CHD refers to structural cardiac abnormalities which comprise the commonest group of congenital malformations. Malta is a small island in the central Mediterranean with excellent diagnostic and therapeutic facilities. It is unique in the European population as termination of pregnancy is illegal. This study was carried out to ascertain patterns in CHD prevalence in comparison with EUROCAT data (European Surveillance of Congenital Anomalies).

Anonymised data were obtained from the EUROCAT website for 1993–2020.

There were a total of 22,833,032 births from all EUROCAT Registries, of which 121,697 were from Malta. The prevalence rate for Malta CHD was 32.38/10,000 births (at the higher end of the range). Malta had a significant excess of commoner, comparatively non-severe CHDs. For most of the severe lesions analysed rates reported were higher than EUROCAT average, however, apart from Ebstein’s anomaly, they all fell within the ranges reported from the different registries.

Wide variations in reported CHD prevalence are known, and the Malta rates may be higher for milder defects due to quicker pickup prior to spontaneous resolution. There may also be a higher pickup of milder forms of more severe conditions. For the more severe conditions, lack of termination may be the explanation. These factors may result in the higher neonatal mortality observed in Malta.

Handgrip strength is a crucial indicator of upper extremity muscular strength and is vital for monitoring disorders like cardiac diseases that restrict a patient’s physical activity and result in muscle atrophy. The aim of our study was to evaluate whether muscle strength loss is present in patients with pulmonary hypertension and whether this test can be an alternative to 6-minute walk test.

The study included 39 healthy children who were admitted to the outpatient clinic and 16 children with a diagnosis of pulmonary hypertension who were being followed in our centre. We assessed the differences in upper extremity handgrip strength using the Jamar Hydraulic Hand Dynamometer device among both healthy children and those diagnosed with pulmonary hypertension. Moreover, we compared the handgrip strength of pulmonary hypertension patients with significant prognostic indicators such as NYHA class, 6-minute walk test, and pro-brain natriuretic peptide.

The mean dominant handgrip strength was 20.8 ± 12 kg in the patient group and 21.6 ± 12.4 kg in the control group (p = 0.970). Handgrip strength was shown to be negatively connected with pro-brain natriuretic peptide (r = −0.565, p = 0.023) and positively correlated with 6-minute walk test (r = 0.586, p = 0.022) during the patient group evaluation.

Six-minute walk test needs a customised physical area (30 m of a straight hallway) and trained personnel for applying the test. The handgrip strength test, a different muscle strength indicator, can be used to more clearly and simply indicate the decline in patients’ ability for effort. Additionally, it was found in our study that handgrip strength decreased as pro-brain natriuretic peptide levels rose, a crucial measure in the monitoring of pulmonary hypertension.

Cardiac arrhythmias are a major concern in patients with CHD. The purpose of this study was to evaluate the long-term outcomes in patients with CHD submitted to catheter ablation.

Observational retrospective study of patients with CHD referred for catheter ablation from January 2016 to December 2021 in a tertiary referral centre. Acute procedural endpoints and long-term outcomes were assessed.

A total of 44 ablation procedures were performed in 36 CHD patients (55% male, mean age 43 ±3 years). Fifty-four arrhythmias were ablated: 23 cavotricuspid isthmus atrial flutters, 10 atrial re-entrant tachycardias, eight focal atrial tachycardias, eight atrial fibrillations, three atrioventricular re-entrant tachycardias, and two ventricular tachycardias. During a median follow-up time of 37 months (interquartile range 12–51), freedom from arrhythmia recurrence was achieved in 93%, with 1.2 procedures per patient (18% with anti-arrhythmic drugs). There were no adverse events related to catheter ablation. No predictors of recurrence were identified.

In patients with CHD, catheter ablation presents a high mid-term efficacy while maintaining a safe profile.

