To compare early neurocognitive development in children born with and without isolated CHD using the Bayley Scales of Infant and Toddler Development (3rd edition) and the Ages and Stages Questionnaire (3rd edition).

Recruitment took place before birth. Women expecting fetuses with and without CHD causing disturbances in the flow of oxygenated blood to the fetal brain were included in a prospective cohort study comprising fetal MRI (previously published) and neurodevelopmental follow-up. We now present the 18- and 36-month neurodevelopmental follow-up using the Bayley Scales according to age and the 6-month-above-age Ages and Stages Questionnaire in 15 children with and 27 children without CHD.

Children with CHD had, compared with the children without CHD, an increased risk of scoring ≤ 100 in the Bayley Scales cognition category at 18 and 36 -months; relative risk 1.7 (95% confidence interval (CI): 1.0–2.8) and 3.1 (CI: 1.2–7.5), respectively. They also achieved lower scores in the 6-month-above-age Ages and Stages Questionnaires (24 and 42 months) communication; mean z-score difference −0.72 (CI: −1.4; −0.1) and −1.06 (CI: −1.8; −0.3) and gross motor; mean z-score difference: −0.87 (CI: −1.7; −0.1) and −1.22 (CI: −2.4; −0.02) categories.

The children with CHD achieved lower scores in the Bayley Scales cognition category and the Ages and Stages Questionnaire communication and gross motor categories possibly indicative of early neurodevelopmental deficiencies. We recommend early screening and monitoring for neurodevelopmental delays in children with CHD in order to improve further neurodevelopment and educational achievements.

Noonan syndrome is a genetic disorder with high prevalence of congenital heart defects, such as pulmonary stenosis, atrial septal defect and hypertrophic cardiomyopathy. Scarce data exists regarding the safety of pregnancy in patients with Noonan syndrome, particularly in the context of maternal cardiac disease.

We performed a retrospective chart review of patients at Yale-New Haven Hospital from 2012 to 2020 with diagnoses of Noonan syndrome and pregnancy. We analysed medical records for pregnancy details and cardiac health, including echocardiograms to quantify maternal cardiac dysfunction through measurements of pulmonary valve peak gradient, structural heart defects and interventricular septal thickness.

We identified five women with Noonan syndrome (10 pregnancies). Three of five patients had pulmonary valve stenosis at the time of pregnancy, two of which had undergone cardiac procedures. 50% of pregnancies (5/10) resulted in pre-term birth. 80% (8/10) of all deliveries were converted to caesarean section after a trial of labour. One pregnancy resulted in intra-uterine fetal demise while nine pregnancies resulted in the birth of a living infant. 60% (6/10) of livebirths required care in the neonatal intensive care unit. One infant passed away at 5 weeks of age.

The majority of mothers had pre-existing, though mild, heart disease. We found high rates of prematurity, conversion to caesarean section, and elevated level of care. No maternal complications resulted in long-term morbidity. Our study suggests that women with Noonan syndrome and low-risk cardiac lesions can become pregnant and deliver a healthy infant with counselling and risk evaluation.

The most serious manifestation of rheumatic fever is carditis, as it can lead to chronic rheumatic heart disease. Neutrophil-to-lymphocyte ratio, platelet-to lymphocyte ratio and mean platelet volume have been accepted as novel indicators of the continuing inflammation that is correlated with the severity of valvular involvement in patients with rheumatic carditis.

As a diagnostic study to investigate the correlation of neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio and mean platelet volume with the severity of carditis in children with rheumatic fever.

This was a cross-sectional study at Sanglah Hospital, Bali. Data were collected retrospectively from medical records. Severity of carditis was characterised by a clinical, audible murmur during physical examination and demonstration of valvular involvement by echocardiography. Neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio and mean platelet volume were calculated from complete blood count. The correlation between parameters was assessed using Spearman’s correlation tests.

