Our aim was to present the initial experience with a protocol-driven early extubation strategy and to identify risk factors associated with failed spontaneous breathing trials within 12 hours after surgery.

A single institutional retrospective study of children up to 18 years of age was conducted in post-operative cardiac surgical patients over a 1-year period. A daily spontaneous breathing trial protocol was used to assess patients’ readiness for extubation. The study population (n = 129) was stratified into two age groups: infants (n = 84) and children (n = 45), and further stratified according to ventilation time: early extubation (ventilation time less than 12 h, n = 86) and deferred extubation (ventilation time more than 12 h, n = 43). Mann–Whitney U-test and binomial logistic regression were used for statistical analysis.

Early extubated infants had shorter ICU (4 versus 6 days, p = 0.003) and hospital length of stays (16 versus 19 days, p = 0.006), lower re-intubation rates (1 versus 7 patients, p = 0.003), and lower mortality (0 versus. 4 patients, p = 0.01) than deferred extubated infants. There was no significant difference in the studied outcomes in the children group. Malnourished infants and longer cardiopulmonary bypass times were independently associated with failed spontaneous breathing trials within 12 hours after cardiac surgery.

Early extubated infants after cardiac surgery had shorter ICU and hospital length of stay, without an increase in morbidity and mortality, compared to infants who deferred extubation. Nutritional status and longer cardiopulmonary bypass times were risk factors for failed spontaneous breathing trial.

Vascular compression of the airway often complicates CHD management. This study evaluated the use of CT in determining cardiovascular causes, clinical manifestations, and outcome of tracheobronchial compression among children with CHD.

A retrospective review of clinical records of all patients with CT scan evidence of tracheobronchial compression from January 2007 to December 2017 at National Heart Institute. Cardiovascular causes of tracheobronchial compression were divided into three groups; group I: vascular ring/pulmonary artery sling, II: abnormally enlarged or malposition cardiovascular structure due to CHD, III: post-CHD surgery.

Vascular tracheobronchial compression was found in 81 out of 810 (10%) patients who underwent CT scan. Group I lesions were the leading causes of vascular tracheobronchial compression (55.5%), followed by group II (34.6%) and group III (9.9%). The median age of diagnosis in groups I, II, and III were 16.8 months, 3 months, and 15.6 months, respectively. Half of group I patients are manifested with stridor and one-third with recurrent chest infections. Persistent respiratory symptoms, lung atelectasis, or prolonged respiratory support requirement were clues in groups II and III. Higher morbidity and mortality in younger infants with severe obstructive airway symptoms, associated airway abnormalities, and underlying complex cyanotic CHD.

Vascular ring/pulmonary artery sling and abnormally enlarged or malposition cardiovascular structure were the leading causes of cardiovascular airway compression. A high index of suspicion is needed for early detection due to its non-specific presentation. The outcome often depends on the severity of airway obstruction and complexity of cardiac lesions.

Parents of infants with CHDs experience increased parenting stress compared to the general population, potentially interfering with parenting practices and bear adverse family outcomes. The changes in stress over the critical period of infancy have yet to be studied. The current study aimed to compare parenting stress changes over time between parents of infants with CHDs and parents of healthy infants during the first year of infants’ life.

Data from a larger prospective cohort study were longitudinally analysed using mixed-effects multivariable regression modelling. Sample included mothers of 129 infants with complex cardiac defects and healthy infants, recruited from the cardiac ICU of a large cardiac centre and outpatient paediatric practices in Northeastern America. Outcome was measured over four visits via the Parenting Stress Index Long Form.

Stress in the cardiac group has significantly decreased over time on the Parent Domain (p = 0.025), and stress in the healthy group has significantly increased over time on the Child Domain (p = 0.033). Parenting stress trajectories demonstrated significant differences between groups on the Parent Domain (p = 0.026) and on the Total Stress (p = 0.039) subscales.

Parenting stress in the paediatric cardiac population changes over time and differs from stress experienced by parents of healthy infants. Findings highlight stressful periods that may be potentially risky for parents of infants with CHDs and introduce additional illness-related and psychosocial/familial aspects to the parenting stress concept.

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

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

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

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

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

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

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

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

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.

