Several studies have reported treatment options for patients with Kawasaki disease refractory to standard immunoglobulin therapy; however, no studies have reported low-dose immunoglobulin therapy for patients with a low risk of Kawasaki disease.

A total of 277 patients with Kawasaki disease were included in this study. We used Kobayashi score and our Less high-risk score to divide the patients into three groups. Patients in the high-risk group (Kobayashi score ≥ 5 points) received 2 g/kg intravenous immunoglobulin and prednisolone. Patients in the moderate-risk group (Kobayashi score < 5 points and Less high-risk score ≥ 2 points) received 2 g/kg intravenous immunoglobulin treatment. Patients in the low-risk group (Kobayashi score < 5 points and Less high-risk score < 2 points) received 1 g/kg intravenous immunoglobulin treatment. The response rate and the incidence of coronary artery lesions at 4 weeks after treatment were evaluated in each group.

The treatment response rates in the high-risk (n = 110), moderate-risk (n = 80), and low-risk (n = 87) groups were 74.5, 72.5, and 77.0%, respectively. Coronary artery lesions occurred in 7.3, 3.8, and 2.3% of patients in the high-, moderate-, and low-risk groups, respectively. There were no significant differences between the groups regarding treatment response or coronary artery lesion rate.

The therapeutic response rate and the therapeutic effect of low-dose intravenous immunoglobulin in the low-risk group identified with our new scoring were satisfactory. Stratified therapies for patients with Kawasaki disease based on the scoring system may be useful.

No established risk prediction tool exists in United Kingdom and Irish Paediatric Cardiology practice for patients undergoing cardiac catheterisation. The Catheterisation RISk score for Paediatrics is used primarily in North American practice to assess risk prior to cardiac catheterisation. Validating the utility and transferability of such a tool in practice provides the opportunity to employ an already established risk assessment tool in everyday practice.

To ascertain whether the Catheterisation RISk score for Paediatrics assessment tool can accurately predict complications within United Kingdom and Irish congenital catheterisation practice.

Clinical and procedural data including National Institute for Cardiovascular Outcomes Research derived outcome data from 1500 patients across five large congenital cardiology centres in the United Kingdom and Ireland were retrospectively collected. Catheterisation RISk score for Paediatrics were then calculated for each case and compared with the observed procedural outcomes. Chi-square analysis was used to determine the relationship between observed and predicted events.

Ninety-eight (6.6%) patients in this study experienced a significant complication as qualified by National Institute for Cardiovascular Outcomes Research classification. 4% experienced a moderate complication, 2.3% experienced a major complication and 0.3% experienced a catastrophic complication resulting in death. Calculated Catheterisation RISk score for Paediatrics scores correlated well with all observed adverse events for paediatric patients across all CRISP categories. The association was also transferable to adult congenital heart disease patients in lower Catheterisation RISk score for Paediatrics categories (CRISP 1–3).

The Catheterisation RISk score for Paediatrics score accurately predicts significant complications in congenital catheterisation practice in the United Kingdom and Ireland. Our data validated the Catheterisation RISk score for Paediatrics assessment tool in five congenital centres using National Institute for Cardiovascular Outcomes Research-derived outcome data.

To evaluate the impact of fetal haemodynamics on surgical and neurodevelopmental outcomes in severe Ebstein anomaly and tricuspid valve dysplasia.

Thirty-four fetuses with Ebstein anomaly/tricuspid valve dysplasia were referred from 2013 to 2019 for fetal echocardiography and clinical management. Nineteen fetuses with Ebstein anomaly/tricuspid valve dysplasia and 30 controls underwent cardiovascular magnetic resonance to quantify the fetal blood flow and to calculate cerebral oxygen delivery (cDO2) and consumption (cVO2). The 3D steady-state free precession acquisition was used to measure fetal brain volume. Surgical outcome, brain MRI, and neurodevelopmental follow-up were reviewed.