From January 2018 to December 2020, we found 45 cases admitted to Sanglah Hospital. Carditis was present in 40 (88.9%) subjects. Neutrophil-to-lymphocyte ratio had a strong positive correlation with severity of carditis (r = 0.66, p = 0.001), while platelet-to-lymphocyte ratio had a weak positive correlation with the severity of carditis (r = 0.23, p = 0.13) and mean platelet volume had a very weak negative correlation with the severity of carditis (r = −0.01, p = 0.95). Using a cut-off of 3.4, neutrophil-to-lymphocyte ratio predicted severe carditis with a sensitivity of 81.8% and specificity of 91.3%.

There was a positive correlation between neutrophil-to-lymphocyte ratio and severity of carditis in children with rheumatic fever. Neutrophil-to-lymphocyte ratio might be used as a diagnostic tool to predict the diagnosis of carditis severity.

Tetralogy of Fallot is a common CHD. Studies have shown a close link between heart failure and myocardial fibrosis. Interleukin-6 has been suggested to be a post-independent factor of heart failure. This study aimed to explore the relationship between IL-6 and myocardial fibrosis during cardiopulmonary bypass.

We downloaded the expression profile dataset GSE132176 from Gene Expression Omnibus. After normalising the raw data, Gene Set Enrichment Analysis and differential gene expression analysis were performed using R. Further, a weighted gene correlation network analysis and a protein–protein interaction network analysis were used to identify HUB genes. Finally, we downloaded single-cell expression data for HUB genes using PanglaoDB.

There were 119 differentially expressed genes in right atrium tissues comparing the post-CPB group with the pre-CPB group. IL-6 was found to be significantly up-regulated in the post-CPB group. Six genes (JUN, FOS, ATF3, EGR1, IL-6, and PTGS2) were identified as HUB genes by a weighted gene correlation network analysis and a protein–protein interaction network analysis. Gene Set Enrichment Analysis showed that IL-6 affects the myocardium during CPB mainly through the JAK/STAT signalling pathway. Finally, we used PanglaoDB data to analyse the single-cell expression of the HUB genes.

Our findings suggest that high expression of IL-6 and the activation of the JAK/STAT signalling pathway during CPB maybe the potential mechanism of myocardial fibrosis. We speculate that the high expression of IL-6 might be an important factor leading to heart failure after ToF surgery. We expect that these findings will provide a basis for the development of targeted drugs.

To explore the feasibility and superiority of applying the WeChat platform in a midterm follow-up of surgical repair for ventricular septal defects in infants.

Eighty-six infants with VSD who underwent surgical repair were divided into an outpatient follow-up group and a WeChat follow-up group. The clinical data, including complications, economic cost, time spent, loss to follow-up rate, and parents’ satisfaction at the 3-month and 1-year follow-ups, were recorded and analysed.

There was no significant difference in the incidence of post-operative complications between the two groups. Although the loss to follow-up rate in the WFU group was lower than that of the OFU group, the difference was not statistically significant. The economic cost and time spent in the 3 months and 1 year after discharge in the WFU group were significantly lower than those in the OFU group. One year after discharge, the PSQ-18 score of the WFU group was significantly higher than that of the OFU group.

Compared with outpatient follow-up, the WeChat platform at the midterm follow-up after surgical repair of VSDs in infants has the advantages of saving time and economic costs and improves parents’ satisfaction.

Differentiation between post-operative inflammation and bacterial infection remains an important issue in infants following congenital heart surgery. We primarily assessed kinetics and predictive value of C-reactive protein for bacterial infection in the early (days 0–4) and late (days 5–28) period after cardiopulmonary bypass surgery. Secondary objectives were frequency, type, and timing of post-operative infection related to the risk adjustment for congenital heart surgery score.

This 3-year single-centre retrospective cohort study in a paediatric cardiac ICU analysed 191 infants accounting for 235 episodes of CPBP surgery. Primary outcome was kinetics of CRP in the first 28 days after CPBP surgery in infected and non-infected patients.

