To characterise the parenting priorities of mothers and fathers of infants hospitalised with CHD and generate recommendations to support parenting during infant hospitalisation.

Through online crowdsourcing, an innovative research methodology to create an online community to serve as a research sample, 79 parents of young children with CHD responded to questions about parenting during hospitalisation via private social networking site. Responses were analysed using qualitative research methods.

Three broad themes were identified: (1) establishing a bond with my baby, (2) asserting the parental role, and (3) coping with fear and uncertainty. Parents value provider support in restoring normalcy to the parenting experience during infant hospitalisation.

Care teams can support parenting during infant hospitalisation by promoting parents’ roles as primary caretakers and decision-makers and attending to the emotional impact of infant hospitalisation on the family.

Non-cardiac chest pain is a common and persistent problem for children; yet, typically, there is no clear medical cause. To date, no behavioural and/or psychological factors have been studied to explain chest pain in a pre-school paediatric sample. We hypothesized that pre-school children with medically unexplained chest pain would have higher rates of behavioural problems compared to healthy controls.

We assessed 41 pre-school children with non-cardiac chest pain and 68 age matched children with benign heart murmurs as the control group using the Child Behaviour Check List-1 1/2–5 to evaluate emotional and behavioural problems.

Internalizing problem scores comprising emotionally reactive, anxiety/depression, and somatic complaints were higher in children with non-cardiac chest pain than in the control group. Among the possible factors, the factor that is related to behaviour problem scores, in univariate analysis, was a significant and inverse correlation between maternal education and behaviour problem scores. Also, maternal employment status was associated with behavioural problems. Children with a housewife mother were more susceptible to having such behavioural problems. Based on multiple regression analyses, being in the non-cardiac chest pain group was found to be significantly related to internalizing problems in our total sample.

These results suggest that pre-school children with non-cardiac chest pain may experience increased levels of certain behavioural comorbidities. Systematic behavioural screening could increase the detection of behavioural problems and improve care for this population. Future studies of non-cardiac chest pain in pre-school children should include larger samples and comprehensive diagnostic assessments as well as long-term follow-up evaluations.

The standard transthoracic echocardiography has some limitations in emergent and community-based situations. The emergence of pocket-sized ultrasound has led to influential advancements.

In this prospective study, in the hospital-based phase, children with suspected structural heart diseases were enrolled. In the school-based phase, healthy children were randomly selected from six schools. All individuals were examined by experienced operators using both the standard and the pocket-sized echocardiography.

A total of 73 individuals with a mean age of 9.9 ± 3.2 years in the hospital-based cohort and 143 individuals with a mean age of 12.8 ± 2.9 years in the school-based cohort were examined. The agreements between the standard and the pocket-sized echocardiography were good or excellent for major CHDs in both cohorts (κ statistics > 0.61). Among valvular pathologies, agreements for tricuspid and pulmonary valves’ regurgitation were moderate among school-based cohorts (0.56 [95% confidence interval 0.12–1] and 0.6 [95% confidence interval 0.28–0.91], respectively). The agreements for tricuspid and pulmonary valves’ regurgitation were excellent (>0.9) among hospital-based population. Other values for valvular findings were good or excellent. The overall sensitivity and specificity were 87.5% (95% confidence interval 47.3–99.7) and 93.8% (95% confidence interval 85–98.3) among the hospital-based individuals, respectively, and those were 88% (95% confidence interval 77.8–94.7) and 68.4% (95% confidence interval 56.7–78.6) among the school-based individuals, respectively. The cost of examination was reduced by approximately 70% for an individual using the pocket-sized device.

When interpreted by experienced operators, the pocket-sized echocardiography can be used as screening tool among school-aged population.

To determine the Final ICU Need in the 24 hours prior to ICU discharge for children with cardiac disease by utilising a single-centre survey.

A cross-sectional survey was utilised to determine Final ICU Need, which was categorised as “Cardiovascular”, “Respiratory”, “Feeding”, “Sedation”, “Systems Issue”, or “Other” for each encounter. Survey responses were obtained from attending physicians who discharged children (≤18 years of age with ICU length of stay >24 hours) from the Cardiac ICU between April 2016 and July 2018.

