Atrial septal defects are a common form of CHD and dependent on the size and nature of atrial septal defects, closure may be warranted. The paper aims to compare outcomes of transcatheter versus surgical repair of atrial septal defects.

A comprehensive electronic literature search was conducted. Primary studies were included if they compared both closure techniques. Primary outcomes included procedural success, mortality, and reintervention rate. Secondary outcomes included residual defect and mean hospital stay.

A total of 33 studies were included in meta-analysis. Mean total hospital stay was significantly shorter in the transcatheter cohort across both the adult (95% confidence interval, mean difference −4.05 (−4.78, −3.32) p < 0.00001) and paediatric populations (95% confidence interval, mean difference −4.78 (−5.97, −3.60) p < 0.00001). There were significantly fewer complications in the transcatheter group across both the adult (odds ratio 0.45, 95% confidence interval, [0.28, 0.72], p < 0.00001) and paediatric cohorts (odds ratio 0.26, 95% confidence interval, [0.14, 0.49], p < 0.00001). No significant difference in overall mortality was found between transcatheter versus surgical closure across the two groups, adult (odds ratio 0.76, 95% confidence interval, [0.40, 1.45], p = 0.41), paediatrics (odds ratio 0.62, 95% confidence interval, [0.21, 1.83], p = 0.39).

Both transcatheter and surgical approaches are safe and effective techniques for atrial septal defect closure. Our study has demonstrated the benefits of transcatheter closure in terms of lower complication rates and mean hospital stay. However, surgery still has a place for more complex closure and, as we have demonstrated, shows no difference in mortality.

Children with critical CHD are at risk for neurodevelopmental impairments, including delays in expressive and receptive language development. However, no study has synthesised the literature regarding language abilities in children with this condition.

We summarised the literature regarding expressive and receptive language in preschool children with critical CHD.

MEDLINE, Embase, Scopus, Child Development and Adolescent Studies, ERIC, PsycINFO, and CINAHL.

We included studies published between January, 1990 and 1 July, 2021, focused on children aged ≤5 years with critical CHD requiring a complex cardiac procedure at age <1 year. Language ability was documented using standardised, validated tools assessing both expressive and receptive language outcomes.

Data (study, patient and language characteristics, and results) were extracted by two reviewers.

Seventeen studies were included. Among children 2–5 years old with critical CHD, there were statistically significant deficits in overall (standardised mean difference: –0.46; 95 % confidence interval: –0.56, –0.35), expressive (standardised mean difference: –0.45;95 % confidence interval: –0.54, –0.37), and receptive (standardised mean difference: –0.32; 95 % confidence interval: –0.40, –0.23) language compared to normative data. Results reported as medians were similar to meta-analysis findings. Subgroup analysis showed that children with univentricular physiology had lower language scores than children with biventricular physiology.

Preschool children with critical CHD had statistically significantly lower language outcomes compared to expected population norms. Healthcare professionals should test early and often for language deficits, referring to individually tailored supports.

To evaluate the association of systolic blood pressure percentile, race, and body mass index with left ventricular hypertrophy on electrocardiogram and echocardiogram to define populations at risk.

This is a retrospective cross-sectional study design utilising a data analytics tool (Tableau) combining electrocardiogram and echocardiogram databases from 2003 to 2020. Customized queries identified patients aged 2–18 years who had an outpatient electrocardiogram and echocardiogram on the same date with available systolic blood pressure and body measurements. Cases with CHD, cardiomyopathy, or arrhythmia diagnoses were excluded. Echocardiograms with left ventricle mass (indexed to height2.7) were included. The main outcome was left ventricular hypertrophy on echocardiogram defined as Left ventricle mass index greater than the 95th percentile for age.

In a cohort of 13,539 patients, 6.7% of studies had left ventricular hypertrophy on echocardiogram. Systolic blood pressure percentile >90% has a sensitivity of 35% and specificity of 82% for left ventricular hypertrophy on echocardiogram. Left ventricular hypertrophy on electrocardiogram was a poor predictor of left ventricular hypertrophy on echocardiogram (9% sensitivity and 92% specificity). African American race (OR 1.31, 95% CI = 1.10, 1.56, p = 0.002), systolic blood pressure percentile >95% (OR = 1.60, 95% CI = 1.34, 1.93, p < 0.001), and higher body mass index (OR = 7.22, 95% CI = 6.23, 8.36, p < 0.001) were independently associated with left ventricular hypertrophy on echocardiogram.

African American race, obesity, and hypertension on outpatient blood pressure measurements are independent risk factors for left ventricular hypertrophy in children. Electrocardiogram has little utility in the screening for left ventricular hypertrophy.

The goal of this meta-analysis is to evaluate the effects of exercise training on long-term health and cardiorespiratory fitness in participants with CHD after surgery and to investigate the optimal type of exercise training for post-operative patients and how to improve adherence to it.

We searched the Cochrane Central Register of Controlled Trials, MEDLINE, EMBASE, and Web of Science from the date of the inception of the database through August 2021.

Altogether, 1424 records were identified in the literature search. Studies evaluating outcomes between exercise training and usual care among post-operative patients with CHD were included. The assessed outcomes were quality of life and cardiorespiratory fitness. We analysed heterogeneity by using the I2 statistic and evaluated the evidence quality according to the recommendation by the Cochrane Collaboration. Nine randomised controlled trials were included. The evidence showed that exercise interventions increased peak oxygen consumption (mean difference = 2.29 [95% CI 0.43, 4.15]; p = 0.02, I2 = 0%). However, no differences in scores of health-related quality of life and pulmonary function were observed between the experimental and control groups.

