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Objective: Ventricular hypertrophy (VH) has been observed in children with congenital hyperinsulinism (CHI), a condition of hypoglycaemia characterised by dysregulated insulin secretion, but the prevalence is not known. Patients and methods: Cardiac assessment was performed in children (nZ49) with CHI at diagnosis and follow-up. Two dimensional and Doppler echocardiography studies were used to assess cardiac structures, while M-mode study was used to measure left ventricular (LV) dimensions, subsequently converted to Z scores. Where possible, LV hypertrophy was confirmed by LV mass index (g/m 2.7 ) O95th centile. Results: Cardiac structural lesions were found in 14 (28%) children. At initial echocardiography, VH was present in 31 (65%) children with median (range) LV posterior wall dimension in diastole Z scores of C1.6 (K2.4 to C5.8) and interventricular septal wall dimension in end diastole Z scores of C1.9 (K1.7 to C17.2). At follow-up echocardiography, performed after an interval of 178 (45-390) days, VH persisted in 16 (33%) children. In regression analysis, the presence of VH (odds ratio (95% confidence intervals) 1.1 (1.0-1.2), PZ0.04) at initial echocardiography was correlated with maximum glucose requirement at diagnosis, indicating that severity of CHI at presentation may play a role in the pathogenesis of VH. Conclusions: A significant proportion of children with CHI have cardiac structural lesions. A majority also have VH, which may be associated with the severity of CHI at diagnosis. VH may persist in some children, which requires careful long-term cardiac review.

Background: The Midlands has amongst the highest rates of neonatal and infant mortality in the UK. A public health parent education and empowerment programme, aimed at reducing key risks associated with this mortality was established and evaluated in the region. This was undertaken in an attempt to identify areas for optimal delivery of the public health messages around reducing risks for neonatal and infant mortality. Method: Qualitatively assessment, using the software package Dedoose®, was undertaken. This involved analysis of reflections by the programme trainers, after the delivery of their training sessions to parents, families and carers, between 01 January and 31 December 2021. These were intended to capture insights from the trainers on parent, family, carer and staff perspectives, perceptions/misperceptions around reducing risks for infant mortality. Potential areas for improvement in delivery of the programme were identified from this analysis. Results: A total of 323 programmes, comprising 524 parents, family members and carers were offered the programme. Analysis of 167 reflections around these interactions and those of staff (n = 29) are reported. The programme was positively received across parents, families, carers and staff. Four overall themes were identified: (a) reach and inclusion, (b) knowledge, (c) practical and emotional support and (d) challenges for delivery of the programme. Recommendations for improved delivery of the programme were identified, based on qualitative analysis. Conclusion: This novel approach to empowerment and education around neonatal public health messaging is a valuable tool for parents, families, carers and staff in the Midlands. Key practical recommendations for enhancing delivery of these critical public health messages were identified from this qualitative research. These are likely to be of value in other parts of the UK and globally.

Congenital hyperinsulinism of Infancy (CHI) can be associated with cardiac problems such as septal hypertrophy and reversible hypertrophic cardiomyopathy1 2 however, the prevalence and range of cardiac abnormalities in CHI has not been well investigated. Aims and methods With National Research Ethics Service approval and consent, we retrospectively reviewed the prevalence of cardiac abnormalities in 48 children with CHI. 43 patients were genotyped for potassium channel (KATP) mutations. Echocardiography was used to detect structural abnormalities and parameters for left/right/biventricular hypertrophy (LVH/RVH/BVH) that is, interventricular septal thickness in diastole (IVSd) and left ventricular posterior wall diameter (LVPWd) were used to quantify cardiac dysfunction, with serial echocardiography to assess cardiac improvement. ECG were used to corroborate muscle hypertrophy. Results In our cohort of 48 children, 94% (45/48) required diazoxide therapy for CHI management and 48.8% (21/43) had positive KATP mutations. Median age at echocardiography was 23 days (5 days to 10.8 years). Cardiac structural abnormalities were present in 15 (31%) (five patent ductus arteriosus, four atrial septal defect, two ventricular septal defect, two branch pulmonary artery stenosis, one aortic stenosis and one aberrant subclavian artery). Myocardial hypertrophy was present in 29(60%) [23 LVH, 1 RVH, 5 BVH]. Z scores for LVPWd and IVSd were more than 2SD above mean in 51% and 48% of patients respectively. ECG abnormalities consistent with myocardial hypertrophy were present in only 24% (5/21). Cardiac medications (diuretics, digoxin, β blockers) were required in seven (15%), with improved function and discontinuation of therapy in six (75%). No significant associations were found between birthweights, genotypes and cardiac defects using χ2 analysis. Conclusion Our results show that 60% of CHI patients had echocardiographic evidence of myocardial hypertrophy and 15% (7/48) with myocardial hypertrophy at baseline required cardiac supportive treatment with reversal of pathology in 75% (6/7). About a third of patients had cardiac structural abnormalities. We advocate early cardiac assessment in infants with CHI, with baseline echocardiography as the investigation of choice.