CHD was the most prevalent congenital anomaly (60.9 per 10,000, 95% CI 59.0–62.8) in England in 2018, with 1767 babies born with severe cardiac defects. The 30-day survival rates for complex procedures continue to improve; however despite care advances, the early post-operative period and first year of life remain a critical time for these infants. The Congenital Heart Assessment Tool was developed to support parental decision-making, standardise care provision, improve communication, and the safety and quality of care.

To further evaluate the Congenital Heart Assessment Tool.

A four centre collaborative mixed-methods quality improvement project funded by The Health Foundation, involving eight phases conducted during 2016–2018. Phases six to eight (clinical simulation exercise, parent workshop, and updated tool) are reported in this paper.

Four themes emerged from the clinical simulation exercise (phase six) including: improving documentation; preparation of parents; preparation of health care professionals; and communication. One main theme emerged from the parent workshop (phase seven): “what parents know versus what professionals know [about CHD]”.

These phases further validated the effectiveness of the CHATm in terms of triggering amber and red indicators and demonstrated parents’ ability to identify deterioration in their infant’s clinical condition. Recommendations arising from the quality improvement project enabled the project team to create an updated version of the Congenital Heart Assessment Tool, CHAT2.

Although serum lactate levels are widely accepted markers of haemodynamic instability, an alternative method to evaluate haemodynamic stability/instability continuously and non-invasively may assist in improving the standard of patient care. We hypothesise that blood lactate in paediatric ICU patients can be predicted using machine learning applied to arterial waveforms and perioperative characteristics.

Forty-eight post-operative children, median age 4 months (2.9–11.8 interquartile range), mean baseline heart rate of 131 beats per minute (range 33–197), mean lactate level at admission of 22.3 mg/dL (range 6.3–71.1), were included. Morphological arterial waveform characteristics were acquired and analysed. Predicting lactate levels was accomplished using regression-based supervised learning algorithms, evaluated with hold-out cross-validation, including, basing prediction on the currently acquired physiological measurements along with those acquired at admission, as well as adding the most recent lactate measurement and the time since that measurement as prediction parameters. Algorithms were assessed with mean absolute error, the average of the absolute differences between actual and predicted lactate concentrations. Low values represent superior model performance.

The best performing algorithm was the tuned random forest, which yielded a mean absolute error of 3.38 mg/dL when predicting blood lactate with updated ground truth from the most recent blood draw.

The random forest is capable of predicting serum lactate levels by analysing perioperative variables, including the arterial pressure waveform. Thus, machine learning can predict patient blood lactate levels, a proxy for haemodynamic instability, non-invasively, continuously and with accuracy that may demonstrate clinical utility.

Chylothorax is the accumulation of chyle fluid in the pleural space. The incidence of chylothorax is quite common post-cardiac surgeries in pediatrics especially in Fontan procedures. Although several treatment lines are known for the management of chylothorax, steroids were scarcely reported as a treatment option. We present a unique case of a 4-year-old child who underwent Fontan procedure and suffered a long-term consequence of chylothorax. The chylothorax only fully resolved after introducing corticosteroids as part of her management.

A literature review about management of chylothorax post-cardiac surgery in children using Ovid Medline (19462021), PubMed, and google scholar was performed.

Conservative management without additional surgical intervention is adequate in most patients. Additionally, somatostatin can be used with variable success rate. However, a few cases mentioned using steroids in such cases. More research and reporting on the use of steroids in the treatment of chylothorax post-cardiac surgeries in children is needed to prove its effectivity. In this article, we describe a case of chylothorax post-Fontan procedure that supports the use of steroids.

To investigate health-related quality of life and life satisfaction in children and adolescents treated for isolated congenital valvular aortic stenosis compared to healthy peers. Our second aim was to investigate the relationship between objectively measured physical activity, health-related quality of life and life satisfaction in the same group.

Forty-eight patients, 8–18 years of age, were recruited, as well as 43 healthy peers matched for age, gender and residential area. Health-related quality of life was assessed by the KIDSCREEN-52 self-report and parent proxy report, and life satisfaction was evaluated with the Satisfaction With Life Scale. Physical activity was measured with an accelerometer for 7 days. Sports participation was self-reported.

No differences in the health-related quality of life domains were found between patients and controls in the self-reports. In the proxy reports, parents of the adolescents rated their child’s autonomy lower than did the parents of the healthy controls. A negative relationship was found between moderate to vigorous physical activity, sports participation, life satisfaction and the psychological well-being domain in adolescent patients. In children there was a positive relationship between moderate physical activity and the physical and psychological well-being domains.

