Giant coronary artery aneurysms and myocardial fibrosis after Kawasaki disease may lead to devastating cardiovascular outcomes. We characterised the vascular and myocardial outcomes in five selected Kawasaki disease patients with a history of giant coronary artery aneurysms that completely regressed.

Five patients were selected who had giant coronary artery aneurysm in early childhood that regressed when studied 12–33 years after Kawasaki disease onset. Coronary arteries were imaged by coronary CT angiography, and coronary artery calcium volume scores were determined. We used endocardial strain measurements from CT imaging to assess myocardial regional wall function. Calprotectin and galectin-3 (gal-3) as biomarkers of inflammation and myocardial fibrosis were measured by enzyme-linked immunosorbent assay.

The five selected patients with regressed giant coronary artery aneurysms had calcium scores of zero, normal levels of calprotectin and gal-3, and normal appearance of the coronary arteries by coronary computed tomography angiography. CT strain demonstrated normal peak systolic and diastolic strain patterns in four of five patients. In one patient with a myocardial infarction at the time of Kawasaki disease diagnosis at the age of 10 months, CT strain showed altered global longitudinal strain, reduced segmental peak strain, and reduced diastolic relaxation patterns in multiple left ventricle segments.

These patients illustrate that regression of giant aneurysms after Kawasaki disease is possible with no detectable calcium, normal biomarkers of inflammation and fibrosis, and normal myocardial function. Individuals with regressed giant coronary artery aneurysm still require longitudinal surveillance to assess the durability of this favourable outcome.

To assess whether isolated very low QRS voltage of ≤0.3 mV in the frontal leads might be a marker for diagnosing paediatric vasovagal syncope and the risk of recurrence.

We included 118 children with vasovagal syncope, comprising 70 males and 48 females in our retrospective analysis. All patients underwent head-up tilt test and supine 12-lead electrocardiography. Furthermore, the QRS voltage was measured from each one of the 12 leads on basal electrocardiography. Patients were followed up for 6–24 months (average, 16 months).

Eighty-six patients (73%) patients displayed isolated very low QRS voltage in frontal leads. Furthermore, the patients were classified into two groups based on the presence or absence of isolated very low QRS voltage. Enhanced syncopic spells over the past 6 months, and the positive rates of head-up tilt test were observed in patients having isolated very low QRS voltage in the frontal leads than those without isolated very low QRS voltage (p < 0.05). The single factor and time-to-event analyses also showed an increased syncope recurrence rate in patients with isolated very low QRS voltage in frontal leads when compared with those without isolated very low QRS voltage (p < 0.01).

Isolated very low QRS voltage in frontal leads is correlated with the positive response of the head-up tilt test in children who experience syncope and its recurrence post-treatment. Hence, isolated very low QRS voltage in frontal leads might become a novel diagnostic indicator and a risk factor for syncope recurrence in children with vasovagal syncope.

Endomyocardial biopsy remains the gold standard for cardiac cellular rejection surveillance after heart transplantation. We studied a novel non-invasive index of left ventricular relaxation to detect cardiac cellular rejection in paediatric heart transplant patients.

This is a single-centre retrospective study of paediatric heart transplant patients who underwent endomyocardial biopsy from June 2014 to September 2021. Left ventricular relaxation index was calculated as the sum of diastolic tissue Doppler imaging velocities (E) of the left ventricular lateral, septal, and posterior walls divided by the percentage of the left ventricular posterior wall thinning by M-mode. Statistical analysis included t-tests and Mann-Whitney tests to compare means and medians between treatment and non-treatment groups. We used the cut-off with the maximum Youden index to compare the sensitivity and specificity of left ventricular relaxation index to detect rejection.

The study included 65 patients who underwent 246 cardiac catheterizations and endomyocardial biopsies. Out of 246, 192 procedures were included and 54 were excluded due to recent transplants or lack of echocardiographic data. A total of 114 demonstrated Grade 0R, 68 Grade 1R, 8 Grade 2R, and 2 Grade 3R allograft rejection. The difference in mean left ventricular relaxation index between treatment versus non-treatment groups (2R, 3R vs. 0R, 1R) was not statistically significant (p = 0.917). A left ventricular relaxation index cut-off of 0.73 had the highest Youden index with good sensitivity (100%) and poor specificity (23%) for detecting rejections with grades 2R and 3R.