We observed 22 infectious episodes in the early and 34 in the late post-operative period. CRP kinetics in the early post-operative period did not accurately differentiate between infected and non-infected patients. In the late post-operative period, infected infants displayed significantly higher CRP values with a median of 7.91 (1.64–22.02) and 6.92 mg/dl (1.92–19.65) on days 2 and 3 compared to 4.02 (1.99–15.9) and 3.72 mg/dl (1.08–9.72) in the non-infection group. Combining CRP on days 2 and 3 after suspicion of infection revealed a cut-off of 9.47 mg/L with an acceptable predictive accuracy of 76%.

In neonates and infants, CRP kinetics is not useful to predict infection in the first 72 hours after CPBP surgery due to the inflammatory response. However, in the late post-operative period, CRP is a valuable adjunctive diagnostic test in conjunction with clinical presentation and microbiological diagnostics.

Adults with systemic right ventricle have a significant risk for long-term complications such as arrhythmias or heart failure.

A nationwide retrospective study based on the German National Register for Congenital Heart Disease was performed. Patients with transposition of the great arteries after atrial switch operation or congenitally corrected TGA were included.

Two hundred and eight-five patients with transposition of the great arteries after atrial switch operation and 95 patients with congenitally corrected transposition of the great arteries were included (mean age 33 years). Systolic function of the systemic ventricle was moderately or severely reduced in 25.5 % after atrial switch operation and in 35.1% in patients with congenitally corrected transposition. Regurgitation of the systemic atrioventricular valve was present in 39.5% and 43.2% of the cases, respectively. A significant percentage of patients also had a history for supraventricular or ventricular arrhythmias. However, polypharmacy of cardiovascular drugs was rare (4.5%) and 38.5 % of the patients did not take any cardiovascular medication. The amount of cardiovascular drugs taken was associated with NYHA class as well as systemic right ventricular dysfunction. Patients with congenitally corrected transposition were more likely to receive pharmacological treatment than patients after atrial switch operation.

A significant portion of patients with systemic right ventricle suffer from a relevant systemic ventricular dysfunction, systemic atrioventricular valve regurgitation, and arrhythmias. Despite this, medication for heart failure treatment is not universally used in this cohort. This emphasises the need for randomised trials in patient with systemic right ventricle.

To assess the feasibility of percutaneous transcatheter device closure of ventricular septal defects in children weighing less than 10 kg.

Although percutaneous transcatheter device closure of ventricular septal defect is a well-established method of treatment in older children and adolescents, there is limited data on device closure in small children weighing less than 10 kg. We present our institutional experience of transcatheter VSD closure in children weighing less than 10 kg.

Medical records were reviewed for 16 children, who were selected for device closure of ventricular septal defects based on the inclusion criteria.

Out of 65 patients with a diagnosis of ventricular septal defect, 16 children less than 10 kg were attempted for percutaneous device closure. In 13 patients, the device was successfully released, and 3 patients needed surgical closure of the defect. Mean age and weight of the patients were 17.3 ± 12.7 months and 6.8 ± 3.2 kg, respectively. Mean defect size was 6 mm (range 3–10). There was no incidence of device embolisation or heart block or death. Five patients had residual left-to-right shunt immediately after the device release, which got closed by the first month’s follow-up. We had one accidental perforation of right ventricular free wall at the time of crossing of the defect, which was successfully repaired surgically.

Percutaneous device closure of ventricular septal defect in small children with weight below 10 kg is feasible with good short-term outcome. Careful patient selection is essential for procedural success and to avoid complications.

Loss of follow-up is a barrier to providing adequate care to paediatric cardiac patients. The purpose of this study was to determine variables associated with loss of appropriate paediatric cardiology follow-up, including potentially modifiable factors. We hypothesised having earlier recommend follow-up intervals was associated with less likelihood of loss of follow-up.