Echocardiography is a key diagnostic tool for medical decision-making following congenital heart surgery. Overall utilisation of echocardiography for specific congenital heart lesions following cardiac surgery has not previously been reported. This study aims to assess echocardiogram utilisation following the surgical repair of CHD to describe the variation in use across centres and provide clinical benchmarks.

All patients < 18 years of age undergoing surgical repair of CHD were identified from the Pediatric Health Information System from 2010 to 2019. Surgeries were grouped based on their Risk Adjustment for Congenital Heart Surgery-1 scores. Detailed billing data were used to assess the frequency/cost of post-operative echocardiograms, phase of hospital care, and hospital length of stay.

In total, 37,238 surgical encounters were identified for inclusion across 48 centres. Higher Risk Adjustment for Congenital Heart Surgery scores were associated with an increased median number of post-operative echocardiograms (2 versus 4 in Risk Adjustment for Congenital Heart Surgery score 1 versus 6, p < 0.001), and longer median post-operative length of stay (3 days versus 31 days in Risk Adjustment for Congenital Heart Surgery score 1 versus 6, p < 0.001). After accounting for surgical complexity, there was significant variability in echocardiogram utilisation across centres (median daily echocardiogram utilisation range 0.2/day–0.6/day, p < 0.001). There is no difference in the proportion of patients with high surgical complexity (Risk Adjustment for Congenital Heart Surgery ≥ 4) between centres with high versus low echocardiogram utilisation (p = 0.44).

Increasing surgical complexity is associated with longer post-operative length of stay and increased utilisation of echocardiography. There is wide variability in echocardiography resource utilisation across centres, even when accounting for surgical complexity.

Informed consent for surgery is a complex process particularly in paediatrics. Complexity increases with procedures such as CHD surgery. Regulatory agencies outline informed consent contents for surgery. We assessed and described CHD surgical informed consent contents through survey dissemination to paediatric CHD centres across United States of America.

Publicly available email addresses for 125 paediatric cardiac clinicians at 70 CHD surgical centres were obtained. Nine-item de-identified survey assessing adherence to The Joint Commission informed consent standards was created and distributed via RedCap® 14 March, 2023. A follow-up email was sent 29 March, 2023. Survey link was closed 18 April, 2023.

Thirty-seven surveys were completed. Results showed informed consent documents were available in both paper (25, 68%) and electronic (3, 8%) format. When both (9, 24%) formats were available, decision on which format to use was based on centre protocols (1, 11%), clinician personal preference (3, 33%), procedure being performed (1, 11%), or other (4, 45%). Five (13%) centres’ informed consent documents were available only in English, with 32 (87%) centres also having a Spanish version. Review of informed consent documents demonstrated missing The Joint Commission elements including procedure specific risks, benefits, treatment alternatives, and expected outcomes.

Informed consent for CHD surgery is a complex process with multiple factors involved. Majority of paediatric CHD surgical centres in the United States of America used a generic informed consent document which did not uniformly contain The Joint Commission specified information nor reflect time spent in discussion with families. Further research is needed on parental comprehension during the informed consent process.

An alternative surgical approach for hypoplastic left heart syndrome is the Hybrid pathway, which delays the risk of acute kidney injury outside of the newborn period. We sought to determine the incidence, and associated morbidity, of acute kidney injury after the comprehensive stage 2 and the cumulative incidence after the first two operations in the Hybrid pathway.

A single centre, retrospective study was conducted of hypoplastic left heart patients completing the second-stage palliation in the Hybrid pathway from 2009 to 2018. Acute kidney injury was defined utilising Kidney Diseases Improving Global Outcomes criteria. Perioperative and post-operative characteristics were analysed.

Sixty-one patients were included in the study cohort. The incidence of acute kidney injury was 63.9%, with 36.1% developing severe injury. Cumulatively after the Hybrid Stage 1 and comprehensive stage 2 procedures, 69% developed acute kidney injury with 36% developing severe injury. The presence of post-operative acute kidney injury was not associated with an increase in 30-day mortality (acute kidney injury 7.7% versus none 9.1%; p = > 0.9). There was a significantly longer median duration of intubation among those with acute kidney injury (acute kidney injury 32 (8, 155) hours vs. no injury 9 (0, 94) hours; p = 0.018).

