The Efficacy of Corticosteroids, NSAIDs, and Colchicine in the Treatment of Pediatric Postoperative Pericardial Effusion

The objective of this study is to investigate and compare the efficacy of corticosteroids, NSAIDs, and colchicine in treating postoperative pericardial effusion (PPE) following cardiac surgery in the pediatric setting, on the basis of available literature. To investigate and compare the efficacy of corticosteroids, NSAIDs, and colchicine in treating postoperative pericardial effusion (PPE) following cardiac surgery in the pediatric setting, on the basis of available literature. A systematic review was conducted by carrying out a database search in PubMed on April 20th, 2021. An English language filter was added, but no time restrictions were applied. Lack of pediatric literature prompted a broadening of the search to include adult literature. One pediatric and four adult studies were included, but the pediatric evidence was not found to be of satisfactory quality, and the findings of adult literature could not be readily generalized to the pediatric setting. No well-founded conclusions could be drawn regarding the efficacy of corticosteroids, NSAIDs, or colchicine in treating PPE, as a striking lack of evidence for their efficacy in the pediatric setting were revealed. A knowledge gap was found in the literature, indicating a need for good-quality randomized controlled trials to bridge this gap

Platelet reactivity in young children undergoing congenital heart disease surgery: a NITRIC randomized clinical trial substudy

Introduction The international NITRIC trial studied the hypothesis that nitric oxide (NO) applied into the cardiopulmonary bypass (CPB) oxygenator in infants would improve recovery after heart surgery. In a substudy, we evaluated the effect of NO applied into the CPB oxygenator on the (re)activity of platelets measured as fibrinogen binding (platelet aggregation) and P-selectin expression (platelet degranulation) in young children. Methods Platelet activity (without agonist exposure) and reactivity (after stimulation by an agonist) was studied in a single center substudy of the NITRIC trial, a multicenter, randomized trial that studied administration of 20 parts per million (ppm) NO during CPB in children younger than 2 years. Blood was collected at 4 time points (T1- T4); before CPB, after CPB start, before and after weaning. Flow cytometry-based platelet activity and reactivity in the presence of 5 agonists was tested. Differences on P-selectin expression and fibrinogen binding (median fluorescence intensity (MFI)) were analyzed with mixed effect modelling (MEM). Results Blood samples were obtained in 22 patients allocated to NO and 20 controls. Platelet counts dropped after T1 due to the hemodilution of blood in all patients (p  0.27) in any of the MEMs. Conclusion 20 ppm NO administration in the sweep gas of the CPB oxygenator did not affect platelet reactivity in young children undergoing heart surgery. Interestingly, duration of CPB exposure also did not have an effect on platelet (re)activity

Playing for cognition: investigating the feasibility and user experience of a virtual reality serious game for cognitive assessment in children with congenital heart disease

Background In order to facilitate the development and implementation of innovative technology in clinical practice, it is important to understand the user experience of end-users. Virtual Reality (VR) offers the possibility to assess cognitive functioning in a dynamic environment that simulates real-world situations. The purpose of this cross-sectional study was to investigate the feasibility of a VR Serious Game for cognitive assessment in school-aged children with congenital heart disease (CHD). The sub-aims were two-fold: (1) to objectively evaluate the feasibility of the VR Serious Game in children with CHD in comparison to typically developing (TD) children and (2) to explore the user experience of both groups following their interaction with the VR Serious Game. Results A total of 101 children participated in this study; 98 children were included in the final analysis (CHD: n = 54; TD: n = 47). The VR Serious Game appeared feasible for both children with CHD and TD children, with 88% children completing the innovative VR assessment without encountering any issues. There were no discernible differences in completion rates between groups. Children with CHD reported significantly lower scores than TD children on three user experience scales: Engagement, Flow and Presence. Nonetheless, the scores for Engagement and Flow were still considered "moderate to good". Both groups reported minimal adverse physiological reactions. Conclusions The findings suggested that the VR Serious Game was feasible for children with CHD and that the user experience was positive. Future research should investigate the effectiveness of the VR Serious Game compared with a conventional or digital neuropsychological assessment, prioritising the development of novel outcome measures that can better estimate and explain the impact of cognitive impairment on daily functioning

Perioperative Management of an Adolescent Fontan Patient with Narrowing of the Aortopulmonary Space for Posterior Scoliosis Surgery

Congenital cardiac patients who received neonatal reconstructive aortic arch surgery are at risk of aortopulmonary space narrowing with compression of the left pulmonary artery and left main bronchus (LMB) later in life. We discuss a challenging adolescent single ventricle patient who presented for surgical treatment of a non-idiopathic thoracic scoliosis (posterior spinal fusion) with severe stenosis of the LMB and left pulmonary artery due to a narrow aortopulmonary space. Careful preoperative imaging, evaluation, and decision making resulted in successful surgical treatment and uneventful perioperative course

Does Repeated Measurement of a 6-Min Walk Test Contribute to Risk Prediction in Children with Dilated Cardiomyopathy?