Twenty-six fetuses were live born (76%) and survival (65%) at a mean follow-up of 4 years. Nine fetuses had a brain MRI before discharge, and all had clinically silent injuries and volume loss. At 18 months, five single-ventricle patients had a neurodevelopmental delay in cognition and language (mean percentile: 11th), with gross-motor skills more affected than fine-motor skills (mean percentiles: 4th and 34th). Fetuses with Ebstein anomaly/tricuspid valve dysplasia had smaller brains, lower combined ventricular output, ascending aorta, superior caval vien and umbilical vein flows, lower oxygen saturation in ascending aorta and superior caval vien, lower cDO2 and cVO2 (p < 0.05). Superior caval vien/combined ventricular output and descending aorta/combined ventricular output ratios were lower in fetuses with circular shunt (p < 0.05). Fetuses requiring the Starnes procedure tended to have smaller brains, lower combined ventricular output, superior caval vien, descending aorta, and umbilical vein flows.

All patients with Ebstein anomaly/tricuspid valve dysplasia are at high risk of neurodevelopmental delay and warrant follow-up. Fetal cardiovascular magnetic resonance revealed impaired brain growth with diminished cerebral blood flow and cDO2, the extenting dependent on the severity of the haemodynamic compromise.

Paediatric obesity is a worldwide health burden, with growing evidence linking obesity to myocardial function impairments. The study aims to evaluate left ventricular functions among prepubertal obese children to obesity-related clinical and metabolic parameters.

Between June 2019 and March 2020, 40 prepubertal children with obesity were recruited and compared to 40 healthy controls. Patients were assessed for body mass index z scores, waist circumference, body adiposity by bioimpedance analysis, and obesity-related laboratory tests, for example, serum chemerin. Left ventricular functions were assessed using variable echocardiographic modalities, such as M-mode, tissue Doppler, and two-dimensional speckle tracking.

Mean patients’ age was 9.25 ± 1.05 years. Left ventricular mass index, E/E’, and myocardial performance index were significantly increased in obese children than controls. Although M-mode-derived ejection fraction was comparable in both groups, two-dimensional speckle tracking-derived ejection fraction, global longitudinal strain, and global circumferential strain were significantly lower in cases than controls. Left ventricular mass index displayed a positive correlation with body mass index z score (p = 0.003), fat mass index (p = 0.037), and trunk fat mass (p = 0.021). Global longitudinal strain was negatively correlated with body mass index z score (p = 0.015) and fat mass index (p = 0.016). Serum chemerin was positively correlated with myocardial performance index (p = 0.01).

Alterations of left ventricular myocardial functions in prepubertal obese children could be detected using different echocardiographic modalities. Chemerin, body mass index z score, fat mass index, and trunk fat mass were correlated with subclinical left ventricular myocardial dysfunction parameters before puberty. Our results reinforce early and strict management of childhood obesity upon detection of changes in anthropometric and body adiposity indices.

We aimed to assess the current nutritional practices in postoperative patients with Congenital Heart Disease.

Cross-sectional electronic survey was sent to members of The Pediatric Cardiac Intensive Care Society.

In Total, 52 members of the Pediatric Cardiac Intensive Care Society responded to the survey consisting of 14% paediatric intensivist, 53% paediatric cardiac intensivist, and 33% nurse/nurse practitioner with a median of 10 years of experience. There was an even distribution between intensivist (55%) and dietitian or nutritionist (45%) in nutrition management. Ninety-eight percent of respondents report that they would feed patients on inotropic or vasoactive support. Only 27% of respondents reported using indirect calorimetry in calculating resting energy expenditure which is the current gold standard. Academic hospitals compared to non-academic hospital were most likely to report feeding patients within 24 hours postoperative (p = 0.014). Having a feeding protocol was associated with feeding within 24 hours postoperative (p = 0.014) and associated with >50% goal intake by 48 hours postoperative (p = 0.025).

Substantial variation in feeding practice still exists despite the American Society for Parenteral and Enteral Nutrition guidelines from 2017. Report of institutional established feeding protocol was associated with increased early feeding and reaching goal feeds by 48 hours postoperative. Very few centres reported use of indirect calorimetry in postoperative paediatric cardiac intensive care patients and many respondents lacked knowledge about applications in this population. Additional work to determine optimal feeding pathways and assessment of caloric needs in this population is needed.