Overall, children and adolescents treated for valvular aortic stenosis reported similar life satisfaction and health-related quality of life as their healthy peers. The negative relationships between intense physical activity and sports participation with health-related quality of life and life satisfaction in adolescent patients might be explained by both physical and psychological factors in these teenagers with complex, lifelong heart disease.

Children with right ventricle outflow tract anomalies require repeated heart surgeries, thereby needing regular preoperative assessments throughout their lifetime. This situation puts a heavy burden on these children. Thus, the aim of this study was to explore how children diagnosed with right ventricle outflow tract anomalies experience their heart disease and their everyday life during the preoperative assessment and after the decision on whether to perform a new cardiac surgery.

Individual interviews were conducted with nine children between 9 to 17 years of age on three occasions from 2014 to 2016. In total there were 27 interviews which all were analyzed with thematic analysis.

The analysis yielded three themes and eight subthemes. The theme Me and my heart disease concerns children’s experiences of the heart disease. Almost all described symptoms and how they adapt in their everyday life. The theme Being me concerns the children’s sense of self, where their heart disease was not prominent. The theme Being placed in someone else’s hands describes how the assessment was more of a safety net at least until the decision of heart surgery.

The children’s symptoms, their experiences during the assessment, their future surgeries and how the heart disease affects their everyday life could be better understood as elements of their adaptation to the heart disease. In order to achieve individualized support based on the child’s experiences and to ensure that these children are involved in their own care a child-centered approach is recommended.

As part of a quality improvement project beginning in October 2011, our centre introduced changes to reduce radiation exposure during paediatric cardiac catheterisations. This led to significant initial decreases in radiation to patients. Starting in April 2016, we sought to determine whether these initial reductions were sustained.

After a 30-day trial period, we implemented (1) weight-based reductions in preset frame rates for fluoroscopy and angiography, (2) increased use of collimators and safety shields, (3) utilisation of stored fluoroscopy and virtual magnification, and (4) hiring of a devoted radiation technician. We collected patient weight (kg), total fluoroscopy time (min), and procedure radiation dosage (cGy-cm2) for cardiac catheterisations between October, 2011 and September, 2019.

A total of 1889 procedures were evaluated (196 pre-intervention, 303 in the post-intervention time period, and 1400 in the long-term group). Fluoroscopy times (18.3 ± 13.6 pre; 19.8 ± 14.1 post; 17.11 ± 15.06 long-term, p = 0.782) were not significantly different between the three groups. Patient mean radiation dose per kilogram decreased significantly after the initial quality improvement intervention (39.7% reduction, p = 0.039) and was sustained over the long term (p = 0.043). Provider radiation exposure was also significantly decreased from the onset of this project through the long-term period (overall decrease of 73%, p < 0.01) despite several changes in the interventional cardiologists who made up the team over this time period.

Introduction of technical and clinical practice changes can result in a significant reduction in radiation exposure for patients and providers in a paediatric cardiac catheterisation laboratory. These reductions can be maintained over the long term.

This study evaluated the effect of music intervention on the anxiety and stress responses of patients who underwent an interventional cardiac catheterisation.

The study design was a pre- and post-test randomised controlled trial that included 94 patients who underwent a transcatheter atrial septal defect closure. Patients were allocated to receive either music intervention (n = 47) or usual care (n = 47) during the interventional cardiac catheterisation. Music intervention effectiveness was examined in terms of anxiety, salivary cortisol level, and heart rate variability.

The average age of participants was 45.40 years (±16.04) in the experimental group and 47.26 years (±13.83) in the control group. Two-thirds (66.0%) of the participants in each group were women. State anxiety (F = 31.42, p < 0.001), anxiety-numerical rating scale (F = 20.08, p < 0.001), salivary cortisol levels (F = 4.98, p = 0.021), and low-frequency component/high-frequency component ratio (F = 17.31, p < 0.001) in the experimental group were significantly reduced compared with those in the control group at the end of the music intervention.

This study provides practical evidence of a reduction in anxiety and stress response from music intervention preceding an interventional cardiac catheterisation, indicating that this intervention should be considered in clinical management.

There is sparse literature on cardiac arrhythmias and the utility of ambulatory rhythm monitoring in patients with postural tachycardia syndrome and orthostatic intolerance. This study’s primary aim was to investigate the prevalence of arrhythmias in this population. Knowing the prevalence and types of arrhythmias in dysautonomia patients could influence the decision to pursue ambulatory rhythm monitoring and ultimately guide therapy.

This retrospective descriptive study examined the frequency of cardiac arrhythmias, as detected by ambulatory rhythm monitoring, in children with postural tachycardia syndrome/orthostatic intolerance or syncope who were seen at the Children’s National Hospital Electrophysiology Clinic between January 2001 and December 2020.

