Paediatricians play an integral role in the lifelong care of children with CHD, many of whom will undergo cardiac surgery. There is a paucity of literature for the paediatrician regarding the post-operative care of such patients.

The aim of this manuscript is to summarise essential principles and pertinent lesion-specific context for the care of patients who have undergone surgery or intervention resulting in a biventricular circulation.

Familiarity with common issues following cardiac surgery or intervention, as well as key details regarding specific lesions and surgeries, will aid the paediatrician in providing optimal care for these patients.

The Pediatric Cardiac Critical Care Consortium (PC4) is a multi-institutional quality improvement registry focused on the care delivered in the cardiac ICU for patients with CHD and acquired heart disease. To assess data quality, a rigorous procedure of data auditing has been in place since the inception of the consortium.

This report describes the data auditing process and quantifies the audit results for the initial 39 audits that took place after the transition from version one to version two of the registry’s database.

In total, 2219 total encounters were audited for an average of 57 encounters per site. The overall data accuracy rate across all sites was 99.4%, with a major discrepancy rate of 0.52%. A passing score is based on an overall accuracy of >97% (achieved by all sites) and a major discrepancy rate of <1.5% (achieved by 38 of 39 sites, with 35 of 39 sites having a major discrepancy rate of <1%). Fields with the highest discrepancy rates included arrhythmia type, cardiac arrest count, and current surgical status.

The extensive PC4 auditing process, including initial and routinely scheduled follow-up audits of every participating site, demonstrates an extremely high level of accuracy across a broad array of audited fields and supports the continued use of consortium data to identify best practices in paediatric cardiac critical care.

To assess the training and the future workforce needs of paediatric cardiac critical care faculty.

REDCap surveys were sent May−August 2019 to medical directors and faculty at the 120 US centres participating in the Society of Thoracic Surgeons Congenital Heart Surgery Database. Faculty and directors were asked about personal training pathway and planned employment changes. Directors were additionally asked for current faculty numbers, expected job openings, presence of training programmes, and numbers of trainees. Predictive modelling of the workforce was performed using respondents’ data. Patient volume was projected from US Census data and compared to projected provider availability.

Sixty-six per cent (79/120) of directors and 62% (294/477) of contacted faculty responded. Most respondents had training that incorporated critical care medicine with the majority completing training beyond categorical fellowship. Younger respondents and those in dedicated cardiac ICUs were more significantly likely to have advanced training or dual fellowships in cardiology and critical care medicine. An estimated 49–63 faculty enter the workforce annually from various training pathways. Based on modelling, these faculty will likely fill current and projected open positions over the next 5 years.

Paediatric cardiac critical care training has evolved, such that the majority of faculty now have dual fellowship or advanced training. The projected number of incoming faculty will likely fill open positions within the next 5 years. Institutions with existing or anticipated training programmes should be cognisant of these data and prepare graduates for an increasingly competitive market.

Prenatal diagnosis of critical CHDs and planned peripartum care is an emerging concept in resource-limited settings.

To report the impact of prenatal diagnosis and planned peripartum care on costs of neonatal cardiac care in a resource-limited setting.

Prospective study (October 2019 to October 2020). Consecutive neonates undergoing surgery or catheter-based interventions included. Patients were divided into prenatal (prenatal diagnosis) and post-natal (diagnosis after birth) groups. Costs of cardiac care (total, direct, and indirect) and health expenses to income ratio were compared between study groups; factors impacting costs were analysed.

A total of 105 neonates were included, including 33 in prenatal group. Seventy-seven neonates (73.3%) underwent surgical procedures while the rest needed catheter-based interventions. Total costs were 16.2% lower in the prenatal group (p = 0.008). Direct costs were significantly lower in the prenatal group (18%; p = 0.02), especially in neonates undergoing surgery (20.4% lower; p = 0.001). Health expenses to income ratio was also significantly lower in the prenatal group (2.04 (1.03–2.66) versus post-natal:2.58 (1.55–5.63), p = 0.01);, particularly in patients undergoing surgery (prenatal: 1.58 (1.03–2.66) vs. post-natal: 2.99 (1.91–6.02); p = 0.002). Prenatal diagnosis emerged as the only modifiable factor impacting costs on multivariate analysis.

Prenatal diagnosis and planned peripartum care of critical CHD is feasible in resource-limited settings and is associated with significantly lower costs of neonatal cardiac care. The dual benefit of improved clinical outcomes and lower costs of cardiac care should encourage policymakers in resource-limited settings towards developing more prenatal cardiac services.