Adolescents with CHD require transition to specialised adult-centred care. Previous studies have shown that adolescents’ knowledge of their medical condition is correlated with transition readiness. Three-dimensional printed models of CHD have been used to educate medical trainees and patients, although no studies have focused on adolescents with CHD. This study investigates the feasibility of combining patient-specific, digital 3D heart models with tele-education interventions to improve the medical knowledge of adolescents with CHD.

Adolescent patients with CHD, aged between 13 and 18 years old, were enrolled and scheduled for a tele-education session. Patient-specific digital 3D heart models were created using images from clinically indicated cardiac magnetic resonance studies. The tele-education session was performed using commercially available, web-conferencing software (Zoom, Zoom Video Communications Inc.) and a customised software (Cardiac Review 3D, Indicated Inc.) incorporating an interactive display of the digital 3D heart model. Medical knowledge was assessed using pre- and post-session questionnaires that were scored by independent reviewers.

Twenty-two adolescents completed the study. The average age of patients was 16 years old (standard deviation 1.5 years) and 56% of patients identified as female. Patients had a variety of cardiac defects, including tetralogy of Fallot, transposition of great arteries, and coarctation of aorta. Post-intervention, adolescents’ medical knowledge of their cardiac defects and cardiac surgeries improved compared to pre-intervention (p < 0.01).

Combining patient-specific, digital 3D heart models with tele-education sessions can improve adolescents’ medical knowledge and may assist with transition to adult-centred care.

CHD affects over 1 million children in the United States. Studies show decreased mortality from CHD with newborn cardiac screening. California began a screening programme on 1 July, 2013. We evaluated the effect of mandatory screening on surgical outcomes at Loma Linda University Children’s Hospital since 1 July, 2013.

We evaluated all infants having congenital heart surgery at Loma Linda University Children’s Hospital between 1 July, 2013 and 31 December, 2018. Primary target diagnoses include hypoplastic left heart syndrome, pulmonary atresia with intact ventricular septum, tetralogy of Fallot, total anomalous pulmonary venous return, transposition of the great arteries, tricuspid atresia, and truncus arteriosus. Secondary target diagnoses include aortic coarctation, double outlet right ventricle, Ebstein anomaly, interrupted aortic arch, and single ventricle. Patients were stratified by timing of diagnosis (pre-screen, screen positive, and screen negative). Primary end points were post-operative length of stay, operative mortality, absolute mortality, and actuarial survival.

The cohort included 274 infants. Of these, 79% were diagnosed prior to screening (46% prenatally). Only 38% of those screened were positive, with 13% of the cohort having a “missed diagnosis.”

Primary targets were more likely to be diagnosed by screening (53%), while secondary targets were unlikely to be diagnosed by screening (10%) (p = 0.004). Outcomes such as length of stay, operative mortality, and actuarial survival were not different based on timing of diagnosis (p > 0.05). Despite late diagnosis, those not diagnosed until after screening did not have adverse outcomes.

The standard transcatheter technique to profile the patent ductus arteriosus requires arterial access through the femoral artery and is associated with arterial complications, longer fluoroscopic time, contrast volume, and longer hospital stay.

To compare exclusive transvenous access with the standard procedures for patent ductus arteriosus closure and evaluate whether exclusive venous approach is a safe and effective alternative.

A total of 320 patients were included. A detailed echocardiographic evaluation of the duct morphology was performed. Patients were classified into group 1 included patients who underwent exclusive femoral venous access, without any injections of contrast media and group 2 included patients who underwent arterial and venous access.