Left ventricular relaxation index, a novel index of left ventricular relaxation, was not a sensitive or specific predictor of cardiac cellular rejection in paediatric heart transplants.

The functional roles of ventricular dominance and additional ventricular chamber after Fontan operation are still uncertain. We aim to assess and correlate such anatomical features to late clinical outcomes.

Fontan patients undergoing cardiac MRI and cardiopulmonary exercise test between January 2020 and December 2022 were retrospectively reviewed. Clinical, cardiac MRI, and cardiopulmonary exercise test data from the last follow-up were analysed.

Fifty patients were analysed: left dominance was present in 29 patients (58%, median age 20 years, interquartile range:16–26). At a median follow-up after the Fontan operation was 16 years (interquartile range: 4–42), NYHA classes III and IV was present in 3 patients (6%), 4 (8%) underwent Fontan conversion, 2 (4%) were listed for heart transplantation, and 2 (4%) died. Statistical analysis showed that the additional ventricular chamber was larger (>20 mL/m2) in patients with a right dominant ventricle (p = 0.01), and right dominance was associated with a higher incidence of post-operative low-cardiac output syndrome (p = 0.043). Left ventricular dominance was associated with a better ejection fraction (p = 0.04), less extent of late gadolinium enhancement (p = 0.022), higher metabolic equivalents (p = 0.01), and higher peak oxygen consumption (p = 0.033). A larger additional ventricular chamber was associated with a higher need for post-operative extracorporeal membrane oxygenation support (p = 0.007), but it did not influence functional parameters on cardiac MRI or cardiopulmonary exercise test.

In Fontan patients, left ventricular dominance correlated to better functional outcomes. Conversely, a larger additional ventricular chamber is more frequent in right ventricular dominance and can negatively affect the early post-Fontan course.

In recent years, the importance of telemedicine has increased significantly. Especially in the field of echocardiography, virtual reality glasses offer the possibility of real-time data transmission without restrictions in the examination process. In particular, the care of critically ill newborns with suspected CHD might be improved by allowing a specialized paediatric cardiologist to remotely guide an echocardiographic examination. The current study aims to prove whether novices, under Google Glass guidance by a paediatric cardiologist, can perform an appropriate neonatal echocardiography.

The current study is a prospective monocentric single-blinded pilot study. Participants were supposed to perform two test runs: The first test run was “unguided” and the second test run was instructed via Google Glass. A validated training simulator for neonatal echocardiography “EchocomNeo, Echocom GmbH” was used. The study took place at the Leipzig Heart Center, Department of Pediatric Cardiology from April 2022 to November 2022.

A total of 21 medical students were enrolled. In total 252 views (126 views in each test run) were recorded. The overall performance was significantly higher in the Google Glass guided test run compared to “unguided” (structure score: 77.6% vs. 63.2%. p < 0.001 and quality score: 58.7% vs. 47.2%, p < 0.001). Also, the time was significantly lower in the Google Glass guided test run than in the unguided test run, p = 0.014.

Google Glass guidance by a paediatric cardiologist could optimize the performance of novices in echocardiography using a standardized neonatal echo-simulator with structural normal cardiac anatomy.

Lack of psychological safety within surgical teams and fear of damaging one’s career or reputation may counteract the effect of formal reporting as a tool for preventing bullying. Also, personality traits of cardiothoracic surgeons may promote bullying. In the current study, we evaluate these factors and their association with bullying in cardiothoracic surgical teams.

Forty-four cardiothoracic surgeons filled in the Negative Acts Questionnaire-Revised, Safety Attitude Questionnaire, and Big Five Inventory.

Of the 44 participants, 18 (40.90%) experienced bullying during their careers. Psychological safety was negatively associated with work-related bullying and positively associated with the agreeableness and openness personality factors.