Survey response rate was 99% (n = 1073), with 667 encounters eligible for analysis. “Cardiovascular” (61%) and “Respiratory” (26%) were the most frequently chosen Final ICU Needs. From a multivariable mixed effects logistic regression model fitted to “Cardiovascular” and “Respiratory”, operations with significantly reduced odds of having “Cardiovascular” Final ICU Need included Glenn palliation (p = 0.003), total anomalous pulmonary venous connection repair (p = 0.024), truncus arteriosus repair (p = 0.044), and vascular ring repair (p < 0.001). Short lengths of stay (<7.9 days) had significantly higher odds of “Cardiovascular” Final ICU Need (p < 0.001). “Cardiovascular” and “Respiratory” Final ICU Needs were also associated with provider and ICU discharge season.

Final ICU Need is a novel metric to identify variations in Cardiac ICU utilisation and clinical trajectories. Final ICU Need was significantly influenced by benchmark operation, length of stay, provider, and season. Future applications of Final ICU Need include targeting quality and research initiatives, calibrating provider and family expectations, and identifying provider-level variability in care processes and mental models.

Norwood surgery has been available in Sweden since 1993. In this national cohort study, we analysed transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia.

Patients were identified from the complete national cohort of live-born with hypoplastic left heart syndrome/aortic atresia 1993–2010. Analysis of survival after surgery was performed using Cox proportional hazards models for the total cohort and for birth period and gender separately. Thirty-day mortality and inter-stage mortality were analysed. Patients were followed until September 2016.

The 1993–2010 cohort consisted of 208 live-born infants. Norwood surgery was performed in 121/208 (58%). The overall transplantation-free survival was 61/121 (50%). The survival was higher in the late period (10-year survival 63%) than in the early period (10-year survival 40%) (p = 0.010) and lower for female (10-year survival 34%) than for male patients (10-year survival 59%) (p = 0.002). Inter-stage mortality between stages I and II decreased from 23 to 8% (p = 0.008). For male patients, low birthweight in relation to gestational age was a factor associated with poor outcome.

The survival after Norwood surgery for hypoplastic left heart syndrome/aortic atresia improved by era of surgery, mainly explained by improved survival between stages I and II. Female gender was a significant risk factor for death or transplantation. For male patients, there was an increased risk of death when birthweight was lower than expected in relation to gestational age.

Pulmonary vascular disease resulting from CHDs may be the most preventable cause of pulmonary artery hypertension worldwide. Many children in developing countries still do not have access to early closure of clinically significant defects, and the long-term outcomes after corrective surgery remain unclear. Focused on long-term results after isolated ventricular septal defect repair, our review sought to determine the most effective medical therapy for the pre-operative management of elevated left-to-right shunts in patients with an isolated ventricular septal defect.

We identified articles specific to the surgical repair of isolated ventricular septal defects. Specific parameters included the pathophysiology and pre-operative medical management of pulmonary over-circulation and outcomes.

Studies most commonly focused on histologic changes to the pulmonary vasculature and levels of thromboxanes, prostaglandins, nitric oxide, endothelin, and matrix metalloproteinases. Only 2/44 studies mentioned targeted pharmacologic management to any of these systems related to ventricular septal defect repair; no study offered evidence-based guidelines to manage pulmonary over-circulation with ventricular septal defects. Most studies with long-term data indicated a measurable frequency of pulmonary artery hypertension or diminished exercise capacity late after ventricular septal defect repair.

Long-term pulmonary vascular and respiratory changes can occur in children after ventricular septal defect repair. Research should be directed at providing an evidenced-based approach to the medical management of infants and children with ventricular septal defects (and naturally all CHDs) to minimise consequences of pulmonary artery hypertension, particularly as defect repair may occur late in underprivileged societies.

Obesity is usually related to insulin resistance and glucose metabolism disorders. The relationship between insulin resistance and epicardial adipose tissue and atrial electromechanical delay has been described in previous studies.

This study aims to demonstrate the effects of metformin on epicardial adipose tissue and electromechanical delay in patients using metformin for insulin resistance.

A total of 30 patients using metformin for insulin resistance were included in the study. Pre-treatment and post-treatment epicardial adipose tissue and electromechanical delay were evaluated.

There was a statistically significant decrease in epicardial adipose tissue thickness after 3 months of metformin therapy (6.4 ± 2.1 versus 4.7 ± 2.0; p = 0.008). Furthermore, the inter-atrial and intra-atrial electromechanical delay also significantly decreased after 3 months of metformin monotherapy (23.6 ± 8.2 versus 18.1 ± 5.8; p < 0.001, 9.1 ± 2.9 versus 6.3 ± 3.6; p = 0.003, respectively).