Acute kidney injury after the comprehensive stage two procedure is common and accounts for most of the kidney injury in the first two operations of the Hybrid pathway. No difference in mortality was detected between those with acute kidney injury and those without, although there may be an increase in morbidity.

To explore how the COVID-19 pandemic affected fetal care, social support, and emotional functioning for women carrying a fetus with CHD.

This was a single-centre qualitative study of 31 women who received a prenatal diagnosis of CHD during the pandemic. Patients completed semi-structured interviews about their experiences with fetal care, social support, and perceptions of risk to themselves and their fetus. Consistent themes regarding the impact of the pandemic were identified using an inductive thematic approach. Demographic data were collected via self-report and chart review.

Women generally reported consistent access to fetal care throughout the pandemic, with frequent use of telemedicine in addition to in-person care, but negative impacts resulting from restrictions on family support at appointments. Limited access to social support overall and a loss of pregnancy traditions were described. Many women reported feeling isolated and experiencing worries and fears about COVID-19 but also noted feeling supported by their healthcare team. Partner/family support during appointments and connection to peer-to-peer support were identified as recommendations to mitigate negative impacts.

Women carrying a fetus with CHD during the COVID-19 pandemic experienced unique stressors that may affect mental health. However, many also experienced unexpected supports that may mitigate effects of pandemic-related stressors. Results can inform efforts to promote positive family outcomes during and following the COVID-19 pandemic.

The most frequent abnormality of the valves involved in rheumatic heart disease is mitral regurgitation. A promising supportive diagnostic tool for rheumatic heart disease is the N-terminal pro-brain natriuretic peptide, which can identify mitral valve damage.

To prove a positive correlation between N-terminal pro-brain natriuretic peptide levels and the severity of mitral regurgitation or accompanied by mild aortic valve dysfunction in children with rheumatic heart disease.

Children aged 1–18 years who had been identified as having rheumatic heart disease with a single mitral regurgitation or accompanied by mild aortic issues at Sanglah General Hospital, Denpasar, met the inclusion criteria for this cross-sectional study. Mitral regurgitation severity was determined using the parameters of vena contracta width, effective regurgitation orifice area, regurgitant jet area, and regurgitation volume. Bivariate analysis using the Spearman correlation test.

From 36 research subjects, the mean age was 11.32 years. In this study, there was a moderate positive correlation between the levels of N-terminal pro-brain natriuretic peptide and the regurgitation jet area. N-terminal pro-brain natriuretic peptide levels also had a moderately positive correlation with the vena contracta width and a weakly positive correlation with the regurgitation volume. Effective regurgitation orifice area and N-terminal pro-brain natriuretic peptide levels did not significantly correlate.

There was a moderately positive correlation between N-terminal pro-brain natriuretic peptide levels and the severity of mitral regurgitation on regurgitation jet area, a moderately positive correlation with the vena contracta width, and a weakly positive correlation with regurgitation volume in rheumatic heart disease patients.

With the rise of online references, podcasts, webinars, self-test tools, and social media, it is worthwhile to understand whether textbooks continue to provide value in medical education, and to assess the capacity they serve during fellowship training.

A prospective mixed-methods study based on surveys that were disseminated to seven paediatric cardiology fellowship programmes around the world. Participants were asked to read an assigned chapter of Anderson’s Pediatric Cardiology 4th Edition textbook, followed by the completion of the survey. Open-ended questions included theming and grouping responses as appropriate.