Arterial access was achieved in 210 (65.6%). Successful closure of patent ductus arteriosus was achieved in 109 (99.1%) patients in group 1 and in 203 (96.7%) patients in group 2. The patent ductus arteriosus was large and was referred for surgical closure in one patient from group 1 and 7 patients from group 2. Residual patent ductus arteriosus was seen in 6 cases from group 1 (5.4%) and 12 patients from group 2 (5.7%). None of the cases in group 1 had vascular complications, while vascular complications were seen in 20 cases, all of them in group 2 (9.5%). Nonvascular complications were seen in one patient from group 1 (0.9%) and 15 patients in group 2 (7.1%). The procedure time and fluoroscopy times were less in patients with exclusive transvenous access.

Patent ductus arteriosus device closure without arterial access can be accomplished safely and effectively.

Acute kidney injury is a common complication following the Norwood operation. Most neonatal studies report acute kidney injury peaking within the first 48 hours after cardiac surgery. The aim of this study was to evaluate if persistent acute kidney injury (>48 postoperative hours) after the Norwood operation was associated with clinically relevant outcomes.

Two-centre retrospective study among neonates undergoing the Norwood operation. Acute kidney injury was initially identified as developing within the first 48 hours after cardiac surgery and stratified into transient (≤48 hours) and persistent (>48 hours) using the neonatal modification of the Kidney Disease: Improving Global Outcomes serum creatinine criteria. Severe was defined as stage ≥2. Primary and secondary outcomes were mortality and duration of ventilation and hospital length of stay.

One hundred sixty-eight patients were included. Transient and persistent acute kidney injuries occurred in 24 and 17%, respectively. Cardiopulmonary bypass and aortic cross clamp duration, and incidence of cardiac arrest were greater among those with persistent kidney injury. Mortality was four times higher (41 versus 12%, p < 0.001) and mechanical ventilation duration 50 hours longer in persistent acute kidney injury patients (158 versus 107 hours; p < 0.001). In multivariable analysis, persistent acute kidney injury was not associated with mortality, duration of ventilation or length of stay. Severe persistent acute kidney injury was associated with a 59% increase in expected ventilation duration (aIRR:1.59, 95% CI:1.16, 2.18; p = 0.004).

Future large studies are needed to determine if risk factors and outcomes change by delineating acute kidney injury into discrete timing phenotypes.

To compare patients treated for incomplete Kawasaki disease whose practitioners followed versus did not follow American Heart Association criteria and to evaluate the association of cardiology consultation with adherence to these guidelines.

Single centre retrospective cohort study of patients <18 years old who received ≥1 dose of intravenous immunoglobulin for Kawasaki disease between 01/2006 and 01/2018. We collected demographics, clinical and laboratory data, coronary artery abnormalities, and cardiology consultation status. Patients treated for incomplete Kawasaki disease were divided into two groups based on adherence versus nonadherence to American Heart Association guidelines and compared by Wilcoxon rank sum test and chi-squared or Fisher’s exact test.

Of the 357 patients treated for Kawasaki disease, 109 (31%) were classified as incomplete Kawasaki disease. The American Heart Association algorithm for identifying patients with incomplete Kawasaki disease was followed in 81/109 (74%). Coronary artery abnormalities were present in 46/109 (42%) of the patients who were treated for incomplete Kawasaki disease. Cardiology consultation was more frequent in those fulfilling American Heart Association criteria for the diagnosis of incomplete Kawasaki disease versus those who did not fulfill criteria (76% versus 48%, p = 0.005).

Over 25% of patients treated for incomplete Kawasaki disease did not meet American Heart Association guidelines. Guidelines were more frequently followed when the paediatric cardiology team was consulted. Consulting physicians with experience and expertise in the evaluation and management of incomplete KD should be strongly considered in the care of these patients.

Vascular rings cause respiratory symptoms in children. Treatment consists of surgical division; however, data regarding mid-term results are scarce. The purpose of this study was to evaluate clinical results of vascular ring surgery.

Retrospective chart review of consecutive patients who underwent vascular ring surgery. Mid-term follow-up consisted of clinic visits and telephone questionnaire over a 1-year period sampling at five points in time.

