Performance of a single-ventricle home-monitoring programme: survival and predictors of adverse outcome.

INTRODUCTION AND BACKGROUND: Mortality between stages 1 and 2 single-ventricle palliation is significant. Home-monitoring programmes are suggested to reduce mortality. Outcomes and risk factors for adverse outcomes for European programmes have not been published. AIMS: To evaluate the performance of a home-monitoring programme at a medium-sized United Kingdom centre with regards survival and compare performance with other home-monitoring programmes in the literature. METHODS: All fetal and postnatal diagnosis of a single ventricle were investigated with in-depth analysis of those undergoing stage 1 palliation and entered the home-monitoring programme between 2016 and 2020. The primary outcome was survival. Secondary outcomes included multiple parameters as potential predictors of death or adverse outcome. RESULTS: Of 217 fetal single-ventricle diagnoses during the period 2016-2020, 50.2% progressed to live birth, 35.4% to stage 1 and 29.5% to stage 2. Seventy-four patients (including 10 with postnatal diagnosis) entered the home-monitoring programme with six deaths making home-monitoring programme mortality 8.1%. Risk factors for death were the hybrid procedure as the only primary procedure (OR 33.0, p < 0.01), impaired cardiac function (OR 10.3, p < 0.025), Asian ethnicity (OR 9.3, p < 0.025), lower mean birth-weight (2.69 kg versus 3.31 kg, p < 0.01), and lower mean weight centiles during interstage follow-up (mean centiles of 3.1 versus 10.8, p < 0.01). CONCLUSION: Survival in the home-monitoring programme is comparable with other home-monitoring programmes in the literature. Hybrid procedure, cardiac dysfunction, sub-optimal weight gain, and Asian ethnicity were significant risk factors for death. Home-monitoring programmes should continue to raise awareness of these factors and seek solutions to mitigate adverse events. Future work to generalise home-monitoring programme and single-ventricle fetus to stage 2 outcomes in the United Kingdom will require multi-centre collaboration.

Out-of-hours versus in-hours delivery of antenatally diagnosed transposition of the great arteries: outcomes from a United Kingdom Tertiary Centre.

OBJECTIVE: To study the impact of out-of-hours delivery on outcome for neonates with antenatally diagnosed transposition of the great arteries. SETTING: Tertiary paediatric cardiology centre (Yorkshire, United Kingdom), with co-located tertiary neonatal unit. PATIENTS: Neonates with antenatally diagnosed simple transposition of the great arteries delivered out-of-hours (Monday to Friday 17:00-08:00 and weekends) versus in-hours between 2015 and 2020. OUTCOME: The primary outcome was survival to hospital discharge. Secondary outcomes included neurological morbidity, length of stay, and time to balloon atrial septostomy. RESULTS: Of 51 neonates, 38 (75%) were delivered out-of-hours. All neonates born in the tertiary centre survived to discharge. Time to balloon atrial septostomy was slightly longer for out-of-hours deliveries compared to in-hours (median 130 versus 93 mins, p = 0.33). Neurological morbidity occurred for nine (24%) patients in the out-of-hours group and one (8%) in-hours (OR 3.72, 95% CI: 0.42-32.71, p = 0.24). Length of stay was also similar (18.5 versus 17.3 days, p = 0.59). Antenatal diagnosis of a restrictive atrial septum was associated with a lower initial pH (7.03 versus 7.13; CI: 0.03-0.17, p = 0.01), longer length of stay (22.6 versus 17.3 days; CI: 0.37-10.17, p = 0.04), and increased neurological morbidity (44% versus 14%; OR 4.80, CI 1.00-23.15, p = 0.05). A further three neonates were delivered in surrounding hospitals, with a mortality of 67% (versus 0 in tertiary centre; OR 172, CI 5-5371, p = 0.003). CONCLUSION: Neonates with antenatally diagnosed transposition of the great arteries have similar outcomes when delivered out-of-hours versus in-hours. Antenatal diagnosis of restrictive atrial septum is a significant predictor of worse outcomes. In our region, delivery outside the tertiary cardiac centre had a significantly higher risk of mortality.

Defining significant childhood illness and injury in the Emergency Department: a consensus of UK and Ireland expert opinion.