Bullying within the cardiothoracic surgical teams is a prominent problem that is negatively associated with psychological safety. Psychological safety is positively associated with agreeableness and openness, and interventions focusing on promoting an organizational culture based on such factors may improve the effectiveness of such interventions.

Interstage monitoring programs for single ventricle disease have been developed to reduce morbidity and mortality. There is increased use of telemedicine and mobile application monitoring. It is unknown if there are disparities in use based on patient socio-demographic factors.

We conducted a retrospective cohort study of patients enrolled in the single ventricle monitoring program and KidsHeart application at a single centre from 4/21/2021 to 12/31/2023. We investigated the association of socio-demographic factors with telemedicine usage, mobile application enrollment and usage. We assessed resource utilisation and weight changes by program era.

There were 94 children in the cohort. Patients with Norwood and ductal stent had higher mean telemedicine visits per month (1.8 visits, p = 0.004), without differences based on socio-demographic factors. There were differences in application enrollment with more Black patients enrolled compared to White patients (p = 0.016). There were less Hispanic patients enrolled than Non-Hispanic patients (p = 0.034). There were no Spaish speaking patient’s enrolled (p = 0.0015). There were no patients with maternal education of less than high school enrolled and all those with maternal education of advanced degree were enrolled (p = 0.0016). There was decreased mobile application use in those from neighbourhoods in the lowest income quartile. There were decreased emergency department visits with mobile application monitoring. Mean weight-for-age z-scores had increased from start to completion of the program in all eras.

Differences were seen in mobile application enrollment and usage based on socio-demographic factors. Further work is needed to ensure that all patients have access to mobile application usage.

To determine the short- and medium-term cardiac outcomes in children admitted with multisystem inflammatory syndrome in children at a tertiary care centre in Pakistan.

Children fulfilling the criteria for multisystem inflammatory syndrome and admitted to the hospital between April 2020 and March 2022 were enrolled in this prospective longitudinal cohort study. From admission to discharge, laboratory and cardiac parameters were recorded for all patients, who were subsequently followed up in clinics at various intervals. Data analysis was conducted using STATA version 15.0.

A total of 51 children were included, with viral myocarditis (41.2%) and toxic shock syndrome (33.3%) being the most common phenotypes. The cardiovascular system was most commonly affected in 27 children (53%) with laboratory evidence of inflammation and myocardial injury with median and interquartile levels of ferritin 1169 (534-1704), C-reactive protein 83 (24-175), lactate dehydrogenase 468 (365-1270), N-terminal pro-B-type natriuretic peptide 8,656 (2,538-31,166), and troponin 0.16 (0.02-2.0).

On admission, decreased left ventricular ejection fraction was observed in 58.8% of patients and impaired global longitudinal strain in 33.3%. At discharge, left ventricular ejection fraction had normalised in 83% of patients. Pericardial effusion resolved in all patients, and valvulitis resolved in 86% by 12 months. Paediatric ICU admission was required in 42 (82%) of patients with an overall mortality of 12% (n = 6).

Our study finds high hospital mortality for multisystem inflammatory syndrome in children compared to 1-2% from previous studies. Yet, in Pakistan, surviving children with multisystem inflammatory syndrome show favourable short- to medium-term cardiac outcomes

Despite advances in treatment and outcomes for paediatric heart failure, both physical and psychosocial comorbidities remain notable among this patient population. We aimed to qualitatively describe the psychosocial experiences of adolescent and young adults with heart failure and their caregivers’ perceptions, with specific focus on personal challenges, worries, coping skills, and resilience.

Structured, in-depth interviews were performed with 16 adolescent and young adults with heart failure and 14 of their caregivers. Interviews were recorded and transcribed. Content analysis was performed, and themes were generated. Transcripts were coded by independent reviewers.