Follow-up of 85 patients who underwent vascular rings surgery revealed significant symptomatic improvement within 6 months. In total, 50% were symptomatic to some degree at their last documented clinic visit complaining of stridor (36.8%), cough (34.2%), wheezing (10.5%), dyspnoea on exertion (10.5%), or recurrent respiratory infections (23.7%). By telephone questionnaires, 83% described a significant improvement in respiratory symptoms, 63.4% had some residual symptoms, 36.6% stridor, 38.8% chronic cough, 32.4% bronchodilator use,18.3% had at least one episode of pneumonia per year, 31% of children had dyspnoea or respiratory limitation, and 12.7% while doing physical activities. We found no association between the age at surgery or anatomic variant to the presence of symptoms at mid-term follow-up.

Surgical division of vascular rings results in significant clinical improvement within 1 year; nevertheless, some patients remain symptomatic to some degree. We found no association between the age at surgery or anatomic variant to the presence of symptoms at mid-term follow-up. Further evaluation is warranted to assess the nature of residual symptoms and explore whether anatomical causes can be identified leading to surgical modifications.

The medical histories, laboratory results, cardiac findings, and treatments of the cases that were diagnosed with Multisystem Inflammatory Syndrome in Children between December 2020 and August 2021 were evaluated retrospectively.

Our study group consisted of 14 males and 12 females whose median age was 3.67 years. Of the 26 patients, 24 had echocardiographic findings and 12 cases had cardiac pathologies that were mostly mild. Among these, mitral valve insufficiency, coronary artery pathology, and pericardial effusion were the most common. Perivascular brightness, aortic and tricuspid insufficiency, systolic dysfunction, and tricuspid thrombosis were less common. The cardiac pathologies of all patients resolved in less than a month following treatment.

Although the cardiac pathologies of Multisystem Inflammatory Syndrome in Children cases disappear fairly rapidly, the long-term cardiac effects of this disease are not known clearly. To improve our current understanding of Multisystem Inflammatory Syndrome in Children, more multi-centred studies with long-term follow-up periods should be conducted, and treatment protocols for cases of different severities should be developed to maximise the treatments’ efficacy.

Children with CHD may be at increased risk for epilepsy. While the incidence of perioperative seizures after surgical repair of CHD has been well-described, the incidence of epilepsy is less well-defined. We aim to determine the incidence and predictors of epilepsy in patients with CHD.

Retrospective cohort study of patients with CHD who underwent cardiopulmonary bypass at <2 years of age between January, 2012 and December, 2013 and had at least 2 years of follow-up. Clinical variables were extracted from a cardiac surgery database and hospital records. Seizures were defined as acute if they occurred within 7 days after an inciting event. Epilepsy was defined based on the International League Against Epilepsy criteria.

Two-hundred and twenty-one patients were identified, 157 of whom were included in our analysis. Five patients (3.2%) developed epilepsy. Acute seizures occurred in 12 (7.7%) patients, only one of whom developed epilepsy. Predictors of epilepsy included an earlier gestational age, a lower birth weight, a greater number of cardiac surgeries, a need for extracorporeal membrane oxygenation or a left ventricular assist device, arterial ischaemic stroke, and a longer hospital length of stay.

Epilepsy in children with CHD is rare. The mechanism of epileptogenesis in these patients may be the result of a complex interaction of patient-specific factors, some of which may be present even before surgery. Larger long-term follow-up studies are needed to identify risk factors associated with epilepsy in these patients.

Advances in surgical techniques and post-operative management of children with CHD have significantly lowered mortality rates. Unplanned cardiac interventions are a significant complication with implications on morbidity and mortality.

We conducted a single-centre retrospective case–control study for patients (<18 years) undergoing cardiac surgery for repair of Tetralogy of Fallot between January 2009 and December 2019. Data included patient characteristics, operative variables and outcomes. This study aimed to assess the incidence and risk factors for reintervention of Tetralogy of Fallot after cardiac surgery. The secondary outcome was to examine the incidence of long-term morbidity and mortality in those who underwent unplanned reinterventions.