We performed a retrospective cohort study of patients >5 years old seen in a large, outpatient paediatric practice from 2013 to 2016. Subjects were considered to be lost to follow-up if they did not have a subsequent outpatient encounter by 6 months after their recommend follow-up time interval.

Of the 8940 eligible patients, 45.9% were lost to follow-up. Recommended follow-up interval of 1 year was associated with less loss of follow-up (41.4%) as compared to 2-year intervals (51.6%) and 3 years (55.7%) (p < 0.001 for both). Other significant predictors of loss of follow-up included less severe heart disease, older age, and non-Hispanic Black race/ethnicity. Sex and payor type were not significant predictors. In the stratified analyses by severity of disease and age, longer recommended follow-up time was associated with greater loss of follow-up among all severity and age categories.

Almost half of the patients in our cohort did not return to clinic within the recommended timeline. Shorter follow-up time was associated with less loss of follow-up among all categories of disease severity and age groups. Recommending shorter follow-up intervals may be one initiative for paediatric cardiologists to improve rates of follow-up.

The purpose of this study is to describe the evolution of pulmonary artery hypertension after repair of complete atrioventricular canal defect and to determine the time to normalisation of pulmonary artery pressure in both patients with and without Trisomy 21.

This is a single centre, retrospective analysis of patients with complete atrioventricular canal defect admitted for surgical repair at the University of Maryland Medical Center from 2005 to 2015. T-test or Mann–Whitney test and Chi-Square or Fisher’s exact tests were used to compare the two groups (patients with Trisomy 21 and those without). Repeated measures of analysis of variance and serial measurement analysis were performed.

Twenty-nine patients meeting the inclusion criteria underwent repair of complete atrioventricular defects during the study period. The right ventricular pressure estimate remained elevated over time and did not show a significant difference between the two groups. Right ventricular to systolic blood pressure ratios for all patients remained > 0.5 over the time periods assessed.

Our study suggests that in patients with complete atrioventricular canal defects, the right ventricular pressure remains elevated and does not normalise on echocardiograms performed up to one year after surgery, suggesting a sustained elevation in pulmonary vascular resistance.

Growing evidence has emphasised the importance of ventricular performance in functionally single-ventricle patients, particularly concerning diastolic function. Cardiac MRI has been proposed as non-invasive alternative to pre-Fontan cardiac catheterisation in selected patients.

To identify clinical and cardiac magnetic resonance predictors of high pre-Fontan end-diastolic ventricular pressure.

In a retrospective single-centre study, 38 patients with functionally univentricular heart candidate for Fontan intervention, who underwent pre-Fontan cardiac catheterisation, beside a comprehensive cardiac MRI, echocardiographic, and clinical assessment were included. Medical and surgical history, cardiac magnetic resonance, cardiac catheterisation, echocardiographic, and clinical data were recorded. We investigated the association between non-invasive parameters and cardiac catheterisation pre-Fontan risk factors, in particular with end-diastolic ventricular pressure. Moreover, the impact of conventional invasive pre-Fontan risk factor on post-operative outcome as also assessed.

Post-operative complications were associated with higher end-diastolic ventricular pressure and Mayo Clinic indexes (p < 0.01 and p = 0.05, respectively). At receiver operating characteristic curve analysis end-diastolic ventricular pressure ≥ 10.5 mmHg predicted post-operative complications with a sensitivity of 75% and specificity of 88% (AUC: 0.795, 95% CI 0.576;1.000, p < 0.05). At multivariate analysis, both systemic right ventricle (OR: 23.312, 95% CI: 2.704–200.979, p < 0.01) and superior caval vein indexed flow (OR: 0.996, 95% CI: 0.993–0.999, p < 0.05) influenced end-diastolic ventricular pressure ≥ 10.5 mmHg.

A reduced superior caval vein flow, evaluated at cardiac magnetic resonance, is associated with higher end-diastolic ventricular pressure a predictor of early adverse outcome in post-Fontan patients.