Glenn procedure is performed for patients with cyanotic CHD and univentricular physiology and has a survival rate above 90%.

To evaluate the risk factors associated with a poor outcome after Glenn procedure.

The data for this retrospective analysis were collected from a regional Brazilian registry of congenital heart surgeries (ASSIST initiative) from 2014 to 2019. Data from 97 patients who underwent the Glenn procedure were considered. The primary outcomes were prolonged mechanical ventilation (>24 hours post-operatively) or in-hospital death, alone and combined.

The overall in-hospital mortality was 13.4% (n = 13). Prolonged mechanical ventilation occurred in 52% (n = 51) of the patients. Our analysis found that risk factors for death or prolonged post-operative mechanical ventilation were cardiopulmonary bypass duration and post-operative lactate, whereas weight-for-age z-score and age at surgery were risk factors for prolonged mechanical ventilation. Cardiopulmonary bypass duration increased (adjusted odds ratio: 1.02; 95 % CI: 1.01, 1.03) and age at surgery decreased (adjusted odds ratio: 0.96; 95 % CI: 0.93, 0.99) the odds of the combined outcome.

Age at surgery, post-operative lactate, and low-weight-for-age z-score are associated with prolonged mechanical ventilation and death following the Glenn procedure.

When encountering adolescents with chest pain and a high troponin level but with no underlying coronary artery illness, it is advisable to consider myopericarditis. Though myopericarditis is a self-limiting, benign condition, it nevertheless causes anxiety in the patient and the family.

Thirty-nine patients diagnosed with myopericarditis were included. We retrospectively analysed the demographic and clinical features, laboratory tests, echocardiography, electrocardiograms, MRI findings, coronary CT angiography, and conventional angiography findings in these patients.

Of the 39 patients (female/male = 4/35) aged 7–17 years, 66.6% had viral infection in the 2 weeks preceding presentation. Eleven patients were tested for high-sensitivity cardiac troponin I, 28 for high-sensitivity cardiac troponin T, and 10 patients were tested for both biomarkers. The median hs-TnI and hs-TnT values were 6.3 (0.05–29.9) ng/mL and 586 (51–9398) ng/L, respectively. Twenty-three patients showed ST changes on electrocardiography, of whom 11 had ST-elevation in the leads supporting left ventricular involvement. Coronary CT angiography and catheter angiography evaluations performed for differential diagnosis of coronary anomaly and acute coronary syndrome were normal. Cardiac MRI was conducted on 28 patients, and the results in 10 (35.7%) were suggestive of myopericarditis.

Myopericarditis is common in the adolescent age group and is generally benign but should be carefully monitored for differential diagnosis and possible complications. Cardiac MRI, which has been used more frequently in recent years, has an important role in differential diagnosis and the follow-up of patients.

Approximate Entropy is an extensively enforced metric to evaluate chaotic responses and irregularities of RR intervals sourced from an eletrocardiogram. However, to estimate their responses, it has one major problem – the accurate determination of tolerances and embedding dimensions. So, we aimed to overt this potential hazard by calculating numerous alternatives to detect their optimality in malnourished children.

We evaluated 70 subjects split equally: malnourished children and controls. To estimate autonomic modulation, the heart rate was measured lacking any physical, sensory or pharmacologic stimuli. In the time series attained, Approximate Entropy was computed for tolerance (0.1→0.5 in intervals of 0.1) and embedding dimension (1→5 in intervals of 1) and the statistical significances between the groups by their Cohen’s ds and Hedges’s gs were totalled.

The uppermost value of statistical significance accomplished for the effect sizes for any of the combinations was −0.2897 (Cohen’s ds) and −0.2865 (Hedges’s gs). This was achieved with embedding dimension = 5 and tolerance = 0.3.

Approximate Entropy was able to identify a reduction in chaotic response via malnourished children. The best values of embedding dimension and tolerance of the Approximate Entropy to identify malnourished children were, respectively, embedding dimension = 5 and embedding tolerance = 0.3. Nevertheless, Approximate Entropy is still an unreliable mathematical marker to regulate this.

Accessory pathways are commonly seen due to delamination of tricuspid valve leaflets. In addition to accessory pathways, an enlarged right atrium due to tricuspid regurgitation and incisional scars creates substrates for atrial re-entries and ectopic tachycardia. We sought to describe our experience with catheter ablation in children with Ebstein’s anomaly.