During the study period 29 patients (6.8%) underwent unplanned reintervention, and were matched to 58 patients by age, weight and sex. Median age was 146 days, and median weight was 5.8 kg. Operative mortality was 7%, and 1-year survival was 86% for the entire cohort (cases and controls). Hispanic patients were more likely to have reinterventions (p = 0.04) in the unadjusted analysis, while Asian, Pacific Islander and Native American (p = 0.01) in the multi-variate analysis. Patients that underwent reintervention were more likely to have post-op arrhythmia, genetic syndromes and higher operative and 1-year mortality (p < 0.05).

Unplanned cardiac interventions following Tetralogy of Fallot repair are common, and associated with increased operative, and 1-year mortality. Race, genetic syndromes and post-operative arrhythmia are associated with increased odds of unplanned reinterventions. Future studies are needed to identify modifiable risk factors to minimise unplanned reinterventions.

Increasing recognition of paediatric inflammatory multi-system syndrome is a cause of concern. This study aimed to evaluate children with paediatric inflammatory multi-system syndrome and compare the clinical and laboratory features of children with and without cardiac involvement.

We conducted a prospective single-centre study including 57 (male 37, 65%) patients with paediatric inflammatory multi-system syndrome at a tertiary care hospital between November, 2020 and March, 2021. The mean age was 8.8 ± 4.5 years (range, 10 months–16.7 years).

The most frequent symptoms were fever (100%), abdominal pain (65%) and diarrhoea (42%). SARS-CoV-2 PCR and serology tests were positive in 3 (5%) and 52 (91%) patients, respectively. Eight patients required intensive care support. Nineteen patients (33%) had cardiac involvement (valvular regurgitation in 15, left ventricular systolic dysfunction in 11 and coronary artery dilation in 1). The presence and duration of cough and intensive care admissions were significantly higher in children with cardiac involvement than those without it. The cut-off values of troponin T, pro-brain natriuretic peptide and interleukin 6 for predicting cardiac involvement were 11.65 ng/L (95% confidence interval, 0.63–0.90; sensitivity, 0.63; specificity, 0.84; area under the curve: 0.775, p = 0.009), 849.5 pg/mL (95% CI, 0.54–0.86; sensitivity, 0.63; specificity, 0.63; area under the curve: 0.706, p = 0.009) and 39.8 pg/mL (95% CI, 0.54–0.85; sensitivity, 0.63; specificity, 0.60; area under the curve: 0.698, p = 0.023), respectively.

Cardiac involvement in children with paediatric inflammatory multi-system syndrome is common. The risk of cardiac involvement can be predicted by troponin T, pro-brain natriuretic peptide and interleukin 6 levels.

To examine relationships amongst parental post-traumatic stress symptoms, parental post-traumatic growth, overprotective parenting, and child emotional/behavioural problems in families of children with critical CHD.

Sixty parents (15 fathers) of children aged 1–6 completed online questionnaires assessing parental post-traumatic stress symptoms and post-traumatic growth, overprotective parenting, and child emotional/behavioural problems. Bivariate correlations and mediational analyses were conducted to evaluate overprotective parenting as a mediator of the association between parental post-traumatic stress symptoms and child emotional/behavioural problems.

Parents reported significant post-traumatic stress symptoms, with over 18% meeting criteria for post–traumatic stress disorder and 70% meeting criteria in one or more clusters. Parental post-traumatic growth was positively correlated with intrusion (r = .32, p = .01) but it was not associated with other post-traumatic stress symptom clusters. Parental post-traumatic stress symptoms were positively associated with overprotective parenting (r = .37, p = .008) and total child emotional/behavioural problems (r = .29, p = .037). Overprotective parenting was positively associated with total child emotional/behavioural problems (r = .45, p = .001) and fully mediated the relationship between parental post-traumatic stress symptoms and child emotional/behavioural problems.

Overprotective parenting mediates the relationship between parental post-traumatic stress symptoms and child emotional and behavioural problems in families of children with CHD. Both parental post-traumatic stress symptoms and overprotective parenting may be modifiable risk factors for poor child outcomes. This study highlights the need for interventions to prevent or reduce parental post-traumatic stress symptoms and to promote effective parenting following a diagnosis of CHD.