Patients with CHD can be exposed to high levels of cumulative ionising radiation. Utilisation of electroanatomic mapping during catheter ablation leads to reduced radiation exposure in the general population but has not been well studied in patients with CHD. This study evaluated the radiation sparing benefit of using three-dimensional mapping in patients with CHD.

Data were retrospectively collected from the Catheter Ablation with Reduction or Elimination of Fluoroscopy multi-institutional registry. Patients with CHD were selected. Those with previous ablations, concurrent diagnostic or interventional catheterisation and unknown arrhythmogenic foci were excluded. The control cohort was matched for operating physician, arrhythmia mechanism, arrhythmia location, weight and age. The procedure time, rate of fluoroscopy use, fluoroscopy time, procedural success, complications, and distribution of procedures per year were compared between the two groups.

Fifty-six patients with congenital heart disease and 56 matched patients without CHD were included. The mean total procedure time was significantly higher in patients with CHD (212.6 versus 169.5 minutes, p = 0.003). Their median total fluoroscopy time was 4.4 minutes (compared to 1.8 minutes), and their rate of fluoroscopy use was 23% (compared to 13%). The acute success and minor complication rates were similar and no major complications occurred.

With the use of electroanatomic mapping during catheter ablation, fluoroscopy use can be reduced in patients with CHD. The majority of patients with CHD received zero fluoroscopy.

Acute respiratory distress is one of the most common reasons for paediatric emergency visits. Paediatric patients require rapid diagnosis and treatment. Our aim in this study was to use N-terminal (1–76) pro-brain natriuretic peptide to differentiate respiratory distress of cardiac and pulmonary origin in children. Our aim was to investigate the role of N-terminal (1–76) pro-brain natriuretic peptide in the detection of patients with new-onset heart failure in the absence of an underlying congenital heart anomaly.

All children aged 0–18 years who presented to the paediatric emergency department due to severe respiratory distress were included in the study prospectively. The patients’ demographic characteristics, presenting complaints, clinical findings, and N-terminal (1–76) pro-brain natriuretic peptide concentrations, were investigated. In patients with severe Pediatric Respiratory Severity Score, congestive heart failure score was calculated using the modified Ross Score.

This study included 47 children between the ages of 1 month and 14 years. The median N-terminal (1–76) pro-brain natriuretic peptide concentration was 5717 (IQR:16158) pg/mL in the 25 patients with severe respiratory distress due to heart failure and in the 22 patients with severe respiratory distress due to lung pathology was 437 (IQR:874) pg/mL (p < 0.001). In the 25 patients with severe respiratory distress due to heart failure, 8281 (IQR:8372) pg/mL in the 16 patients with underlying congenital heart anomalies, and 1983 (IQR:2150) pg/mL in the 9 patients without a congenital heart anomaly (p < 0.001). The 45 patients in the control group had a median N-terminal (1–76) pro-brain natriuretic peptide concentration of 47.2 (IQR:56.2) pg/mL.

Using scoring systems in combination with N-terminal (1–76) pro-brain natriuretic peptide cut-off values can help direct and manage treatment.

Acute kidney injury is a severe complication following complete repair of tetralogy of Fallot. Anaerobic metabolism is believed to contribute to the development of acute kidney injury. The ratio of central venous to arterial carbon dioxide tension to arterio-venous oxygen content (PV-ACO2/CA-VO2) has been proposed as a surrogate for respiratory quotient and an indicator of tissue oxygenation. We hypothesised that a small increase of PV-ACO2/CA-VO2 might have superior discrimination ability in subsequent acute kidney injury prediction.

This study is retrospective and single-centre design study. The study population consisted of 61 children with tetralogy of Fallot that underwent a complete surgical repair between July 2017 and January 2021. Baseline characteristics and intra-operative parameters were collected through a retrospective chart review. PV-ACO2/CA-VO2 was collected within 12 hours of surgical completion. Acute kidney injury was defined according to the criteria established by the Kidney Disease: Improving Global Outcomes group. Univariate and logistic regression analyses were performed to determine risk factors for acute kidney injury.