In conclusion, participation in a physical exercise training programme was safe and improved fitness in patients after surgery for CHD. We recommend that post-operative patients with CHD participate in physical exercise training. Additional research is needed to study the various forms of exercise training and their impact on quality of life.

Patients with single ventricle heart disease are living into adulthood due to medical and surgical advancements but have significant physical comorbidities and an increased risk for psychological comorbidities compared to healthy subjects or those with other CHD diagnoses. This study aimed to systematically review psychological functioning in paediatric single ventricle heart disease.

Literature was searched using PubMed, Embase, PsycInfo, CINAHL Complete and Scopus. Peer-reviewed articles that included patients ages 0–25 years with single ventricle heart disease, and quantitative measures of psychological outcomes were included. Meta-analysis using a fixed-effect model was conducted for internalising and externalising t-scores, utilised by the Achenbach Child Behavior Checklist.

Twenty-nine records met the criteria for inclusion. 13/24 studies demonstrated increased risk for internalising disorders, such as anxiety/depression; 16/22 studies demonstrated risk for externalising disorders, such as attention or behavioural problems. Meta-analysis of four studies revealed that paediatric single ventricle heart disease patients had no significant difference in internalising and externalising t-scores compared to normative values.

The current review demonstrates the need for further studies to better understand psychological functioning in patients with single ventricle heart disease, with a majority of studies showing increased risk for psychological problems despite no difference seen in a small meta-analysis. This summary of the literature underscores the need for regular psychological screening, earlier intervention and integrated mental health therapies in paediatric single ventricle heart disease.

Pain following surgery for cardiac disease is ubiquitous, and optimal management is important. Despite this, there is large practice variation. To address this, the Paediatric Acute Care Cardiology Collaborative undertook the effort to create this clinical practice guideline.

A panel of experts consisting of paediatric cardiologists, advanced practice practitioners, pharmacists, a paediatric cardiothoracic surgeon, and a paediatric cardiac anaesthesiologist was convened. The literature was searched for relevant articles and Collaborative sites submitted centre-specific protocols for postoperative pain management. Using the modified Delphi technique, recommendations were generated and put through iterative Delphi rounds to achieve consensus

60 recommendations achieved consensus and are included in this guideline. They address guideline use, pain assessment, general considerations, preoperative considerations, intraoperative considerations, regional anaesthesia, opioids, opioid-sparing, non-opioid medications, non-pharmaceutical pain management, and discharge considerations.

Postoperative pain among children following cardiac surgery is currently an area of significant practice variability despite a large body of literature and the presence of centre-specific protocols. Central to the recommendations included in this guideline is the concept that ideal pain management begins with preoperative counselling and continues through to patient discharge. Overall, the quality of evidence supporting recommendations is low. There is ongoing need for research in this area, particularly in paediatric populations.

Young patients with Fontan circulation may have low serum 25-hydroxyvitamin D levels, an affected liver, and unhealthy body compositions. This study aimed to explore the association between vitamin D intake/levels, liver biomarkers, and body composition in young Fontan patients.

We collected prospective data in 2017 to 2018, obtained with food-frequency questionnaires, biochemical analyses of liver biomarkers, and dual-energy X-ray absorptiometry scans in 44 children with Fontan circulation. Body compositions were compared to matched controls (n = 38). Linear regression analyses were used to investigate associations of biomarkers, leg pain, and lean mass on serum levels of 25-hydroxyvitamin D. Biomarkers were converted to z scores and differences were evaluated within the Fontan patients.

Our Fontan patients had a daily mean vitamin D intake of 9.9 µg and a mean serum 25-hydroxyvitamin D of 56 nmol/L. These factors were not associated with fat or lean mass, leg pain, or biomarkers of liver status. The Fontan patients had significantly less lean mass, but higher fat mass than controls. Male adolescents with Fontan circulation had a greater mean abdominal fat mass than male controls and higher cholesterol levels than females with Fontan circulation.

Vitamin D intake and serum levels were not associated with body composition or liver biomarkers in the Fontan group, but the Fontan group had lower lean mass and higher fat mass than controls. The more pronounced abdominal fat mass in male adolescents with Fontan circulation might increase metabolic risks later in life.

While there is evidence that cognitive impairment of children with congenital heart disease (CHD) may persist into adolescence, little is known about the spectrum of neurocognitive functioning of young adults with this disorder. The aim of this study was to assess neurocognitive functioning in a population of young adults with different types of CHD.

Cross-sectional cohort study in young adults with CHD and a group-matched healthy control group. We assessed neurocognitive and general intellectual functioning with a comprehensive battery of standardised neuropsychological tests. In addition to task-based assessments, questionnaire data of executive dysfunctions in everyday life were measured with the Behaviour Rating Inventory of Executive Function – Adult Version.

A total of 67 patients (55% men) with CHD and 55 healthy controls (51% men) were included for analysis. Mean age at assessment was 26.9 (3.68) and 26.0 (3.32) years, respectively. The CHD group performed poorer in the domains of Executive Functions, Memory, Attention & Speed, and general intellectual functioning. Patients with a CHD of severe complexity were more affected than patients with simple or moderate complexity. Behaviour Rating Inventory of Executive Function – Adult Version scores indicated that patients’ self-rated deficits in behaviour regulation in everyday life was higher compared with healthy controls.

Our findings indicate lower neurocognitive functioning in young adults with a CHD, particularly in those with severe defect complexity. In view of the potentially enhanced risk for cerebrovascular and neurodegenerative disease in this patient group as reported in the literature, systematic longitudinal monitoring of cognitive functioning is recommended.