In this study, we show that metformin monotherapy significantly decreases epicardial adipose tissue thickness and electromechanical delay in obese children.

Focal atrial tachycardia accounts for up to 10–15% of supraventricular tachycardiasubstrates in patients < 30 years. In this study, we aimed to demonstrate the outcome of transcatheter ablation procedures performed through three-dimensional electroanatomic mapping systems using minimal fluoroscopy in a paediatric cohort with focal atrial tachycardia.

Forty-nine consecutive patients with focal atrial tachycardia who underwent an electrophysiologic study and a transcatheter ablation procedure in our hospital from September 2014 to February 2020 were included into the study.

The mean weight of the patients was 48.63 ± 15.4 kg, and the mean age was 14.56 ± 3.5 (5.5–18.4) years. The tachycardia was defined as incessant in 26 patients. Thirteen patients had left ventricular systolic dysfunction with a mean left ventricular ejection fraction of 38.47 ± 12.4% on echocardiography. The mean procedure time was 148.7 ± 94.5 minutes. Transseptal puncture and thus fluoroscopy were required in nine patients. The mean fluoroscopy time was 4.51 ± 5.9 minutes. No fluoroscopy was needed in ablations performed in the right atrium. The acute success rate of the ablation procedures was 97.9%. The mean follow-up period was 50.71 ± 23.5 months. Recurrence was noted in two patients (4.2%).

The outcomes of three-dimensional electroanatomic mapping-guided transcatheter ablation procedures are promising with high acute success, low recurrence and complication rates in children with focal atrial tachycardia. The use of fluoroscopy can be significantly decreased with three-dimensional mapping systems in this group of patients.

Ventilation-associated changes in blood carbon dioxide levels are associated with various physiological changes in infants undergoing surgery. Studies on the effects of mechanical ventilation on cerebral haemodynamics especially for infants with CHD are scarce.

This study was done to compare the changes in regional cerebral oxygen saturation and cerebral blood flow velocity when the end-tidal carbon dioxide partial pressure changed during different minute ventilation settings in infants undergoing ventricular septal defect repair.

A total of 67 patients less than 1 year old with ventricular septal defect were enrolled, and 65 patients (age: 6.7 ± 3.4 months, weight: 6.4 ± 1.5 kg) were studied. After anaesthesia induction and endotracheal intubation, the same mechanical ventilation mode (The fraction of inspired oxygen was 50%, and the inspiratory-to-expiratory ratio was 1:1.5.) was adopted. The end-tidal carbon dioxide partial pressure of 30 mmHg (T1), 35 mmHg (T2), 40 mmHg (T3), or 45 mmHg (T4) were obtained, respectively, by adjusting tidal volume and respiratory rate. Minute ventilation per kilogram was calculated by the formula: minute ventilation per kilogram = tidal volume * respiratory rate/kg. Regional cerebral oxygen saturation was monitored by real-time near-infrared spectroscopy. Cerebral blood flow velocity (systolic flow velocity, end-diastolic flow velocity, and mean flow velocity), pulsatility index, and resistance index were measured intermittently by transcranial Doppler. Systolic pressure, diastolic pressure, stroke volume index, and cardiac index were recorded using the pressure recording analytical method.

