Left ventricular rotation is physiologically affected by acute changes in preload. We investigated the acute effect of preload changes in chronically underloaded and overloaded left ventricles in children with shunt lesions.

A total of 15 patients with atrial septal defects (Group A: 7.4 ± 4.7 years, 11 females) and 14 patients with patent arterial ducts (Group B: 2.7 ± 3.1 years, 10 females) were investigated using 2D speckle-tracking echocardiography before and after interventional catheterisation. The rotational parameters of the patient group were compared with those of 29 matched healthy children (Group C).

Maximal torsion (A: 2.45 ± 0.9°/cm versus C: 1.8 ± 0.8°/cm, p < 0.05), apical peak systolic rotation (A: 12.6 ± 5.7° versus C: 8.7 ± 3.5°, p < 0.05), and the peak diastolic torsion rate (A: −147 ± 48°/second versus C: −110 ± 31°/second, p < 0.05) were elevated in Group A and dropped immediately to normal values after intervention (maximal torsion 1.5 ± 1.1°/cm, p < 0.05, apical peak systolic rotation 7.2 ± 4.1°, p < 0.05, and peak diastolic torsion rate −106 ± 35°/second, p < 0.05). Patients in Group B had decreased maximal torsion (B: 1.8 ± 1.1°/cm versus C: 3.8 ± 1.4°/cm, p < 0.05) and apical peak systolic rotation (B: 8.3 ± 6.1° versus C: 13.9 ± 4.3°, p < 0.05). Defect closure was followed by an increase in maximal torsion (B: 2.7 ± 1.4°/cm, p < 0.05) and the peak diastolic torsion rate (B: −133 ± 66°/second versus −176 ± 84°/second, p < 0.05).

Patients with chronically underloaded left ventricles compensate with an enhanced apical peak systolic rotation, maximal torsion, and quicker diastolic untwisting to facilitate diastolic filling. In patients with left ventricular dilatation by volume overload, the peak systolic apical rotation and the maximal torsion are decreased. After normalisation of the preload, they immediately return to normal and diastolic untwisting rebounds. These mechanisms are important for understanding the remodelling processes.

Paediatric heart failure is a common clinical syndrome that may be experienced by children with congenital heart disease (CHD) and/or cardiomyopathy. It is characterised by clinical signs/symptoms which reflect the underlying pathophysiology based on one of three main clinical states: Pulmonary over-circulation, pressure overload, and ventricular failure. Current diagnosis relies on clinical assessment and echocardiogram imaging as cardiac biomarkers has been predominantly scientific to date. This review provides a comprehensive overview of paediatric heart failure pathophysiology and considers the available evidence for cardiac biomarkers in this setting.

A literature review was completed using MEDLINE ALL, EMBASE, and PubMed on 10th November, 2022. Search terms included biomarkers, heart failure, heart defects, congenital heart disease, fontan circulation, single ventricle circulation, cardiomyopathy, and child. This allowed the identification of individual cardiac biomarkers which are the focus of this review. These included NT-proBNP, MR-proANP, MR-proADM, troponin, sST2, galectin 3, and growth differentiation factor-15.

Paediatric studies have established reference ranges for NT-proBNP and troponin for children with structurally normal hearts. Of all the biomarkers reviewed, NT-proBNP appears to correlate most closely with symptoms of heart failure and ventricular dysfunction on echocardiogram. However, there remains limited longitudinal data for NT-proBNP, and no validated reference ranges for patients with CHD and/or cardiomyopathy. None of the other biomarkers reviewed were consistently superior to NT-proBNP.

Further large paediatric studies of patients with heart failure are needed to validate NT-proBNP in CHD and to evaluate the role of novel biomarkers in specific types of CHD, e.g. single ventricle physiology.

Impaired isometric muscle strength was previously reported in adults with Fontan circulation. However, it is unclear if this impairment is present in children and adolescents with Fontan circulation. We investigated isometric muscle strength of the lower limb in patients (6–18 years) with Fontan circulation in comparison with healthy controls.

In this cross-sectional study, 43 patients (6–18 years) with Fontan circulation and 43 age- and sex-matched controls were included. Isometric knee extension and plantar flexion muscle strength were assessed using dynamometry (Newton, N). Lean mass of the legs was assessed with dual-energy X-ray absorptiometry. Analyses were performed on group level (n = 43), and for subgroups that included children aged 6–12 years (n = 18) and adolescents aged 13–18 years (n = 25).

On group level, the patients with Fontan circulation had impaired isometric knee extension strength in comparison with the controls (p = 0.03). In subgroup analyses, impaired isometric knee extension strength was present in the adolescents (p = 0.009) but not in the children groups. For plantar flexion, there was no difference between patients and controls. There was no difference in lean mass between patients and controls (9.6 ± 4.3 kg vs. 10.8 ± 5.6 kg, p = 0.31). However, the lean mass was highly correlated to isometric knee extension strength (patients r = 0.89, controls r = 0.96, p < 0.001) and isometric plantar flexion strength (patients r = 0.7, controls r = 0.81, p < 0.001).