Ten (63%) adolescent and young adults with heart failure identified as male and six (37.5%) patients self-identified with a racial or ethnic minority group. Adolescent and young adults with heart failure generally perceived their overall illness experience more positively and less burdensome than their caregivers. Some adolescent and young adults noted specific worries related to surgeries, admissions, major complications, death, and prognostic/treatment uncertainty, while caregivers perceived their adolescent and young adult’s greatest worries to be around major complications and death. Adolescent and young adults and their caregivers were able to define and reflect on adolescent and young adult experiences of resilience, with many adolescent and young adults expressing a sense of optimism and gratitude as it relates to their medical journey.

This study is the first of its kind to qualitatively describe the psychosocial experiences of a racially and socioeconomically diverse sample of adolescent and young adults with heart failure, as well as their caregivers’ perceptions of patient experiences. Findings underscore the importance of identifying distress and fostering resilient processes and outcomes in young people with advanced heart disease.

Guidelines recommend screening for psychiatric co-morbidities in patients with congenital heart defects alongside cardiac outpatient follow-ups. These recommendations are not implemented in Denmark. This study aimed to investigate the psychiatric co-morbidities in children and adolescents with Fontan circulation in Denmark and to evaluate the feasibility of an online screening measure for psychiatric disorders.

Children, adolescents, and their families answered the Development and Well-Being Assessment questionnaire and a questionnaire about received help online. Development and Well-Being Assessment ratings present psychiatric diagnoses in accordance with ICD-10 and DSM-5. Parent-reported received psychiatric help is also presented. Feasibility data are reported as participation rate (completed Development and Well-Being Assessments) and parental/adolescent acceptability from the feasibility questionnaire.

The participation rate was 27%. Of the participating children and adolescents, 53% (ICD-10)/59% (DSM-5) met full diagnostic criteria for at least one psychiatric diagnosis. Of these, 50% had not received any psychiatric or psychological help. Only 12% of participants had an a priori psychiatric diagnosis.

We found that a large proportion of children and adolescents with Fontan circulation are underdiagnosed and undertreated for psychiatric disorders. The results from our study emphasise the need for psychiatric screening in this patient group. Development and Well-Being Assessment may be too comprehensive for online electronic screening in children and adolescents with CHD.

Our objective was to assess the predictive value of physiologic dead space fraction for mortality in patients undergoing the comprehensive stage 2 operation.

This was a single-centre retrospective observational study conducted at a quaternary free-standing children’s hospital specialising in hybrid palliation of single ventricle cardiac disease. 180 patients underwent the comprehensive stage 2 operation. 76 patients (42%) underwent early extubation, 59 (33%) standard extubation, and 45 (25%) delayed extubation. We measured time to extubation, post-operative outcomes, length of stay and utilised Fine gray models, Youden’s J statistic, cumulative incidence function, and logistic regression to analyse outcomes.

Delayed extubation group suffered significantly higher rates of mortality (31.1% vs. 6.8%), cardiac arrest (40.0% vs. 10.2%), stroke (37.8% vs. 11.9%), and need for catheter (28.9% vs. 5.1%) and surgical intervention (24.4% vs. 8.5%) (P < 0.001). Physiologic dead space fraction was significantly higher in the delayed extubation group and in non-survivors with a value of 0.3, which was found to be the discriminatory point by Youden’s J statistic. For a 0.1 unit increase in physiologic dead space fraction on post-operative day 1, the odds of a patient expiring increase by a factor of 2.26 (95% CI 1.41–3.97, p < 0.001) and by a factor of 3.79 (95% CI 1.65–11.7, p 0.01) on post-operative day 3.

Delayed extubation impacts morbidity and mortality in patients undergoing the comprehensive stage 2 operation. Increased physiologic dead space fraction in the first 60 hours after arrival to the ICU is associated with higher mortality.

Complexity stratification for CHD is an integral part of clinical research due to its heterogenous clinical presentation and outcomes. To support our ongoing research efforts into CHD requiring disease severity stratifications, a simplified CHD severity classification system was developed and verified, with potential utility for clinical researchers without specialist CHD knowledge or access to clinical/medical records.

