The American College of Cardiology has published clinical practice algorithms for common congenital heart lesions, including atrial septal defect, patent ductus arteriosus, valvar pulmonary stenosis, aortic coarctation, and ventricular septal defect. The purpose of this study was to define the current practice patterns in the management of these lesions and describe the impact of departure from these recommendations.

This was a retrospective analysis of the most recent 100 outpatient appointments for each lesion at our centre. Electronic medical records were queried to determine whether the scheduling, testing, and follow-up plan for each appointment were consistent with the published algorithms.

A total of 500 visits were evaluated (150 new visits; 350 follow-up visits); 32% (n = 162) of encounters did not receive appropriate testing, 37% (n = 186) departed from recommended follow-up plans, and of the 350 follow-up visits, 45% (n = 156) departed from scheduling guidelines. Impact of these departures was quantified in reference to over- or under-expenditure of clinical resources. Of the aberrant testing encounters, 60% (n = 97) saw too few tests. Of the deviant follow-up plans created, 74% (n = 138) brought patients back to clinic too soon.

This study explores the deviation between current practice patterns and published clinical care guidelines. There is considerable variation across domain of analysis, diagnosis, and encounter type, resulting in uneven resource utilisation. Standardisation of care in these areas will improve utilisation and can be a starting point for improvement work.

Kawasaki disease is a systemic vasculitis that primarily affects young children and represents a major cause of acquired heart disease in children in developed countries. The incidence of Kawasaki disease exhibits significant global variation, and the worldwide burden remains limited.

A systematic review was conducted to investigate the global incidence of Kawasaki disease in children under 5 years of age. A comprehensive literature search was performed in PubMed, Embase, and KoreaMed up to July 15, 2024. Studies reporting population-level Kawasaki disease incidence were included. Data extraction and quality assessment were performed independently by two reviewers.

The search yielded 3,197 articles, of which 105 met the inclusion criteria. These studies examined Kawasaki disease incidence in children under 5 years of age across 34 countries, with the majority focusing on the Western Pacific Region and the Region of the Americas. The results demonstrated a wide range of Kawasaki disease incidence globally, with significant geographic variations. The highest incidence rates were observed in Japan, Korea, and Taiwan, with a trend of gradual increase over time.

This study represents the most comprehensive review of global Kawasaki disease incidence to date. The substantial variation in incidence underscores the need to understand the factors influencing regional differences.

Children with congenital adrenal hyperplasia are considered to be at an elevated risk for cardiovascular morbidity and mortality. The objective of this study was to evaluate the association between periaortic fat thickness and the cardiometabolic profile in children diagnosed with congenital adrenal hyperplasia.

A total of 20 children and adolescents with congenital adrenal hyperplasia and 20 healthy control subjects were enrolled in the study. We investigated metabolic and anthropometric parameters, comparing these values to those of the control group. Periaortic fat thickness was assessed using an echocardiographic method that has not previously been applied to paediatric patients with congenital adrenal hyperplasia.

The subjects in the congenital adrenal hyperplasia group were significantly shorter than the control subjects (p = 0.021) and exhibited a higher body mass index (p = 0.044) and diastolic blood pressure (p = 0.046). No significant differences were observed between the congenital adrenal hyperplasia group and control subjects concerning age, weight, high-density lipoprotein cholesterol, and low-density lipoprotein cholesterol levels. Additionally, dyslipidemia was identified in 5% (N = 1) of the congenital adrenal hyperplasia group. The mean fasting glucose, fasting insulin, homeostasis model assessment of insulin resistance, and fasting glucose-to-fasting insulin ratio were similar between the congenital adrenal hyperplasia group and the control subjects. However, 15% (n = 3) of the congenital adrenal hyperplasia group had insulin resistance. Two children with congenital adrenal hyperplasia (10%) were diagnosed with hypertension.