The survey was completed by 36 participants. When asked about the content, organisation, and utility of the chapter, responses were generally positive, at greater than 89%. The chapters, overall, were rated relatively easy to read, scoring at 6.91, with standard deviations plus or minus 1.72, on a scale from 1 to 10, with higher values meaning better results. When asked to rank their preferences in where they obtain educational content, textbooks were ranked the second highest, with in-person teaching ranking first. Several themes were identified including the limitations of the use of textbook use, their value, and ways to enhance learning from their reading. There was also a near-unanimous desire for more time to self-learn and read during fellowship.

Textbooks are still highly valued by trainees. Many opportunities exist, nonetheless, to improve how they can be organised to deliver information optimally. Future efforts should look towards making them more accessible, and to include more resources for asynchronous learning.

Previous reports indicate bone deficits in patients with Fontan circulation. However, the consequences of these deficits on bone strength and when these changes occur are unclear.

To compare the tibial bone strength-strain index between young patients (6–19 years) with Fontan circulation and age- and sex-matched controls, and to determine strength-strain-index in subgroups of children (6–12 years) and adolescents (13–19 years) versus controls.

The tibia was examined with peripheral quantitative CT. Based on the assessed data, bone strength-strain index was calculated in the lateral and anterior-posterior directions.

Twenty patients with Fontan and twenty controls (mean age 13.0 ± 4.4 years; 50% females) were examined. Patients had a lower strength-strain index in the lateral direction compared to controls (808.4 ± 416.8mm3 versus 1162.5 ± 552.1mm3, p = 0.043). Subgroup analyses showed no differences regarding strength-strain index in children (6–12 years) with Fontan circulation compared to controls. However, the adolescents (13–19 years) with Fontan circulation had lower strength-strain indexes in both the lateral and anterior-posterior directions compared to controls (1041.4 ± 299.8mm3 versus 1596.4 ± 239.6mm3, p < 0.001, and 771.7 ± 192.4mm3 versus 1084.9 ± 215.0mm3, p = 0.004). When adjusted for height, there were differences between patients (6–19 years) and controls in strength-strain indexes in both the lateral and anterior-posterior directions. In subgroup analyses, the results remained robust.

Young patients (6–19 years) with Fontan circulation have a lower strength-strain index in the tibia compared to controls. Subgroup analyses show that this deficit is mainly driven by the differences in adolescents (13–19 years), which might suggest that bone strength decreases with age.

CHD is a unique group of medical pathologies. Literature worldwide reports significant decrements in the case volume of patients with these conditions due to the recent global pandemic of coronavirus disease 2019. The only centre providing congenital cardiac care for Lithuanian population is in a hospital which was the main medical institution for the sickest coronavirus disease 2019 patients. Hence, this centre had to maintain its service alongside the mobilisation of resources to tackle the crisis.

To evaluate the effect of the pandemic on the service of congenital heart surgery in Lithuania.

The activity of a single centre providing congenital heart care working in a main coronavirus 2019 pandemic hospital during the pandemic was analysed and compared to a matched period of pre-pandemic activity.

The number of admitted patients was similar during both pre-pandemic and pandemic periods. During the pandemic period, younger patients were more often operated as urgent cases. Their postoperative length of stay was longer. However, there were no differences in early postoperative mortality between the two groups.

It was possible to maintain an accessible and high-quality specialised congenital cardiac care for various age patients during global pandemic events, while working in the main pandemic hospital.

Children who develop coronary artery aneurysms after Kawasaki disease are at risk for cardiovascular morbidity, requiring health care transition and lifelong follow-up with an adult specialist. Follow-up losses after health care transition have been reported but without outcome and patient experience evaluation.

The Theoretical Domains Framework underpinned our aim to explore the required self-care behaviours and experiences of young adults’ post-health care transition.

A qualitative description approach was used for virtual, 1:1 interviews with 11 participants, recruited after health care transition from a regional cardiac centre in Ontario. Directed content analysis was employed.

Health, psychosocial, and lifestyle challenges were compounded by a sense of loss. Six themes emerged within the Theoretical Domains Framework categories. Participants offered novel health care transition programme recommendations.