BACKGROUND: Clarifying whether paediatric early warning scores (PEWS) accurately predict significant illness is a research priority for UK and Ireland paediatric emergency medicine (EM). However, a standardised list of significant conditions to benchmark these scores does not exist. OBJECTIVES: To establish standardised significant illness endpoints for use in determining the performance accuracy of PEWS and safety systems in emergency departments (ED), using a consensus of expert opinion in the UK and Ireland. DESIGN: Between July 2017 and February 2018, three online Delphi rounds established a consensus on 'significant' clinical conditions, derived from a list of common childhood illness/injury ED presentations. Conditions warranting acute hospital admission in the opinion of the respondent were defined as 'significant', using a 5-point Likert scale. The consensus was a priori ≥80% (positive or negative). 258 clinical conditions were tested. PARTICIPANTS AND SETTINGS: Eligible participants were consultants in acute or EM paediatrics, or adult EM, accessed via 53 PERUKI (Paediatric Emergency Research in the UK and Ireland)'s research collaborative sites, and 27 GAPRUKI (General and Adolescent Paediatric Research in the UK and Ireland)'s sites, 17 of which overlap with PERUKI. MAIN OUTCOME MEASURES: To create a list of conditions regarded as 'significant'with ≥80% expert consensus. RESULTS: 43 (68%) of 63 PERUKI and GAPRUKI sites responded; 295 experts were invited to participate. Participants in rounds 1, 2 and 3 were 223 (76%), 177 (60%) and 148 (50%), respectively; 154 conditions reached positive consensus as 'significant'; 1 condition reached a negative consensus (uncomplicated Henoch-Schönlein purpura); and 37 conditions achieved non-consensus. CONCLUSIONS: A list of significant childhood conditions has been created using UK and Irish expert consensus, for research purposes, for the first time. This will be used as the benchmark endpoint list for future research into PEWS/safety systems performance in EDs.

Is the medical treatment for arterial hypertension after primary aortic coarctation repair related to age at surgery? A retrospective cohort study.

BACKGROUND: Hypertension following primary coarctation repair affects up to a third of subjects. A number of studies suggest that future hypertension risk is reduced if primary repair is performed at a younger age. OBJECTIVES: The objective of this study was to evaluate the risk of future medical treatment for hypertension depending on age of primary coarctation repair. METHODS: This study was carried out at a tertiary paediatric cardiology referral centre. Retrospective database evaluation of children aged 28 days and ⩽12 months), and children (>12 months). Main outcome measure is the need for long-term anti-hypertensive medication. The risk for re-coarctation was also evaluated. RESULTS: A total of 87 patients were analysed: 60 neonates, 17 infants, 10 children. Among them, 6.7% neonates, 29.4% infants, and 40% children required long-term anti-hypertensive medications. Group differences were statistically significant (p=0.004). After adjustment for type of repair, the risk of long-term anti-hypertensive therapy was 4.5 (95% confidence interval 1.2-16.9, p=0.025) and 10.5 times (95% confidence interval 2.6-42.3, p=0.001) higher if primary repair was carried out in infancy and childhood, respectively, compared with neonates. Among all, 13 patients developed re-coarctation: 21.7% in the neonatal group, 5.9% in the infant group, and 20% in the child group. We could not demonstrate a significant difference between these proportions or calculate a reliable risk for developing re-coarctation. CONCLUSIONS: Risk of medical treatment for hypertension was lowest when primary repair was carried out during the neonatal period, rising 10-fold if first operated on as a child. Knowing the likelihood of hypertension development depending on age of primary repair is useful for long-term surveillance and counselling.

Can paediatric early warning scores (PEWS) be used to guide the need for hospital admission and predict significant illness in children presenting to the emergency department? An assessment of PEWS diagnostic accuracy using sensitivity and specificity.

OBJECTIVES: Designed to detect early deterioration of the hospitalised child, paediatric early warning scores (PEWS) validity in the emergency department (ED) is less validated. We aimed to evaluate sensitivity and specificity of two commonly used PEWS (Brighton and COAST) in predicting hospital admission and, for the first time, significant illness. METHODS: Retrospective analysis of PEWS data for paediatric ED attendances at St Mary's Hospital, London, UK, in November 2012. Patients with missing data were excluded. Diagnoses were grouped: medical and surgical. To classify diagnoses as significant, established guidelines were used and, where not available, common agreement between three acute paediatricians. RESULTS: 1921 patients were analysed. There were 211 admissions (11%). 1630 attendances were medical (86%) and 273 (14%) surgical. Brighton and COAST PEWS performed similarly. hospital admission: PEWS of ≥3 was specific (93%) but poorly sensitive (32%). The area under the receiver operating curve (AUC) was low at 0.690. Significant illness: for medical illness, PEWS ≥3 was highly specific (96%) but poorly sensitive (44%). The AUC was 0.754 and 0.755 for Brighton and COAST PEWS, respectively. Both scores performed poorly for predicting significant surgical illness (AUC 0.642). PEWS ≥3 performed well in predicting significant respiratory illness: sensitivity 75%, specificity 91%. CONCLUSIONS: Both Brighton and COAST PEWS scores performed similarly. A score of ≥3 has good specificity but poor sensitivity for predicting hospital admission and significant illness. Therefore, a high PEWS should be taken seriously but a low score is poor at ruling out the requirement for admission or serious underlying illness. PEWS was better at detecting significant medical illness compared with detecting the need for admission. PEWS performed poorly in detecting significant surgical illness. PEWS may be particularly useful in evaluating respiratory illness in a paediatric ED.