During the study period, of 89 patients diagnosed with Ebstein’s anomaly, 26 (30.9%) of them who underwent 33 ablation procedures were included in the study. Accessory pathways were observed in the majority of procedures (n = 27), whereas atrial flutter was observed in five, atrioventricular nodal reentrant tachycardia in five, and atrial tachycardia in two procedures. Accessory pathways were commonly localised in the right posteroseptal (n = 10 patients), right posterolateral (n = 14 patients), septal (n = two patients), and left posteroseptal (n = one patient) areas. Multiple accessory pathways and coexistent arrhythmia were observed in six procedures. All ablation attempts related to the accessory pathways were successful, but recurrence was observed in five (19%) of the ablations. Ablation for atrial flutter was performed in five patients; two of them were ablated successfully. One of the atrial tachycardia cases was ablated successfully.

Ablation in patients with Ebstein’s anomaly is challenging, and due to nature of the disease, it is not a rare occasion in this group of patients. Ablation of accessory pathways has high success, but also relatively high recurrence rates, whereas ablation of atrial arrhythmias has lower success rates, especially in operated patients.

Intracardiac echocardiography Doppler-derived gradients have previously been shown to correlate with post-procedure echocardiographic evaluations when compared with invasive gradients measured during percutaneous pulmonary valve implantation, suggesting that intracardiac echocardiography could offer an accurate and predictable starting point to estimate valve function after percutaneous pulmonary valve implantation.

We performed a retrospective chart review of 51 patients who underwent percutaneous pulmonary valve implantation between September 2018 and December 2019 in whom intracardiac echocardiography was performed immediately after valve implantation. We evaluated the correlation between intracardiac echocardiography gradients and post-procedural Doppler-derived gradients. Among the parameters assessed, those which demonstrated the strongest correlation were used to create a predictive model of expected echo-derived gradients after percutaneous pulmonary valve implantation. The equation was validated on the same sample data along with a subsequent cohort of 25 consecutive patients collected between January 2020 and July 2020.

All the assessed correlation models between intracardiac echocardiography evaluation and post-procedure transthoracic echocardiographic assessments were statistically significant, presenting moderate to strong correlations. The strongest relationship was found between intracardiac echocardiography mean gradients and post-procedural transthoracic echocardiographic mean gradients. Therefore, an equation was created based on the intracardiac echocardiography-derived mean gradient, to allow prediction of the post-procedural and follow-up transthoracic echocardiographic-derived mean gradients within a range of ±5 mmHg from the observed value in more than 80% of cases.

There is a strong correlation between intracardiac echocardiography and post-procedure transthoracic echocardiographic. This allowed us to derive a predictive equation that defines the expected transthoracic echocardiographic Doppler-derived gradient following the procedure and at out-patient follow-up after percutaneous pulmonary valve implantation.

We present our experience and outcomes with the BeGraft in the treatment of aortic coarctation in a predominantly paediatric population.

This study includes a retrospective analysis of patients who had Begraft aortic stent implantation between 2018 and 2020 from a single centre.

The BeGraft aortic stent was used in 11 patients (7 males, 4 females) with a median age of 14 (13–21) years and a median weight of 65 (46–103) kg. Coarctation was native in five patients and recurrent in six patients. Median stent diameter and length were 16 mm and 38 mm, respectively. The median peak-to-peak pressure was 30 (12–55) mmHg before the procedure and 5 (0–17) mmHg after the procedure. The stenting procedure was successful in 10 of the 11 patients. Stent migration to the abdominal aorta occurred on post-procedure day 1 in the 21-year-old patient, who had previously undergone surgical closure of the ventricular septal defect and balloon angioplasty for coarctation. After repositioning failed, the stent was safely fixed in the abdominal aorta. Strut distortion also occurred during balloon retrieval in one patient, but no aneurysm or in-stent restenosis was observed at 1-year follow-up. The patients were followed for a median of 14 (4–25) months and none required redilation.

Our initial results demonstrated that the BeGraft aortic stent effectively reduced the pressure gradient in selected native and recurrent cases. Despite advantages such as a smaller sheath and low profile, more experience and medium- to long-term results are needed.