The realities of health care transition involve multiple, overlapping stressors for young adults with Kawasaki disease and coronary artery aneurysms. Our findings will inform a renewed health care transition programme and will include outcome evaluation.

Cardiac hypertrophy, acting as a pathologic process of chronic hypertension and coronary disease, and its underlying mechanisms still need to be explored. Long non-coding RNA (LncRNA) potassium voltage-gated channel subfamily Q member 1 Transcript 1 (KCNQ1OT1) has been implicated in myocardial infarction. However, its role in cardiac hypertrophy remains reported.

To explore the regulated effect of lncRNAKCNQ1OT1 and miR-301b in cardiac hypertrophy, gain-and-lose function assays were tested. The expression of lncRNAKCNQ1OT1 and miR-301b were tested by quantitative real time polymerase chain reaction (qRT-PCR). The levels of transcription factor 7 (Tcf7), Proto-oncogene c-myc (c-myc), Brainnatriureticpeptide (BNP) and β-myosin heavy chain (β-MHC) were detected by Western blot. Additionally, luciferase analysis revealed interaction between lncRNAKCNQ1OT1, BNPβ-MHCmiR-301b, and Tcf7.

LncRNAKCNQ1OT1 overexpression significantly induced cardiac hypertrophy. Furthermore, lncRNAKCNQ1OT1 acts as a sponge for microRNA-301b, which exhibited lower expression in cardiac hypertrophy model, indicating an anti-hypertrophic role. Furthermore, the BNP and β-MHC expression increased, as well as cardiomyocyte surface area, with Ang II treatment, while the effect was repealed by miR-301b. Moreover, the protein expression of Tcf7 was inversely regulated by miR-301b and Antisense miRNA oligonucleotides (AMO)-301b.

Our study has shown that overexpression of lncRNAKCNQ1OT1 could promote the development of cardiac hypertrophy by regulating miR-301b and Tcf7. Therefore, inhibition of lncRNAKCNQ1OT1 might be a potential therapeutic strategy for cardiac hypertrophy.

There are limited studies with medium-term follow-up following percutaneous pulmonary valve implantation and no studies with a gender-specific analysis.

To report clinical outcomes up to five years following percutaneous pulmonary valve implantation using the two most common balloon expandable valves in a mixed population of paediatric and adult patients with an age and gender-specific analysis.

This was a single-centre retrospective observation study. Relevant data were obtained retrospectively from the case files. Age and gender- specific analysis was performed using SPSS.

Totally, 58 patients (13 children, 45 adults) underwent percutaneous pulmonary valve implantation. Statistically significant reduction in median right ventricular outflow tract flow velocity following valve implantation was maintained for the whole five years in adults but not in children. There were no gender-specific differences despite the study being adequately powered. Independent of valve type used, there was significant reduction of the right ventricular outflow tract flow velocity in the immediate post valve implantation period (Edwards P = 0.001, Melody P = 0.013). There was a significant negative correlation between implanted valve Z-score and subsequent right ventricular outflow tract gradient during the first two years following valve implantation.

Gender does not significantly affect valve function following percutaneous pulmonary valve implantation. It is important to consider patients’ age and body surface area in relation to existing right ventricular outflow tract size during decisions for percutaneous pulmonary valve implantation.

Coronary artery aneurysms have been considered the most serious complication of Kawasaki disease. However, some coronary artery aneurysms do regress. Therefore, the ability to predict the expected time of coronary artery aneurysm regression is critical. Herein, we have created a nomogram prediction system to determine the early regression (<1 month) among patients with small to medium coronary artery aneurysms.

Seventy-six Kawasaki disease patients identified with coronary artery aneurysms during the acute or subacute phase were included. All the patients who met inclusion criteria demonstrated regression of coronary artery aneurysms within the first-year post Kawasaki disease diagnosis. The clinical and laboratory parameters were compared between the groups of coronary artery aneurysms regression duration within and beyond 1 month. Multivariate logistic regression analysis was used to identify the independent parameters for early regression based on the results from the univariable analysis. Then nomogram prediction systems were established with associated receiver operating characteristic curves.

