 21,838 pediatric cardiac surgical procedure episodes in 18,834 patients with 539 deaths (raw 30-day mortality rate, 2.5%). In addition to surgical procedure type, primary cardiac diagnosis, univentricular status, age, weight, procedure type (bypass, nonbypass, or hybrid), and era, the new risk factor groups of non-Down congenital anomalies, acquired comorbidities, increased severity of illness indicators (eg, preoperative mechanical ventilation or circulatory support) and additional cardiac risk factors (eg, heart muscle conditions and raised pulmonary arterial pressure) all independently increased the risk of operative mortality. CONCLUSIONS: In an era of low mortality rates across a wide range of operations, non-procedure-based risk factors form a vital element of risk adjustment and their presence leads to wide variations in the predicted risk of a given operation.

Real time monitoring of risk-adjusted paediatric cardiac surgery outcomes using variable life-adjusted display: implementation in three UK centres.

OBJECTIVE: To implement routine in-house monitoring of risk-adjusted 30-day mortality following paediatric cardiac surgery. DESIGN: Collaborative monitoring software development and implementation in three specialist centres. PATIENTS AND METHODS: Analyses incorporated 2 years of data routinely audited by the National Institute of Cardiac Outcomes Research (NICOR). Exclusion criteria were patients over 16 or undergoing non-cardiac or only catheter procedures. We applied the partial risk adjustment in surgery (PRAiS) risk model for death within 30 days following surgery and generated variable life-adjusted display (VLAD) charts for each centre. These were shared with each clinical team and feedback was sought. RESULTS: Participating centres were Great Ormond Street Hospital, Evelina Children's Hospital and The Royal Hospital for Sick Children in Glasgow. Data captured all procedures performed between 1 January 2010 and 31 December 2011. This incorporated 2490 30-day episodes of care, 66 of which were associated with a death within 30 days.The VLAD charts generated for each centre displayed trends in outcomes benchmarked to recent national outcomes. All centres ended the 2-year period within four deaths from what would be expected. The VLAD charts were shared in multidisciplinary meetings and clinical teams reported that they were a useful addition to existing quality assurance initiatives. Each centre is continuing to use the prototype software to monitor their in-house surgical outcomes. CONCLUSIONS: Timely and routine monitoring of risk-adjusted mortality following paediatric cardiac surgery is feasible. Close liaison with hospital data managers as well as clinicians was crucial to the success of the project.

Cardiovascular magnetic resonance imaging in congenital heart disease as an alternative to diagnostic invasive cardiac catheterization: a single center experience.

OBJECTIVE: The study aims to assess whether the increasing use of cardiovascular magnetic resonance imaging in place of diagnostic cardiac catheterization in the management of pediatric patients with congenital heart disease has had an impact on pediatric cardiac care. DESIGN: Retrospective analysis of data was used. SETTING: The study was performed at the Evelina Children's Hospital Cardiology Department. PATIENTS. : Elective diagnostic cardiac catheterization or magnetic resonance imaging (MRI) from 2005-2010 are included (n = 896). OUTCOME MEASURES: Indication, length of stay, and incidence of complications were recorded. In cases used to plan surgery, 30-day survival following the procedure was recorded. Surgical outcomes were compared between the two groups. Surgical outcomes planned using MRI were compared with national outcomes from Congenital Cardiac Audit Database. RESULTS: For catheterizations (50 patients, [31 male, median age 3 years, interquartile range 1 to 12]), median hospital stay was 1 day (interquartile range 0 to 3), and complications occurred in 11 (22%). Median hospital stay for MRI (846 patients [517 male, median age 3 years, interquartile range 0 to 9]) was significantly shorter: 0 days (interquartile range 0 to 1, P <.001), with fewer complications (16 [1.9%], P <.0001). Twenty-four catheter and 283 MRI patients underwent surgery within 18 months. One catheter patient (2.0%) and four MRI patients (1.4%) died within 30 days (P =.48). CONCLUSION: Replacing catheterization with cardiovascular magnetic resonance imaging has resulted in reduced rates of complication and shorter hospital stays without a significant impact on surgical outcome.

Serial magnetic resonance imaging in hypoplastic left heart syndrome gives valuable insight into ventricular and vascular adaptation.

OBJECTIVES: This study sought to investigate changes in magnetic resonance imaging (MRI) ventricular volumes and vascular dimensions before hemi-Fontan (HF) and before total cavopulmonary connection (TCPC) in children with hypoplastic left heart syndrome (HLHS). BACKGROUND: The systemic right ventricle (RV) in HLHS is subject to significant changes in volume loading throughout the surgical stages of palliation, particularly after the HF. METHODS: Fifty-eight patients had paired pre-HF and pre-TCPC MRI for assessment of changes of RV volumes, neoaortic flow, and vascular dimensions. RESULTS: Comparison of pre-HF and pre-TCPC MRI results showed a decrease of indexed RV end-diastolic volume and end-systolic volume (98 ml/m(2) to 87 ml/m(2) and 50 ml/m(2) to 36 ml/m(2), respectively) with stroke volume remaining constant (49 ml/m(2) vs. 51 ml/m(2)), leading to an increased RV ejection fraction (51% vs. 59%). These findings persisted after excluding the 3 patients who underwent tricuspid valve repair as part of their HF procedure. Indexed RV end-diastolic volume plotted against neoaortic stroke volume demonstrated a Frank-Starling-like curve that shifted upward after HF. The indexed distal left and right cross-sectional pulmonary artery areas were reduced after HF. CONCLUSIONS: In HLHS, serial MRI shows the adaptation of the systemic RV after HF with volume reduction in the context of a preserved stroke volume and an increased ejection fraction. The staged palliation in HLHS may be a risk factor particularly for reduced left pulmonary artery growth in itself as no factors investigated in this study were found to significantly impact on this.