Of the 61 patients, 20 (32.8%) developed acute kidney injury. Multivariate logistic analyses showed that age, height, haematocrit, and Pv-aCO2/Ca-vO2 were independently associated with the development of acute kidney injury. The addition of Pv-aCO2/Ca-vO2 to the model significantly increased model discrimination [AUROC 0.939 (95% CI 0.894–0.984) and AUROC 0.922 (95% CI 0.869–0.975), respectively].

The increase of PV-ACO2/CA-VO2 could improve the predictive ability for subsequent development of acute kidney injury in children with tetralogy of Fallot.

The objective of this study was to investigate changes in serum biomarkers of acute brain injury, including white matter and astrocyte injury during chronic foetal hypoxaemia. We have previously shown histopathological changes in myelination and neuronal density in fetuses with chronic foetal hypoxaemia at a level consistent with CHD.

Mid-gestation foetal sheep (110 ± 3 days gestation) were cannulated and attached to a pumpless, low-resistance oxygenator circuit, and incubated in a sterile fluid environment mimicking the intrauterine environment. Fetuses were maintained with an oxygen delivery of 20–25 ml/kg/min (normoxemia) or 14–16 ml/kg/min (hypoxaemia). Myelin Basic Protein and Glial Fibrillary Acidic Protein serum levels in the two groups were assessed by ELISA at baseline and at 7, 14, and 21 days of support.

Based on overlapping 95% confidence intervals, there were no statistically significant differences in either Myelin Basic Protein or Glial Fibrillary Acidic Protein serum levels between the normoxemic and hypoxemic groups, at any time point. No statistically significant correlations were observed between oxygen delivery and levels of Myelin Basic Protein and Glial Fibrillary Acidic Protein.

Chronic foetal hypoxaemia during mid-gestation is not associated with elevated serum levels of acute white matter (Myelin Basic Protein) or astrocyte injury (Glial Fibrillary Acidic Protein), in this model. In conjunction with our previously reported findings, our data support the hypothesis that the brain dysmaturity with impaired myelination found in fetuses with chronic hypoxaemia is caused by disruption of normal developmental pathways rather than by direct cellular injury.

We describe a cohort of children referred with multisystem inflammatory syndrome in children associated with severe acute respiratory syndrome coronavirus 2 and compare this cohort with a 2019 cohort of children with Kawasaki disease.

We conducted a retrospective cohort study of 2019 and 2020 referrals to the inflammatory cardiology service at Great Ormond Street Hospital for Children. We compared cardiac and inflammatory parameters of a sub-section of the 2020 cohort who presented with reduced left ventricular ejection fraction with the remainder of the cohort.

Referrals significantly increased between February and June 2020 compared to 2019 (19.8/30 days versus 3.9/30 days). Frequency of coronary artery aneurysms (11/79 (13.9%) versus 7/47 (14.9%)) or severe coronary artery aneurysms (6/79 (7.6%) versus 3/47 (6.4%)) was similar between 2020 and 2019, respectively. The 2020 cohort was older (median age 9.07 years versus 2.38 years), more likely to be of Black, Asian, or other minority ethnic group (60/76 (78.9%) versus 25/42 (59.5%)), and more likely to require inotropic support (22 (27.5%) versus 0 (0%)). Even children with significantly reduced left ventricular ejection fraction demonstrated complete recovery of cardiac function within 10 days (mean 5.25 days ± 2.7).

We observed complete recovery of myocardial dysfunction and an overall low rate of permanent coronary sequelae, indicating that the majority of children with multisystem inflammatory syndrome in children are unlikely to encounter long-term cardiac morbidity. Although the frequency of myocardial dysfunction and inotropic support requirement is not consistent with a diagnosis of Kawasaki disease, the frequency of coronary artery abnormalities and severe coronary artery abnormalities suggests a degree of phenotypic overlap.