A two-tiered analysis approach was undertaken. First-tier analysis included the audit of a comprehensive system based on: i) timing of intervention, ii) cardiac morphology, and iii) cardiovascular physiology using real patient data (n = 30), across 10 common CHD lesions. Second-tier analysis allowed for a simplified version of the classification system using morphology as a stand-alone predictor. Twelve clinicians of varying specialities involved in CHD care ranked 10 common lesions from least to most severe based on typical presentation and clinical course.

First-tier analysis identified that cardiac morphology was the principal driver of complexity. Second-tier analysis largely confirmed the ranking and classification of the lesions into the broad CHD severity groups, although some variation was noted, specifically among non-cardiac specialists. This simplified version of the classicisation system, with morphology as a stand-alone predictor of severity, allowed for effective stratification for the purposes of analysis.

The findings presented here support this comprehensive and simple CHD severity classification system with broad utility in CHD research, particularly among clinicians and researchers with limited knowledge of CHD. The model may be applied to produce locally relevant research tools.

Thromboembolism is a complication in paediatric patients with CHD requiring cardiac surgery. Previous research has focused on post-operative thromboembolism. This study aimed to describe thromboembolism frequency before or after cardiac surgery in children with CHD.

We performed a single-centre retrospective study from October 2020 to June 2023 (inclusive). Patients were eligible for inclusion if they were <21 years of age and underwent cardiac surgery. Outcomes of interest included the occurrence and characteristics of thromboembolism in the 12 months before and after surgery, antithrombotic therapies, recurrent thromboembolism, and clinically significant bleeding.

Among 260 patients included, 35 (13.5%) developed an index thromboembolism. Twelve (34.3%) patients had an index thromboembolism <12 months before surgery and 23 (65.7%) had an index thromboembolism <12 months after surgery, including 8 (22.9%) patients who had thromboembolism during both exposure periods. The median interquartile range (IQR) time of thromboembolism relative to cardiac surgery was –26 (–4 to –140) days and 15 (4 to 41) days, respectively. Seven (20%) patients had arterial, 18 (51.4%) venous, and 3 (8.6%) had both arterial and venous thromboembolism. Median (IQR) antithrombotic therapy duration was 49 (24–84) days. Nine (25.7%) patients developed recurrent thromboembolism and five (14.3%) patients experienced clinically significant bleeding.

The risk of thromboembolism and recurrence is high both before and after cardiac surgery among paediatric patients with CHD. Prospective multi-centre studies should seek to identify risk factors for preoperative and postoperative thromboembolism to inform the design of future risk-stratified thromboembolism prevention trials in children with CHD.

New drugs to target different pathways in pulmonary hypertension has resulted in increased combination therapy, but details of this use in infants are not well described. In this large multicenter database study, we describe the pharmacoepidemiology of combination pulmonary vasodilator therapy in critically ill infants.

We identified inborn infants discharged home from a Pediatrix neonatal ICU from 1997 to 2020 exposed to inhaled nitric oxide, sildenafil, epoprostenol, or bosentan for greater than two consecutive days. We compared clinical variables and drug utilisation between infants receiving simultaneous combination and monotherapy. We reported each combination’s frequency, timing, and duration and graphically represented drug use over time.

Of the 7681 infants that met inclusion criteria, 664 (9%) received combination therapy. These infants had a lower median gestational age and birth weight, were more likely to have cardiac and pulmonary anomalies, receive cardiorespiratory support, and had higher in-hospital mortality than those receiving monotherapy. Inhaled nitric oxide and sildenafil were most frequently used, and utilisation of combination and monotherapy for all drugs increased over time. Inhaled nitric oxide and epoprostenol were used in infants with a higher gestational age, earlier postnatal age, and shorter duration than sildenafil and bosentan. Dual therapy with inhaled nitric oxide and sildenafil was the most common combination therapy.

Our study revealed an increased use of combination pulmonary vasodilator therapy, favouring inhaled nitric oxide and sildenafil, yet with considerable practice variation. Further research is needed to determine the optimal combination, sequence, dosing, and disease-specific indications for combination therapy.