As the end-tidal carbon dioxide partial pressure increased from 30 to 45 mmHg, regional cerebral oxygen saturation increased significantly from 69 ± 5% to 79 ± 4% (p < 0.001). Cerebral blood flow velocity (systolic flow velocity, end-diastolic flow velocity, and mean flow velocity) increased linearly, while pulsatility index and resistance index decreased linearly from T1 (systolic flow velocity, 84 ± 19 cm/second; end-diastolic flow velocity, 14 ± 4 cm/second; mean flow velocity, 36 ± 10 cm/second; pulsatility index, 2.13 ± 0.59; resistance index, 0.84 ± 0.12) to T4 (systolic flow velocity, 113 ± 22 cm/second; end-diastolic flow velocity, 31 ± 6 cm/second; mean flow velocity, 58 ± 11 cm/second; pulsatility index, 1.44 ± 0.34; resistance index, 0.72 ± 0.07) (p < 0.001). There were significant differences in changes of systolic flow velocity, end-diastolic flow velocity, mean flow velocity, pulsatility index, and resistance index as the end-tidal carbon dioxide partial pressure increased from 30 to 45 mmHg between subgroups of infants ≤6 and infants >6 months, while the changes of regional cerebral oxygen saturation between subgroups were not statistically different. Regional cerebral oxygen saturation and cerebral blood flow velocity (systolic flow velocity, end-diastolic flow velocity, and mean flow velocity) were negatively correlated with minute ventilation per kilogram (r = −0.538, r = −0.379, r = −0.504, r = −0.505, p < 0.001). Pulsatility index and resistance index were positively related to minute ventilation per kilogram (r = 0.464, r = 0.439, p < 0.001). The diastolic pressure was significantly reduced from T1 (41 ± 7 mmHg) to T4 (37 ± 6 mmHg) (p < 0.001). There were no significant differences in systolic pressure, stroke volume index, and cardiac index with the change of end-tidal carbon dioxide partial pressure from T1 to T4 (p = 0.063, p = 0.382, p = 0.165, p > 0.05).

A relative low minute ventilation strategy increases regional cerebral oxygen saturation and cerebral blood flow, which may improve cerebral oxygenation and brain perfusion in infants undergoing ventricular septal defect repair.

Post-operative severe vascular stenosis and proliferating endothelial tissue lead to severe circulatory disorders and impair organ perfusion. Bioabsorbable magnesium scaffolds may help to overcome these obstructions without leaving obstructing stent material. We analyse their role in the treatment of vascular stenosis in infants.

Since 2016, 15 magnesium scaffolds with a diameter of 3.5 mm were implanted in 9 patients aged 15 days to 7.6 years. Eight scaffolds were implanted in pulmonary venous restenoses, five in pulmonary arterial stenosis including one in-stent stenosis, one into a stenotic brachiocephalic artery, and one in a recurrent innominate vein thrombosis.

All patients clinically improved after the implantation of a scaffold. The magnesium scaffolds lost integrity after 30–48 days (mean 42 days). The innominate vein thrombosed early, while all other vessels remained open. Two patients died after 1.3 and 14 weeks not related to the scaffolds. Five patients needed further balloon dilations or stent implantations after the scaffold had fractured. At first recatheterisation after in mean 2.5 months, the mean minimum/maximum diameter in relation to the scaffold’s original diameter was 89%/99% in the arterial implantations (n = 6) and 66%/77% in the pulmonary venous implantations.

The magnesium scaffolds can be used as a bridging solution to treat severe vascular stenosis in different locations. Restenosis can occur after degradation and make further interventions necessary, but neither vessel growth nor further interventions are hindered by stent material. Larger diameters may improve therapeutic options.

Management strategies for pulmonary atresia with intact ventricular septum are variable and are based on right ventricular morphology and associated abnormalities. Catheter perforation of the pulmonary valve provides an alternative strategy to surgery in the neonatal period. We sought to assess the long-term outcome in terms of survival, re-intervention, and functional ventricular outcome in the setting of a 26-year single-centre experience of low threshold inclusion criteria for percutaneous valvotomy.

Retrospective analysis of patients diagnosed with pulmonary atresia with intact ventricular septum from 1990 to 2016 at a tertiary referral centre, was performed. Of 71 patients, 48 were brought to the catheterisation laboratory for intervention. Catheter valvotomy was successful in 45 patients (94%). Twenty-three patients (51%) also underwent ductus arteriosus stenting. The length of intensive care and hospital stay was significantly shorter, and early re-interventions were significantly reduced in the catheterisation group. There were eight deaths (17%); all within 35 days of the procedure. Of the survivors, only one has required a Fontan circulation. Twenty-eight patients (74%) have undergone biventricular repair and nine patients (24%) have one-and-a-half ventricle circulation. Following successful valvotomy, 80% of patients required further catheter-based or surgical interventions.

A low threshold for initial interventional management yielded a high rate of successful biventricular circulations. Although mortality was low in patients who survived the peri-procedural period, the rate of re-intervention remained high in all groups.

We aim to assess the diagnostic role of QRS fragmentation in children with suspected acute myocarditis.