Periaortic fat thickness was significantly greater in the congenital adrenal hyperplasia group compared to the control group (p = 0.000), with measurements of 0.2039 ± 0.045 mm in the congenital adrenal hyperplasia group and 0.1304 ± 0.022 mm in the control group. In children with congenital adrenal hyperplasia, periaortic fat thickness exhibited a negative correlation with high-density lipoprotein cholesterol (r = −0.549, p = 0.034) and a positive correlation with the dose of hydrocortisone (r = 0.688, p = 0.001).

Our results provide further evidence of subclinical cardiovascular disease in children with congenital adrenal hyperplasia. It is crucial to regularly assess cardiometabolic risk in children with congenital adrenal hyperplasia. The measurement of periaortic fat thickness in this population may serve as a valuable tool for identifying individuals at high risk for developing early atherosclerosis.

A subgroup of CHDs can only be treated palliatively through a Fontan circulation. In case of a failing Fontan situation, serum proteins are lost unspecifically and can also lead to a loss of vaccine antibodies. In a failing Fontan situation, heart transplantation may be the only feasible option.

We describe a 17-year-old patient born with a hypoplastic left heart complex, who underwent Fontan completion at the age of 4 years and developed a failing Fontan physiology. Therefore, a Fontan takedown with creation of a reverse 1½-circulation was performed. Multiple exacerbations of protein losing enteropathy occurred with a hypoproteinaemia, hypalbuminaemia, and hypogammaglobulinaemia. The patient was hospitalised several times and treated with intravenous application of immunoglobulins and albumin for symptom control. Before one of this substitutions, the immunoglobulin G against measles, mumps, and rubella was determined: the patient’s serum demonstrated a positive measles and rubella, but mumps was negative. After administration of the iv-therapy, the lacking antibodies were replenished, and there was a positive test for mumps.

Serum samples were analysed by neutralisation test and enzyme-linked immunosorbent assay (ELISA).

Although the patient had been vaccinated according to national guidelines, we saw an intermittent immune deficiency for mumps, but not for rubella and measles. For patient with a failing Fontan circulation, we recommend to test to vaccine antibodies for mumps, measles, and rubella with an ELISA an if its negative with a neutralisation test, especially in view of a possible heart transplantation to find a possible immune deficiency.

Understanding complex three-dimensional cardiac structures is the key to knowing CHD. Many learners have limited access to cadaveric specimens, and most alternative teaching modalities are two-dimensional. Therefore, we have developed virtual cardiac models using photogrammetry of actual heart specimens to address this educational need.

A descriptive study was conducted at a single institution during a week-long cardiac morphology conference in October 2022 and 2023. Conference attendees viewed virtual cardiac models via laptop screen and virtual reality headset. Learners were surveyed on their opinions of the virtual models and their perceived effectiveness compared to existing educational materials.

Forty-six learners completed the survey. Participants reported the virtual cardiac models to be more effective than textbook diagrams (60%), and equally or more effective compared to didactic teaching (78%) and specimen videos (78%). Approximately half of participants (54%) found the virtual models to be less effective than hands-on cadaveric specimen inspection. Attitudes towards the virtual specimens were overall positive with most responders finding the tool engaging (87%) and enjoyable (85%). A majority reported that the models deepened their understanding of cardiac morphology (79%) and that they would recommend them to other trainees (87%).

This study demonstrates that a novel teaching tool, virtual cardiac specimens, is equivalent to or more effective than many current materials for learning cardiac morphology. While they may not replace direct cadaveric specimen review, virtual models are an engaging alternative with the ability to reach a wider audience.

Cardiac catheterisation in the postoperative period emerges as a primary tool, providing effectiveness and safety in diagnosis, treatment guidance, and resolution of major residual lesions.

This is a retrospective case-control study. We collected the clinical records of patients who underwent cardiac catheterisation between January 2003 and December 2022 within the initial 72 hours after surgery in the pediatric cardiac ICU of a national referral hospital in Mexico City. Descriptive, univariate, and multivariate analyses were performed.

