Early Cardiac Catheterizations within 30 Days Post Congenital Heart Surgery in Children

Background:This study set out to assess the indications,feasibility,safety,and outcome of early cardiac catheterizations(CC)within 30 days after congenital heart surgery(CHS)in children.Methods and Results:This is a retrospective,single-center case review study of all CC within 30 days after CHS between 1/2010-12/2020.A total of 317(138 diagnostic,179 interventional)CC were performed in 245 patients at a median of 4 days(IQR 13)after CHS.The median age was 3 months(IQR 6),and body weight was 5 kg(IQR 4).A total of 194(61.2%)CC were performed in patients with univentricular hearts.CC revealed significant pathologies leading to early redo-surgery in 37 patients(12%).The transcatheter interventions primarily were needed in patients after cavo-pulmonary connection(n=69%,21.8%),right ventricle to pulmonary artery conduit(n=39%,12.3%),and Norwood-I surgery(n=34%,10.7%)presenting with hypoxemia,prolonged postoperative course,and suspected arterial stenosis on echocardiography.The clinical impact of an early postoperative transcatheter intervention for the following clinical course was high in most cases.There were nine(2.8%)major and 20(6.3%)minor intra-procedural complications.Risk factor analysis revealed no difference for the occurrence of complications for patients’age,weight,and time from initial CHS,underlying uni-vs.biventricular heart disease,or ECMO.Conclusion:Early CC within 30 days after CHS in children can be performed safely with a high diagnostic and therapeutic value.The rate of complications is low,while the therapeutic consequence is relevant.

Decreased expression of hyperpolarisation-activated cyclic nucleotide-gated channel 3 in Hirschsprung's disease

AIM: To determine if hyperpolarisation-activated nucleotide-gated(HCN) channels exist in human colon, and to investigate the expression of HCN channels in Hirschsprung's disease.METHODS:We investigated HCN1,HCN2,HCN3 and HCN4 protein expression in pull-through specimens from patients with Hirschsprung’s disease(HSCR,n=10)using the proximal-most ganglionic segment and distalmost aganglionic segment,as well as in healthy control specimens obtained at the time of sigmoid colostomy closure in children who had undergone anorectoplasty for imperforate anus(n=10).Fluorescent immunohistochemistry was performed to assess protein distribution,which was then visualized using confocal microscopy.RESULTS:No HCN1 channel expression was observed in any of the tissues studied.Both HCN2 and HCN4proteins were found to be equally expressed in the aganglionic and ganglionic bowel in HSCR and controls.HCN3 channel expression was found to be markedly decreased in the aganglionic colon vs ganglionic colon and controls.HCN2-4 channels were seen to be expressed within neurons of the myenteric and submucosal plexus of the ganglionic bowel and normal controls,and also co-localised to interstitial cells of Cajal in all tissues studied.CONCLUSION:We demonstrate HCN channel expression in human colon for the first time.Reduced HCN3expression in aganglionic bowel suggests its potential role in HSCR pathophysiology.

Update on nutritional status,body composition and growth in paediatric inflammatory bowel disease

Growth and nutritional status are important issues in paediatric inflammatory bowel disease(IBD).While linear growth is easy to assess,nutritional status is more complicated,with reports often compromised by the use of simple measures,such as weight and the body mass index,to assess nutritional status rather than more appropriate and sophisticated techniques to measure body composition.This review is an update on what is currently known about nutritional status as determined by body composition in paediatric IBD.Further,this review will focus on the impact of biologics on growth in paediatric IBD.Significant lean mass deficits have been reported in children with IBD compared with controls,and there is evidence these deficits persist over time.Furthermore,data imply that gender differences exist in body composition,both at diagnosis and in response to treatment.With respect to growth improvements following treatment with biologics,there are conflicting data.While some studies report enhancement of growth,others do not.The relationship between disease severity,impaired growth and the requirement for biologics needs to be considered when interpreting these data.However,key features associated with improvements in growth appear to be successful clinical response to treatment,patients in early stages of puberty,and the presence of growth failure at the onset of treatment.