Young adults with heart disease constitute a growing group with the risk of cognitive and physical impairment. The knowledge of their academic performance and mental and physical health is, however, scant. This study aimed to compare young adults with CHDs or arrhythmia with their peers.

Information on physical health (Somatic Symptom Scale-8), mental health problems (Hopkins Symptoms Checklist-25), quality of life (Satisfaction With Life Scale), physical activity, and academic performance was collected online in a national cross-sectional survey in Norway among students in higher education (the SHoT2018 study).

Among 50,054 students, 172 (0.34%) reported CHD and 132 (0.26%) arrhythmias. Students reporting arrhythmias scored significantly higher than the control group on somatic symptoms (OR = 2.3 (95% CI: 1.62–3.27)), anxiety (OR = 1.60 (1.08–2.37)), depression (OR = 1.49 (1.05–2.11)), self-harm, and suicide attempt (OR = 2.72 (1.56–4.75)), and lower quality of life (OR 1.64 (1.16–2.32)) and more loneliness (OR = 1.99 (1.28–3.10)) compared to participants without heart disease. Participants with CHD reported an increased somatic symptom burden (OR = 1.58 (1.16–2.16)). Despite a tendency to a higher score, this group did not differ significantly from the control group on anxiety or depression, quality of life, or loneliness. However, the risk of self-harm thoughts and suicidality was significantly increased (OR for suicide attempt 2.22 (1.3–3.77)). There was no difference between the groups on academic performance.

Although Norwegian students with heart disease reported more somatic symptoms, their academic progress was not reduced compared to students without heart disease. Students with CHD or arrhythmias showed an increased risk of self-harm thoughts and suicidality.

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.

With increased survival, children with CHD are reaching adulthood, however, obesity amongst this cohort is an emerging problem. Making every contact count encourages clinicians to utilise contact to elicit behaviour change. The aim of this work was to identify whether the body habitus of children classified as obese was addressed during a clinical review.

A retrospective observational cohort study was completed using a cardiology outpatient dataset from 2010 to 2019. Inclusion criteria are all children with a body mass index z score classified as obese (≥ 2 z scores). Individual electronic patient records were reviewed to identify long-term anthropometric measures including (i) recognition of body habitus, (ii) prescription of physical activity or dietary intervention, and (iii) referral to a weight management programme or dietitian.

From the cohort of 95 patients, 285 “obese clinical encounters” were identified, at the time of a cardiology clinic attendance. Of those, obesity was acknowledged in 25 clinic letters (8.65%), but only 8 used the correct terms “obese” or “obesity” (2.77%). Action to tackle obesity was recorded in 9.3% of cases with a direct referral to a dietitian being made on 3 occasions (1.04%).

Body habitus is not being routinely addressed by cardiologists caring for paediatric and young adult cardiac patients. This study has recognised an alarmingly high incidence of missed opportunities to make every contact count, to manage those with obesity and associated risk factors.

Prior to the recent release of appropriate use criteria for imaging valvulopathies in children, follow-up of valvular lesions, including isolated bicuspid aortic valve, was not standardised. We describe current follow up, treatment, and intervention strategies for isolated bicuspid aortic valve with varying degrees of stenosis, regurgitation, and dilation in children up to 18 years old and compare them with newly released appropriate use criteria.

Online survey was sent to members of the American Academy of Pediatrics Section on Cardiology and Cardiac Surgery and PediHeartNet.

Totally, 106 responses with interpretable data were received. For asymptomatic patients with isolated BAV without stenosis, regurgitation, or dilation follow-up-intervals increased from 7+/−4 months in the newborn period to 28 +/− 14 months at 18 years of age. Respondents recommended more frequent follow-up for younger patients and those with greater disease severity. More than 80% of respondents treat aortic regurgitation or aortic dilation in the setting of bicuspid aortic valve medically. In general, intervention was recommended once stenosis or regurgitation became severe (stenosis of >4 m/s; regurgitation with LV Z score 4) regardless of age, but was not routinely recommended for younger children (newborn – age 6 years) with severe dilation. Exercise was restricted at 38+/−11 mmHg echocardiographic mean gradient.