Diagnosis of myocarditis in the paediatric population is challenging. Clinical suspicion, electrocardiogram, and laboratory tests are the main diagnostic features at presentation. However, electrocardiogram in patients with myocarditis is usually considered aspecific. We have previously described QRS fragmentation in adult patients with acute myocarditis.

Patients aged less than 18 years, admitted between 2003 and 2019, and discharged with a diagnosis of acute myocarditis were included. Standard electrocardiogram, laboratory, and echocardiographic findings at admission and follow-up were reviewed. QRS fragmentation was defined by the presence of multiphasic R′ spikes. Cardiac magnetic resonance and biopsy were performed in selected patients.

Twenty-one patients were analysed, 16 males (76%), median age 9.5 (2.5–16) years. At presentation, 12 patients (57%) displayed QRS fragmentation. Median ejection fraction was 40% (27–60). Nine patients (43%) underwent cardiac magnetic resonance and displayed late gadolinium enhancement. One patient underwent biopsy that showed borderline findings. Electrocardiogram leads showing QRS fragmentation correlated with distribution of late gadolinium enhancement. Median follow-up was 600 (190–2343) days. All patients were alive at last follow-up. Six patients (33%) patients displayed persistence of QRS fragmentation. Median ejection fraction was 60% (60–65%). In three patients (14%), ejection fraction remained depressed, two of which showed persistence of QRS fragmentation.

In this cohort of children with suspected myocarditis, QRS fragmentation was confirmed as a new additional diagnostic finding to look for at admission and during follow-up.

N-terminal pro-B-type natriuretic peptide (NT-proBNP), a well-known marker of cardiac disease, may be elevated in acute infections and other non-cardiac diseases. The aim of this study was to evaluate NT-proBNP levels in cardiac and non-cardiac diseases and found out the patient’s clinical status that affects the NT-proBNP levels.

NT-proBNP levels were measured in three groups of children: a cardiac disease group, an infectious disease group, and a non-cardiac, non-infectious disease group.

In total, 348 children were enrolled, including 134 patients (38.5%) with cardiac disease, 170 patients (48.9%) with infectious disease, and 44 patients (12.6%) with non-cardiac, non-infectious disease. The NT-proBNP level of the cardiac disease group (median: 548 pg/mL; range: 5–35,000 pg/mL) was significantly higher than that of the infectious disease group (median: 193 pg/mL; range: 10–35,000 pg/mL) and the non-cardiac, non-infectious disease group (median: 280 pg/mL; range: 6–35,000 pg/mL). Regarding clinical status, the NT-proBNP levels were significantly higher in patients who needed mechanical ventilation support, oxygen therapy, or inotropic medication or had a change in mental status than in other patients. However, the systemic inflammatory response syndrome and mortality were not related to the NT-proBNP level.

Among heterogeneous group of children, NT-proBNP level can be a useful marker of cardiac disease. Furthermore, the NT-proBNP levels were related to patients’ clinical deteriorations, such as shock rather than the inflammatory status of patients.

While the overall prevalence of autism is 1.7% in the United States of America, research has demonstrated a two- to five-fold increase in CHD. The Cardiac Neurodevelopmental Outcome Collaborative recommends screening for autism from infancy through adolescence. This study investigated the frequency of autism concerns at a single Cardiac Neurodevelopmental Program and examined current clinical practice as a way to improve quality of care.

Patients (n = 134; mean age = 9.0 years) included children with high-risk CHD who completed a neurodevelopmental evaluation following a formalised referral to the Cardiac Neurodevelopmental Program between 2018 and 2019. Retrospective chart review included parent report on the Behaviour Assessment System for Children-3 and Adaptive Behaviour Assessment System-3. Descriptive and correlation analyses were completed.

In this sample, 11.2% presented with autism-related concerns at referral, 2 were diagnosed with autism, 9 were referred to an autism specialist (6 confirmed diagnosis; 3 not completed). Thus, at least 5.9% of the sample were diagnosed with autism following thorough clinical evaluation. Analyses showed atypicality, along with deficient adaptability, leisure, social, and communication skills. Frequency of early intervention, school supports, and relation with comorbidities are reported.

Prior to assessment recommendations by the Cardiac Neurodevelopmental Outcome Collaborative, autism screening may not be completed systematically in clinical care for CHD. The current sample demonstrates a high frequency of autism in the typically referred clinical sample. Commonly used parent-report measures may reveal concerns but will not help diagnosis. Systematic use of an autism screener is essential.