In postural tachycardia syndrome/orthostatic intolerance patients, arrhythmia was detected on 15% of 332 ambulatory rhythm monitors. In syncope patients, arrhythmia was detected on 16% of 157 ambulatory rhythm monitors, not significantly different from the postural tachycardia syndrome/orthostatic intolerance group. The difference in rate of arrhythmia detection between 24-hour Holter and 2-week Zio® monitoring was not statistically significant.

This study suggests that a substantial proportion of postural tachycardia syndrome/orthostatic intolerance patients may have concomitant underlying cardiac arrhythmias, at a frequency similar to what is seen in patients undergoing primary evaluation for cardiac symptoms such as chest pain, palpitations, and syncope. In the appropriate clinical context, physicians caring for postural tachycardia syndrome/orthostatic intolerance patients should consider additional evaluation for arrhythmias beyond sinus tachycardia.

Following cardiac surgery, infants often remain endotracheally intubated upon arrival to the cardiac ICU. High-flow nasal cannula and non-invasive positive pressure ventilation are used to support patients following extubation. There are limited data on the superiority of either mode to prevent extubation failure.

We conducted a single-centre retrospective study for infants (<1 year) and/or <10 kg who underwent cardiac surgery between 3/2019–3/2020. Data included patient and clinical characteristics and operative variables. The study aimed to compare high-flow nasal cannula versus non-invasive positive pressure ventilation following extubation and their association with extubation failure. Secondarily, we examined risk factors associated with extubation failure.

There were 424 patients who met inclusion criteria, 320 (75%) were extubated to high-flow nasal cannula, 104 (25%) to non-invasive positive pressure ventilation, and 64 patients (15%) failed extubation. The high-flow nasal cannula group had lower rates of extubation failure (11%, versus 29%, p = 0.001). Infants failing extubation were younger and had higher STAT score (p < 0.05). Compared to high-flow nasal cannula, non-invasive positive pressure ventilation patients were at 3.30 times higher odds of failing extubation after adjusting for patient factors (p < 0.0001).

Extubation failure after cardiac surgery occurs in smaller, younger infants, and those with higher risk surgical procedures. Patients extubated to non-invasive positive pressure ventilation had 3.30 higher odds to fail extubation than patients extubated to high-flow nasal cannula. The optimal mode of respiratory support in this patient population is unknown.

Limited data exist on training of European paediatric and adult congenital cardiologists.

A structured and approved questionnaire was circulated to national delegates of Association for European Paediatric and Congenital Cardiology in 33 European countries.

Delegates from 30 countries (91%) responded. Paediatric cardiology was not recognised as a distinct speciality by the respective ministry of Health in seven countries (23%). Twenty countries (67%) have formally accredited paediatric cardiology training programmes, seven (23%) have substantial informal (not accredited or certified) training, and three (10%) have very limited or no programme. Twenty-two countries have a curriculum. Twelve countries have a national training director. There was one paediatric cardiology centre per 2.66 million population (range 0.87–9.64 million), one cardiac surgical centre per 4.73 million population (range 1.63–10.72 million), and one training centre per 4.29 million population (range 1.63–10.72 million population). The median number of paediatric cardiology fellows per training programme was 4 (range 1–17), and duration of training was 3 years (range 2–5 years). An exit examination in paediatric cardiology was conducted in 16 countries (53%) and certification provided by 20 countries (67%). Paediatric cardiologist number is affected by gross domestic product (R2 = 0.41).

Training varies markedly across European countries. Although formal fellowship programmes exist in many countries, several countries have informal training or no training. Only a minority of countries provide both exit examination and certification. Harmonisation of training and standardisation of exit examination and certification could reduce variation in training thereby promoting high-quality care by European congenital cardiologists.

COVID-19 has markedly impacted the provision of neurodevelopmental care. In response, the Cardiac Neurodevelopmental Outcome Collaborative established a Task Force to assess the telehealth practices of cardiac neurodevelopmental programmes during COVID-19, including adaptation of services, test protocols and interventions, and perceived obstacles, disparities, successes, and training needs.

A 47-item online survey was sent to 42 Cardiac Neurodevelopmental Outcome Collaborative member sites across North America within a 3-week timeframe (22 July to 11 August 2020) to collect cross-sectional data on practices.