A total of 6,243 surgeries were performed, of which 264 were cardiac catheterizations carried out within the first 72 hours of the postoperative period;these included 73 diagnostic procedures and 191 interventional procedures. One hundred and thirty-five (135) catheterisations targeted recent suture intervention sites. The primary indications for cardiac catheterisation included low cardiac output and the suspicion of major residual lesions. Approximately 65% of interventions occurred within the first 24 hours and solved 426 residual lesions. No significant associations were found between mortality, complications, and the need for surgical reintervention in patients who underwent interventional catheterisation at recent suture sites (OR 1.93;95% CI:0.94–4.07:p = 0.076). Seventeen patients (17) were extubated in the initial 24 hours post-catheterisation. Two major complications were identified: rupture of the systemic-to-pulmonary shunt in the anastomosis, and a pulmonary artery laceration requiring emergency surgery. One patient died.

Cardiac catheterisation has evolved into a vital instrument to diagnose and resolve abnormalities and significant residual lesions without increasing the morbidity and mortality risks.

In North America, less than 30% of children with complex CHD receive recommended follow-up for neurodevelopmental and psychosocial care. While rates of follow-up care at surgical centres have been described, little is known about similar services outside of surgical centres.

This cohort study used Maine Health Data Organization’s All Payer Claims Data from 2015 to 2019 to identify developmental and psychosocial-related encounters received by children 0–18 years of age with complex CHD. Encounters were classified as developmental, psychological, and neuropsychological testing, mental health assessment interventions, and health and behaviour assessments and interventions. We analysed the association of demographic and clinical characteristics of children and the receipt of any encounter.

Of 799 unique children with complex CHD (57% male, 56% Medicaid, and 64% rural), 185 (23%) had at least one developmental or psychosocial encounter. Only 13 children (1.6%) received such care at a surgical centre. Developmental testing took place at a mix of community clinics/private practices (39%), state-based programmes (31%), and hospital-affiliated clinics (28%) with most encounters billing Medicaid (86%). Health and behavioural assessments occurred exclusively at hospital-affiliated clinics, predominately with Medicaid claims (82%). Encounters for mental health interventions, however, occurred in mostly community clinics/private practices (80%) with the majority of encounters billing commercial insurance (64%).

Children with complex CHD in Maine access developmental and psychosocial services in locations beyond surgical centres. To better support the neurodevelopmental outcomes of their patients, CHD centres should build partnerships with these external providers.

A series of webinars covering widespread knowledge on paediatric cardiology and cardiac surgery topics was initiated by Association for European Paediatric and Congenital Cardiology, serving towards preparation for the Association for European Paediatric and Congenital Cardiology certification in paediatric and congenital cardiology. This study investigated the impact of webinars as educational tools for junior paediatric cardiologists in the post-COVID-19 pandemic era.

A cross-sectional survey design study using an online survey as a tool for the assessment of trainees. An open and closed-ended SurveyMonkey questionnaire was used to document the learners’ opinions on webinars. Results were reported using descriptive statistical analyses.

Twenty-seven Association for European Paediatric and Congenital Cardiology junior members participated in the online survey from twelve different countries. Most of the participants were trainees in paediatric cardiology (56%), and the remainder were junior consultants in paediatric cardiology. Approximately 70% found no difficulties in participating in the webinars. The webinars were appreciated by participants, who found the webinars interactive and highly educational with contents highly applicable to everyday clinical practice. Significant heterogeneity emerged in training programmes across Europe and worldwide in terms of programme duration, number of fellows, teaching approach, and assessments. Training opportunities such as courses, grants, and more webinars were suggested as tools to support continuous learning by the Association for European Paediatric and Congenital Cardiology.

The Association for European Paediatric and Congenital Cardiology webinar series has confirmed the crucial role of online-based learning resources in the new generation of junior paediatric cardiologists. Association for European Paediatric and Congenital Cardiology webinars and the examination in paediatric cardiology may help standardise training across Europe, promoting the highest standards in patient care.