Current follow-up, treatment, and intervention strategies for isolated bicuspid aortic valve deviate from appropriate use criteria. Differences between the two highlight the need to better delineate the disease course, clarify recommendations for care, and encourage wider adoption of guidelines.

There are a few number of case reports and small-scale case series reporting dilated cardiomyopathy due to vitamin D-deficient rickets. The present study evaluates the clinical, biochemical, and echocardiographic features of neonates with vitamin D deficiency.

In this prospective single-arm observational study, echocardiographic evaluation was performed on all patients before vitamin D3 and calcium replacement. Following remission of biochemical features of vitamin D deficiency, control echocardiography was performed. Biochemical and echocardiographic characteristics of the present cohort were compared with those of 27 previously published cases with dilated cardiomyopathy due to vitamin D deficiency.

The study included 148 cases (95 males). In the echocardiographic evaluation, none of the patients had dilated cardiomyopathy. All of the mothers were also vitamin D deficient and treated accordingly. Comparison of patients with normocalcaemia and hypocalcaemia at presentation revealed no statistically significant difference between the ejection fraction and shortening fraction, while left ventricle end-diastolic diameter and left ventricle end-systolic diameter were higher in patients with hypocalcaemia. Previously published historical cases were older and had more severe biochemical features of vitamin D deficiency.

To the best of our knowledge, in this first and largest cohort of neonates with vitamin D deficiency, we did not detect dilated cardiomyopathy. Early recognition and detection before developing actual rickets and preventing prolonged hypocalcaemia are critically important to alleviate cardiac complications.

Ventriculo-ventricular interactions are known to exist, though not well quantified. We hypothesised that the ventricular–vascular coupling ratio assessed by cardiovascular MRI would provide insight into this relationship. We also sought to compare MRI-derived ventricular–vascular coupling ratio to echocardiography and patient outcomes.

Children with cardiac disease and biventricular physiology were included. Sanz’s and Bullet methods were used to calculate ventricular–vascular coupling ratio by MRI and echocardiography, respectively. Subgroup analysis was performed for right and left heart diseases. Univariate and multivariate regressions were performed to determine associations with outcomes.

A total of 55 patients (age 14.3 ± 2.5 years) were included. Biventricular ventricular–vascular coupling ratio by MRI correlated with each other (r = 0.41; p = 0.003), with respect to ventricle’s ejection fraction (r = −0.76 to −0.88; p < 0.001) and other ventricle’s ejection fraction (r = −0.42 to −0.47; p < 0.01). However, biventricular ejection fraction had only weak correlation with each other (r = 0.31; p = 0.02). Echo underestimated ventricular–vascular coupling ratio for the left ventricle (p < 0.001) with modest correlation to MRI-derived ventricular–vascular coupling ratio (r = 0.43; p = 0.002). There seems to be a weak correlation between uncoupled right ventricular–vascular coupling ratio with the need for intervention and performance on exercise testing (r = 0.33; p = 0.02).

MRI-derived biventricular ventricular–vascular coupling ratio provides a better estimate of ventriculo-ventricular interaction in children and adolescents with CHD. These associations are stronger than traditional parameters and applicable to right and left heart conditions.

The citation history of a published article reflects its impact on the literature over time. We conducted a comprehensive bibliometric analysis to identify the most cited papers on CHD in children.

One-hundred and ninety journals listed in Journal Citation Reports were accessed via Web of Science. Publications with 250 or more citations were identified from Science Citation Index Expanded (1900–2020), and those relating to structural CHD in children were reviewed. Articles were ranked by citation count and the 100 most cited were analysed.

The number of citations ranged from 2522 to 309 (median 431, IQR 356–518), with 35 published since 2000. All were written in English, most originated from the United States (74%), and were published in cardiovascular journals, with Circulation (28%) the most frequent. There were 86 original research articles, including 50 case series, 14 cohort studies, and 10 clinical trials. The most cited paper was by Hoffman JI and Kaplan S on the incidence of CHD. Thirteen authors had 4 or more publications in the top 100, all of whom had worked in Boston, Philadelphia, San Francisco, or Dallas, and the most prolific author was Newburger JW (9 articles).