A single 6-min walk test (6MWT) can be used to identify children with dilated cardiomyopathy (DCM) with a high risk of death or heart transplantation. To determine if repeated 6MWT has added value in addition to a single 6MWT in predicting death or heart transplantation in children with DCM. Prospective multicenter cohort study including ambulatory DCM patients >= 6 years. A 6MWT was performed 1 to 4 times per year. The distance walked was expressed as percentage of predicted (6MWD%). We compared the temporal evolution of 6MWD% in patients with and without the study endpoint (SE: all-cause death or heart transplantation), using a linear mixed effects model. In 57 patients, we obtained a median of 4 (IQR 2-6) 6MWTs per patient during a median of 3.0 years of observation (IQR 1.5-5.1). Fourteen patients reached a SE (3 deaths, 11 heart transplantations). At any time during follow-up, the average estimate of 6MWD% was significantly lower in patients with a SE compared to patients without a SE. In both patients groups, 6MWD% remained constant over time. An absolute 1% lower 6MWD% was associated with an 11% higher risk (hazard) of the SE (HR 0.90, 95% CI 0.86-0.95 p <0.001). Children with DCM who died or underwent heart transplantation had systematically reduced 6MWD%. The performance of all patients was stable over time, so repeated measurement of 6MWT within this time frame had little added value over a single test

Predicting outcome in children with dilated cardiomyopathy:the use of repeated measurements of risk factors for outcome

Aims We aimed to determine whether in children with dilated cardiomyopathy repeated measurement of known risk factors for death or heart transplantation (HTx) during disease progression can identify children at the highest risk for adverse outcome. Methods and results Of 137 children we included in a prospective cohort, 36 (26%) reached the study endpoint (SE: all-cause death or HTx), 15 (11%) died at a median of 0.09 years [inter-quartile range (IQR) 0.03-0.7] after diagnosis, and 21 (15%) underwent HTx at a median of 2.9 years [IQR 0.8-6.1] after diagnosis. Median follow-up was 2.1 years [IQR 0.8-4.3]. Twenty-three children recovered at a median of 0.6 years [IQR 0.5-1.4] after diagnosis, and 78 children had ongoing disease at the end of the study. Children who reached the SE could be distinguished from those who did not, based on the temporal evolution of four risk factors: stunting of length growth (-0.42 vs. -0.02 length Z-score per year, P 6 years at presentation (all P < 0.001) were predictive of adverse outcome. In multivariate analysis, NT-proBNP appeared the only independent predictor for adverse outcome, a two-fold higher NT-proBNP was associated with a 2.8 times higher risk of the SE (hazard ratio 2.78, 95% confidence interval 1.81-3.94, P < 0.001). Conclusions The evolution over time of NT-proBNP, LVIDd, length growth, and NYU PHFI identified a subgroup of children with dilated cardiomyopathy at high risk for adverse outcome. In this sample, with a limited number of endpoints, NT-proBNP was the strongest independent predictor for adverse outcome

Genetic Evaluation of A Nation-Wide Dutch Pediatric DCM Cohort:The Use of Genetic Testing in Risk Stratification

BACKGROUND: This study aimed to describe the current practice and results of genetic evaluation in Dutch children with dilated cardiomyopathy and to evaluate genotype-phenotype correlations that may guide prognosis. METHODS: We performed a multicenter observational study in children diagnosed with dilated cardiomyopathy, from 2010 to 2017. RESULTS: One hundred forty-four children were included. Initial diagnostic categories were idiopathic dilated cardiomyopathy in 67 children (47%), myocarditis in 23 (16%), neuromuscular in 7 (5%), familial in 18 (13%), inborn error of metabolism in 4 (3%), malformation syndrome in 2 (1%), and "other" in 23 (16%). Median follow-up time was 2.1 years [IQR 1.0-4.3]. Hundred-seven patients (74%) underwent genetic testing. We found a likely pathogenic or pathogenic variant in 38 children (36%), most often in MYH7 (n = 8). In 1 patient initially diagnosed with myocarditis, a pathogenic LMNA variant was found. During the study, 39 patients (27%) reached study endpoint (SE: all-cause death or heart transplantation). Patients with a likely pathogenic or pathogenic variant were more likely to reach SE compared with those without (hazard ratio 2.8; 95% CI 1.3-5.8, P = 0.007), while transplant-free survival was significantly lower (P = 0.006). Clinical characteristics at diagnosis did not differ between the 2 groups. CONCLUSIONS: Genetic testing is a valuable tool for predicting prognosis in children with dilated cardiomyopathy, with carriers of a likely pathogenic or pathogenic variant having a worse prognosis overall. Genetic testing should be incorporated in clinical work-up of all children with dilated cardiomyopathy regardless of presumed disease pathogenesis

Does Repeated Measurement of a 6-Min Walk Test Contribute to Risk Prediction in Children with Dilated Cardiomyopathy?

A single 6-min walk test (6MWT) can be used to identify children with dilated cardiomyopathy (DCM) with a high risk of death or heart transplantation. To determine if repeated 6MWT has added value in addition to a single 6MWT in predicting death or heart transplantation in children with DCM. Prospective multicenter cohort study including ambulatory DCM

Predicting outcome in children with dilated cardiomyopathy: the use of repeated measurements of risk factors for outcome

Aims: We aimed to determine whether in children with dilated cardiomyopathy repeated measurement of known risk factors for death or heart transplantation (HTx) during disease progression can identify children at the highest risk for adverse outcome. Methods and results: Of 137 children we included in a prospective cohort, 36 (26%) reached the study endpoint (SE: all-cause death or HTx), 15 (11%) died at a median of 0.09 years [inter-quartile range (IQR) 0.03–0.7] after diagnosis, and 21 (15%) underwent HTx at a median of 2.9 years [IQR 0.8–6.1] after diagnosis. Median follow-up was 2.1 years [IQR 0.8–4.3]. Twenty-three children recovered at a median of 0.6 years [IQR 0.5–1.4] after diagnosis, and 78 children had ongoing disease at the end of the study. Children who reached the SE could be distinguished from those who did not, based on the temporal evolution of four risk factors: stunting of length growth (−0.42 vs. −0.02 length Z-score per year, P < 0.001), less decrease in N-terminal pro-B-type natriu