The finding of impaired isometric knee extension muscle strength in adolescents (13–18 years) with Fontan circulation and no corresponding impairment in the children group (6–12 years) could imply that isometric muscle strength gets more impaired with age.

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.

Catheter interventions for residual lesions in the early postoperative period after CHD operations are still not established as a reliable treatment option.

We retrospectively reviewed our institutional experience of cardiac catheterisations and catheter interventions performed in the early postoperative period. We classified our patients into two groups. The “hyper” acute phase group – operation to cardiac catheterisation of ⩽7 days – and acute phase group – operation to cardiac catheterisation from 7 to 30 days.

Of the 47 patients, catheter interventions were performed in 38 patients (81%). The success rate of the intervention was 96% in the acute phase group and 90% in the “hyper” acute phase group. The overall success rate was 95%. There were two self-limited complications in the acute phase group, but not in the “hyper” acute phase group. There were four cases of catheter interventions performed for a newly reconstructed aortic arch, and those procedures were also safe and effective.

Cardiac catheterisations and catheter interventions were safe and effective not only in the early postoperative period but also in the very early postoperative period. Catheter interventions for the left-sided heart in the early postoperative period were also safe and effective.

Adults with CHD are known to greatly benefit from a prompt access to continuous expert care. On the other hand, coronavirus disease 2019 pandemic has determined a dramatic worldwide reconfiguration of the healthcare systems, with rapid redeployment of resources towards this emergency. Italy was the first Western country affected by a large-scale spread of coronavirus disease 2019. The aim of our study is to analyse the impact of the coronavirus disease 2019 outbreak on in-hospital care of patients with CHD in an Italian tertiary centre.

We retrospectively reviewed data on CHD hospital admissions in our centre since 1 March, 2020, when the adoption of a strict infection containment policy throughout the country resulted in limited access of patients to routine hospital care and resources reallocation to the care of infected patients. Comparison with data from the previous year was performed in order to identify any relevant differences attributable to the outbreak. Despite cancellation of all elective procedures, the overall number of urgent hospital admission remained stable throughout the period of study. Patients admitted during the pandemic had greater disease complexity (p = 0.001) with longer length of in-hospital stay (p = 0.01). No adverse events or positive swabs were reported among CHD patients who were admitted to hospital or medical personnel caring for these patients.

Data from our early experience suggest that coronavirus disease 2019 pandemic did not impact significantly on the provision of urgent care to adult patients with CHD.

CHDs form a complex and heterogeneous group of clinical entities, with high morbidity and mortality. With the advancement of surgical and invasive techniques and clinical treatment, the survival of these patients has increased significantly, and there are reports of a high prevalence of ocular abnormalities in this group. The objective of this study was to estimate the prevalence of ocular findings in children and adolescents diagnosed with CHD.

A systematic search was conducted in the following databases: MEDLINE (via PubMed), EMBASE, and Cochrane CENTRAL, in addition to a manual search on studies published on the patient, from inception until August, 2014. Observational studies assessing the prevalence of ocular abnormalities in children and adolescents with CHDs were included.

Of the 2413 articles identified, eight were included, comprising a total of 1061 patients. Among them, the lowest and highest prevalences observed were 6.3 and 65%, respectively. The weighted average prevalence of ocular abnormalities was 32.5% (CI95% 19.3–49.3). Strabismus, cataracts, and retinopathy were the most frequently observed alterations.

The prevalence of ocular abnormalities in children and adolescents with CHDs was 32.5%, demonstrating that ocular consequences are not uncommon in this population and may have relevant clinical impact. These results reinforce the need for ophthalmological evaluation of patients with CHDs.

To identify interstage best practices associated with lower mortality, we studied National Pediatric Cardiology Quality Improvement Collaborative centres registry using a positive deviance approach.

Positive deviant and control centre team members were interviewed to identify potential interstage best practices. Subsequently, all collaborative centres were surveyed on the use of these practices to test their associations with centre mortality. Questionnaires were scored using Likert scales; the overall score was linearly transformed to a 0–100-point scale with higher scores indicating increased use of practices. Mortality was based on patients enrolled after a centre’s first year in the collaborative. Centre mortality rates were divided into tertiles. Survey scores for the low mortality tertile were compared with the other tertiles.

For this study, seven positive deviant and four control teams were interviewed. A total of 20 potential best practices were identified, including team composition, improvement practices, and parent involvement. Questionnaires were completed by 36/43 eligible centres, providing 1504 patients for analysis. Average survey score was 50.2 (SD 13.4). Average mortality was 6.1% (SD 4.1). There was no correlation between survey scores and mortality (r=0.14, p=0.41). The one practice associated with the low mortality tertile was frequency of discussion of interstage results: 58.3% of low mortality teams discussed results at least monthly versus 8.4% of the middle and high tertile centres (p=0.02).

Low-mortality centres more frequently discuss interstage results than high-mortality centres. Heightened awareness of outcomes may influence practice; however, further study is needed to understand the variation in outcomes across centres.