Although coronary artery bypass grafting is not frequently performed in children, Kawasaki disease is one of the most common indications for coronary artery bypass grafting in children. Here, we reviewed the long-term clinical outcomes including graft patency after coronary artery bypass grafting.

Between March 2004 and March 2013, six patients with Kawasaki disease underwent coronary artery bypass grafting. All patients were male. Their median age was 13.0 years (interquartile range, 7.8–17.8 years) at the timing of coronary artery bypass grafting, and the median age at the onset of Kawasaki disease was 3.3 years (interquartile range, 1.0–7.0 years). Four patients presented with multiple lesions including aneurysms.

The median follow-up duration was 12.1 years (interquartile range, 9.5–13.1 years), and there were no operative complications or overall mortality. One patient had pre-operative symptoms such as exertional chest pain and dyspnoea on exertion, whereas one patient had ventricular tachyarrhythmia. There was an improvement in subjective symptoms after surgery in two patients. The left internal thoracic artery, right internal thoracic artery, and saphenous vein were used in five (83.3%), one (16.7%), and two (33.3%) cases, respectively. In all six patients, post-operative single-photon emission CT findings showed improved perfusion compared with pre-operative single-photon emission CT. All grafts were patent as confirmed by coronary angiography or CT angiography.

Coronary artery bypass grafting could be a good surgical option in children with coronary lesions caused by Kawasaki disease in terms of graft patency and myocardial perfusion.

Caring for infants after the first-stage palliative surgery for single-ventricle heart disease bring challenges beyond the usual parenting responsibilities. Current studies fail to capture the nuances of caregivers’ experiences during the most critical “interstage” period between the first and second surgery.

To explore the perceptions of caregivers about their experiences while transitioning to caregiver roles, including the successes and challenges associated with caregiving during the interstage period.

Constructivist Grounded Theory methodology guided the collection and analysis of data from in person or telephonic interviews with caregivers after their infants underwent the first-stage palliative surgery for single-ventricle heart disease, and were sent to home for 2–4 months before returning for their second surgery. Symbolic interactionism informed data analyses and interpretation.

Our sample included 14 parents, who were interviewed 1–2 times between November, 2019 and July, 2020. Most patients were mothers (71%), Latinx (64%), with household incomes <$30K (42%). Data analysis led to the development of a Grounded Theory called Developing a Sense of Self-Reliance with three categories: (1) Owning caregiving responsibilities despite grave fears, (2) Figuring out how “to make it work” in the interstage period, and (3) Gaining a sense of self-reliance.

Parents transitioned to caregiver roles by developing a sense of self-reliance and, in the process, gained self-confidence and decision-making skills. Our study responded to the key research priority from the AHA Scientific Statement to address the knowledge gap in home monitoring for interstage infants through qualitative research design.

We aimed to evaluate the efficacy and role of high-sensitivity troponin T in children with a confirmed SARS-CoV-2 infection and also the correlation of troponin T levels with symptoms, and echocardiographic findings were analysed.

Two hundred and fourteen patients with a confirmed SARS-CoV-2 infection between the dates of 28 March and 15 August 2020 were enrolled in this retrospective single-centre study. Patients with comorbidities and diagnosed as multisystem inflammatory syndrome in children were excluded. Demographic data, clinical and laboratory parameters were evaluated. The patients were classified and compared according to the troponin positivity. The correlation of troponin T with symptoms and echocardiographic findings was analysed.

The most common symptoms in the whole study group were fever (53.3%) and cough (24.8%). Troponin T levels were elevated in 15 (7%) patients. The most common symptom in patients with troponin positivity was also fever (73.3%). Troponin T positivity was significantly higher in patients under the age of 12 months and troponin T levels were negatively correlated with age. C-reactive protein levels were elevated in 77 (36%) of the patients in the whole group and 7 (46.7%) of 15 patients with troponin positivity. C-reactive protein levels were similar between groups.

Routine troponin screening does not yield much information in previously healthy paediatric COVID-19 patients without any sign of myocardial dysfunction. Elevated troponin levels may be observed but it is mostly a sign of myocardial injury without detectable myocardial dysfunction in this group of patients.