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.

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.

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.

Improvements in mortality after congenital heart surgery have necessitated a shift in focus to postoperative morbidity as an outcome measure. We examined late morbidity after congenital heart surgery based on prescription medication use.

Between 1953 and 2009, 10,635 patients underwent congenital heart surgery at <15 years of age in Finland. We obtained 4 age-, sex-, birth-time, and hospital district-matched controls per patient. The Social Insurance Institution of Finland provided data on all prescription medications obtained between 1999 and 2012 by patients and controls. Patients were assigned one diagnosis based on a hierarchical list of cardiac defects and dichotomised into simple and severe groups. Medications were divided into short- and long-term based on indication. Follow-up started at the first operation and ended at death, emigration, or 31 December, 2012.

Totally, 8623 patients met inclusion criteria. Follow-up was 99.9%. In total, 8126 (94%) patients required prescription medications. Systemic anti-bacterials were the most common short-term prescriptions among patients (93%) and controls (88%). Patients required betablockers (simple hazard ratio 1.9, 95% confidence interval 1.7–2.1; severe hazard ratio 6.5, 95% confidence interval 5.3–8.1) and diuretics (simple hazard ratio 3.2, 95% CI 2.8–3.7; severe hazard ratio 38.8, 95% CI 27.5–54.7) more often than the general population. Both simple and severe defects required medication for cardiovascular, gastrointestinal, psychiatric, neurologic, metabolic, autoimmune, and infectious diseases more often than the general population.

The significant risk for postoperative cardiovascular and non-cardiovascular disease warrants close long-term follow-up after congenital heart surgery for all defects.

Data regarding preoperative mortality in neonates with critical CHD are sparse and would aid patient care and family counselling. The objective of this study was to utilise a multicentre administrative dataset to report the rate of and identify risk factors for preoperative in-hospital mortality in neonates with critical CHD across US centres.

The Pediatric Health Information System database was utilised to search for newborns ≤30 days old, born 1 January 2009 to 30 June 2018, with an ICD-9/10 code for d-transposition of the great arteries, truncus arteriosus, interrupted aortic arch, or hypoplastic left heart syndrome. Preoperative in-hospital mortality was defined as patients who died prior to discharge without an ICD code for cardiac surgery or interventional catheterisation.

Overall preoperative mortality rate was at least 5.4% (690/12,739) and varied across diagnoses (d-TGA 2.9%, TA 8.3%, IAA 5.5%, and HLHS 7.3%) and centres (0–20.5%). In multivariable analysis, risk factors associated with preoperative mortality included preterm delivery (<37 weeks) (OR 2.3, 95% CI: 1.8–2.9; p < 0.01), low birth weight (<2.5 kg) (OR 3.8, 95% CI: 3.0–4.7; p < 0.01), and genetic abnormality (OR 1.6, 95% CI: 1.2–2.2; p < 0.01). Centre average surgical volume was not a significant risk factor.

Approximately 1 in 20 neonates with critical CHD suffered preoperative in-hospital mortality, and rates varied across diagnoses and centres. Better understanding of the factors that drive the variation (e.g. patient factors, preoperative care models, surgical timing) could help identify patient care improvement opportunities and inform conversations with families.

This study explored the effects of condition notification based on virtual reality technology on the anxiety levels of parents of children with simple CHD.

The subjects of the study were the parents of 60 children with simple CHD who were treated in our centre. The state anxiety scale was used to assess the anxiety status of the parents of the children before and after the condition notification in different ways (based on virtual reality explanations and written explanations).

In this study, a total of 55 parents of children with CHD successfully completed the survey. At the time of admission, there was no significant difference in the state anxiety scale scores of the parents between the two groups. The anxiety status of the virtual reality group was relieved after the condition notification assisted by virtual reality technology, and there was a significant difference compared with the control group.

Condition notification based on virtual reality technology can effectively alleviate the anxiety of parents of children with simple CHD, which is worthy of clinical application.

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.

Obesity increases the risk of post-operative arrhythmias in adults undergoing cardiac surgery, but little is known regarding the impact of obesity on post-operative arrhythmias after CHD surgery.

Patients undergoing CHD surgery from 2007 to 2019 were prospectively enrolled in the parent study. Telemetry was assessed daily, with documentation of all arrhythmias. Patients aged 2–20 years were categorised by body mass index percentile for age and sex (underweight <5, normal 5–85, overweight 85–95, and obese >95). Patients aged >20 years were categorised using absolute body mass index. We investigated the impact of body mass index category on arrhythmias using univariate and multivariate analysis.