Citation analysis provides a historical perspective on scientific progress by assessing the impact of individual articles. Our study highlights the dominant position of US-based researchers and journals in this field. Most of the highly cited articles remain case series, with few randomised controlled trials in CHD appearing in recent years.

Aortic root dilation is a major complication of Marfan syndrome and is one of the most important criteria in establishing the diagnosis. Currently, different echocardiographic nomograms are used to calculate aortic root Z-scores. The aim of the present study was to assess the potential differences in aortic root measurements when aortic root Z-scores were obtained in a cohort of paediatric Marfan patients using several published nomograms.

In a cohort of 100 children with Marfan syndrome, Z-scores for aortic root dimensions were calculated according to the nomograms of Pettersen et al, Gautier et al, Colan et al, and Lopez et al. Bland–Altman plots were used to estimate mean differences in Z-scores and to establish limits of agreement.

The mean Z-score of the sinus of Valsalva for Lopez et al was significantly higher compared to Gautier et al (p < 0.01) and Pettersen et al (p = 0.03). The nomogram of Lopez et al resulted in substantially higher Z-scores in patients with a large sinus of Valsalva diameter. Thirty-five percentage of the studied patients would have a Z-score ≥ 2 using Lopez et al compared to 20% for Pettersen et al, 21% for Gautier et al, and 33% for Colan et al.

The currently available nomograms for calculating Z-scores of aortic dilation in children with Marfan syndrome lead to clinically relevant differences in Z-scores, especially in children with a relative large aortic root diameter. This could have impact on both the diagnosis and treatment of patients with Marfan syndrome.

Growth differentiation factor-15 is a novel biomarker of increasing importance in cardiovascular diseases. This study aimed to evaluate the relationship between ventricular measurements assessed by cardiac magnetic resonance imaging (MRI) and serum growth differentiation factor-15 levels in children with surgically corrected tetralogy of Fallot.

Serum growth differentiation factor-15 levels were measured in 40 patients (mean age: 15.2 ± 2.9 years; 52.5% male; 87.5% NYHA I). End-diastolic volume index, end-systolic volume index, and ejection fractions of both ventricles and pulmonary regurgitation fraction were measured on cardiac MRI. The correlation between growth differentiation factor-15 levels and cardiac MRI parameters of the patients was investigated. Also, growth differentiation factor-15 levels of the patients were compared with healthy controls since reference values have not been determined in children.

The mean growth differentiation factor-15 level was 254.9 ± 6.3 pg/ml in the patient group. There was no correlation between growth differentiation factor-15 levels and cardiac MRI parameters in patients. Also, there was no significant difference in growth differentiation factor-15 levels between the patients and control groups.

The serum levels of growth differentiation factor-15 were uncorrelated with ventricular size, function, and pulmonary regurgitation fraction assessed by cardiac MRI in children with operated tetralogy of Fallot. Moreover, growth differentiation factor-15 levels were not different in these patients from healthy children.

To compile a literature overview of physical activity in children with CHD and to critically evaluate the methodology used for physical activity assessment.

A review of the literature was performed using PubMed to identify studies examining accelerometer and subjectively assessed physical activity in children and adolescents with CHD.

A total of 15 studies were included (6 studies using subjective measures and 9 articles using accelerometers for the assessment of physical activity). The patients generally failed to meet the recommendations of physical activity. When compared to healthy controls, the results were widely divergent in the subjectively assessed measures and the accelerometer-based studies showed a tendency of no difference in physical activity. Neither subjective methods nor accelerometer-based studies reported any difference in physical activity in general, in relation to the severity of the heart disease.

Methodological variation and limitations in the assessment of physical activity largely explain the divergent results and the inability to establish differences in physical activity between children with CHD of different severity and compared to healthy controls. Methodological knowledge and guidelines are provided for improved assessment of physical activity using accelerometers in clinical research.