Of the 30 participating sites (71.4% response rate), all were providing at least some clinical services at the time of the survey and 24 sites (80%) reported using telehealth. All but one of these sites were offering new telehealth services in response to COVID-19, with the most striking change being the capacity to offer new intervention services for children and their caregivers. Only a third of sites were able to carry out standardised, performance-based, neurodevelopmental testing with children and adolescents using telehealth, and none had completed comparable testing with infants and toddlers. Barriers associated with language, child ability, and access to technology were identified as contributing to disparities in telehealth access.

Telehealth has enabled continuation of at least some cardiac neurodevelopmental services during COVID-19, despite the challenges experienced by providers, children, families, and health systems. The Cardiac Neurodevelopmental Outcome Collaborative provides a unique platform for sharing challenges and successes across sites, as we continue to shape an evidence-based, efficient, and consistent approach to the care of individuals with CHD.

The Pediatric Acute Care Cardiology Collaborative (PAC3) was established to improve acute care cardiology outcomes through the development of an accurate and well-validated clinical registry. We report the validation results of the initial PAC3 registry audits and describe a novel regional audit format developed to accommodate a rapidly expanding membership facilitate collaborative learning and allow for necessary modification due to the COVID-19 pandemic.

Six hospitals were audited using a regional audit format and three hospitals were subsequently audited virtually. Critical and challenging-to-collect data elements were audited among at least 40 randomly selected cases. Discrepancies were categorised as either major or minor depending on their relative importance to patient outcomes and clinical care. Results were tabulated and reported.

We audited 386 encounters and 27,086 individual data fields across 9 hospitals. The aggregate overall accuracy rate was 99.27% and the aggregate major discrepancy rate was 0.51%. The overall accuracy rate ranged from 98.77% to 99.59%, and the major discrepancy rate ranged from 0.26% to 0.88% across the cohort. No appreciable difference was seen between audit formats. Both the regional and virtual audit methods were viewed favourably by participants.

A low data discrepancy rate was found demonstrating that the PAC3 registry is a highly accurate data source for use in quality improvement, benchmarking, and research. Regional audits and virtual audits were both successfully implemented.

The aim of this study was to evaluate the association of bicuspid aortic valve on contemporary outcomes, including reoperation rates, after one-stage correction for interrupted aortic arch with ventricular septal defect or for aortic coarctation with hypoplastic aortic arch and ventricular septal defect.

Seventy-four consecutive patients (35 boys, 47% and 39 girls, 53%) with interrupted aortic arch (n = 41, 55%) or aortic coarctation with hypoplastic aortic arch (n = 33, 45%) with ventricular septal defect underwent early one-stage correction. Twenty (27%) patients had bicuspid aortic valve, and the remaining 54 (73%) had a tricuspid aortic valve. The median aortic valve annulus diameter was 6.0 mm (IQR: 2.0). Patients’ median age was 7 ± 29 days (range, 2–150); median weight was 3.3 ± 0.7 kg (range, 1.5–6.0), with 21 (28%) patients <3.0 kg. Selective brain perfusion through the innominate artery and selective coronary perfusion through the aortic root during aortic arch reconstruction were used in all patients. Statistical analysis was performed using SPSS version 20.0 software (SPSS Inc., Chicago, IL, USA).

The early mortality was 1.3%. One premature neonate died in the hospital with extracorporeal membrane oxygenation after aortic coarctation plus ventricular septal defect repair. There was no further mortality. Median follow-up was 5.7 years (IQR: 10.48). Reinterventions occurred in 36 (49%) patients: balloon angioplasty in 18 (24%) patients, reoperations in 4 (5%) patients, and both in 14 (19%) patients. A total of 86 follow-up procedures were required in these 36 (49%) patients: aortic valve valvulopasty (n = 6, 8%), stent implantation (n = 8, 11%), balloon dilatation (n = 39, 53%), and reoperation (n = 33, 45%). The median time to reinterventions was 9.094 years (SE 0.890). A potential risk factor for reintervention after interrupted aortic arch and aortic coarctation with ventricular septal defect repair was bicuspid aortic valve (p = 0.019, Chi2 (1) = 5.457). In addition, a multivariate Cox analysis with backward selection and significance level <0.015 was applied to all variables that showed significant effects in univariable analyzes. This regression confirmed that bicuspid aortic valve (HR = 0.381, p = .016), and interrupted aortic arch (HR = 0.412, p = 0.043) were predictors of late reintervention. All patients had no obvious neurologic impairment in routine examinations at last follow-up.

Bicuspid aortic valve was a significant risk factor for valve-related reintervention after one-stage repair for aortic arch obstruction with ventricular septal defect due to later development of stenosis associated with higher late morbidity and mortality. Particularly neonates with bicuspid aortic valve will possibly require reintervention in the future. Regular lifelong cardiac follow-up is recommended.