Among the 76 included patients, 40 cases recovered within 1 month. Haemoglobin, globulin, activated partial thromboplastin time, the number of lesions, location of the aneurysm, and coronary artery aneurysm size were identified as independent factors for early regression of coronary artery aneurysms in Kawasaki disease patients. The predictive nomogram models revealed a high efficacy in predicting early regression of coronary artery aneurysms.

The size of coronary artery aneurysms, the number of lesions, and the location of aneurysms presented better predictive value for predicting coronary artery aneurysms regression. The nomogram system created from the identified risk factors successfully predicted early coronary artery aneurysm regression.

Critical CHD is associated with morbidity and mortality, worsened by delayed diagnosis. Paediatric residents are front-line clinicians, yet identification of congenital CHD remains challenging. Current exposure to cardiology is limited in paediatric resident education. We evaluated the impact of rapid cycle deliberate practice simulation on paediatric residents’ skills, knowledge, and perceived competence to recognise and manage infants with congenital CHD.

We conducted a 6-month pilot study. Interns rotating in paediatric cardiology completed a case scenario assessment during weeks 1 and 4 and participated in paired simulations (traditional debrief and rapid cycle deliberate practice) in weeks 2–4. We assessed interns’ skills during the simulation using a checklist of “cannot miss” tasks. In week 4, they completed a retrospective pre-post knowledge-based survey. We analysed the data using summary statistics and mixed effect linear regression.

A total of 26 interns participated. There was a significant increase in case scenario assessment scores between weeks 1 and 4 (4, interquartile range 3–6 versus 8, interquartile range 6–10; p-value < 0.0001). The percentage of “cannot miss” tasks on the simulation checklist increased from weeks 2 to 3 (73% versus 83%, p-value 0.0263) and from weeks 2–4 (73% versus 92%, p-value 0.0025). The retrospective pre-post survey scores also increased (1.67, interquartile range 1.33–2.17 versus 3.83, interquartile range 3.17–4; p-value < 0.0001).

Rapid cycle deliberate practice simulations resulted in improved recognition and initiation of treatment of simulated infants with congenital CHD among paediatric interns. Future studies will include full implementation of the curriculum and knowledge retention work.

The increasing global popularity of smartphone usage has increased concerns about the negative effects of smartphone addiction, such as lack of sleep, sedentary life, bad eating habits, anxiety, stress, and depression, especially among the young population. These problems caused by smartphone addiction are also well-known risk factors for atherosclerosis. However, according to our observation, there is no research in the literature that directly shows the relationship between smartphone addiction and subclinical atherosclerosis. In this study, carotid intima-media thickness, an important surrogate marker in demonstrating subclinical atherosclerosis, was used to examine the relationship between smartphone addiction and subclinical atherosclerosis.

This cross-sectional study was conducted on 96 high school students aged between 13 and 22 years. A smartphone addiction questionnaire consisting of 33 questions was applied to measure smartphone addiction. Along with the socio-demographic characteristics of the patients, factors such as eating habits, sleep patterns, and activity levels were also questioned. The mean carotid intima-media thickness was measured by an experienced sonographer according to the published and accepted methods.

When we set the threshold for smartphone addiction at over 66 points, we discovered that the group with smartphone addiction had considerably thicker carotid intima-media (0.68 ± 0.2 versus 0.45 ± 0.1; p < 0.001). In addition, logistics regression analysis had shown that smartphone addiction level independently affects the carotid intima-media thickness (odds ratio:1.111; %95 GA:1.057–1.168, p < 0.001).

Smartphone addiction may help prediction of subclinical atherosclerosis via carotid intima-media thickness among teenagers.