There were 1250 operative cases: 12% underweight, 65% normal weight, 12% overweight, and 11% obese. Post-operative arrhythmias were observed in 38%. Body mass index was significantly higher in those with arrhythmias (18.8 versus 17.8, p = 0.003). There was a linear relationship between body mass index category and incidence of arrhythmias: underweight 33%, normal 38%, overweight 42%, and obese 45% (p = 0.017 for trend). In multivariate analysis, body mass index category was independently associated with post-operative arrhythmias (p = 0.021), with odds ratio 1.64 in obese patients as compared to normal-weight patients (p = 0.036). In addition, aortic cross-clamp time (OR 1.007, p = 0.002) and maximal vasoactive–inotropic score in the first 48 hours (OR 1.03, p = 0.04) were associated with post-operative arrhythmias.

Body mass index is independently associated with incidence of post-operative arrhythmias in children after CHD surgery.

Congenital heart diseases (CHD) are the most common causes of birth defects that have increased the risk of infections. Neonatal sepsis is a life-threatening condition and early diagnosis can be life-saving. We aimed to evaluate the potential role of the systemic immune-inflammatory index in the early diagnosis of neonatal sepsis.

A retrospective cohort study was conducted on 166 newborns with a diagnosis of neonatal sepsis who were admitted to our hospital with CHD between January 2017 and June 2021. Haematological indices including neutrophil/lymphocyte ratio, platelet/lymphocyte ratio, and systemic immune-inflammatory index were calculated for all patients at the time of diagnosis of neonatal sepsis (sepsis). The sepsis values of these indices were compared with the admission values (pre-sepsis) of the patients.

The mean gestational age and birth weight of the patients were 38.36 ± 1.42 weeks and 3057.75 ± 484.68 g. It was found that absolute neutrophil count, systemic immune-inflammatory index, neutrophil/lymphocyte ratio, but not platelet/lymphocyte ratio were significantly increased at the time of sepsis. The receiver operating characteristic curve showed that systemic immune-inflammatory index, neutrophil/lymphocyte ratio, and absolute neutrophil count have predictive ability to define neonatal sepsis among newborns with CHD. The systemic immune-inflammatory index produced an area under the curve receiver operating characteristic curve of 0.76 (70% sensitivity, 70.5% specificity). To discriminate neonatal sepsis, the cut-off values of systemic immune-inflammatory index, neutrophil/lymphocyte ratio, and absolute neutrophil count were 517.19, 2.62, and 9210/mm3, respectively.

As an easily accessible and reliable indicator, systemic immune-inflammatory index may be used in combination with the other parameters in the early diagnosis of neonatal sepsis.

We investigated the risk of increased nosocomial infections and the associated pathogens in patients who underwent paediatric cardiovascular surgery and were put on extracorporeal membrane oxygenation support. We studied the duration of extracorporeal membrane oxygenation use and other variables that may be associated with increased nosocomial infection risk.

Patients who were treated with an extracorporeal membrane oxygenation in paediatric cardiovascular surgery ICU between 2010 and 2020 were included in this retrospective study. We analysed the site of infection and microbiological profile of infections occurring in these patients according to CDC and National Healthcare Safety Network criteria.

The onset of infection development in patients after extracorporeal membrane oxygenation was found to be median 8 (3–15, 25–75 IQR) days in the whole group, and median 11 (3–16, 25–75 IQR) days in those who developed infection without being put on extracorporeal membrane oxygenation. When patients were divided into those with and without infection, duration of ICU was found to be 19 (16–28, IQR 25–75) days in patients with infection vs. 8 (2–16, IQR 25–75; p: <0.001) days in patients without infection. Duration of extracorporeal membrane oxygenation support was found to be 14 (10–25, IQR 25–75) days in patients with infection versus 5 (2–10, IQR 25–75; p: <0.001) days in patients without infection and total hospital stay was 26 (18–33, IQR 25–75) days in patients with infection versus 8 (2–23, IQR 25–75) days in those without infection. A total of 24 patients out of the 70 patients experienced 32 infectious episodes during extracorporeal membrane oxygenation support. Culture-positive infections were detected at a single site in 19 patients, and multiple sites in 5 patients.

We propose that prolonged extracorporeal membrane oxygenation support is associated with an increased risk of infection. Although extracorporeal membrane oxygenation is a life-saving treatment method, prolonged extracorporeal membrane oxygenation may increase the development of infectious complications and the associated mortality and morbidity of the patient.