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.

Although global knowledge on paediatric cardiomyopathies has advanced, prospective cohort studies from Brazil, particularly those integrating clinical and genetic data, remain limited.

To describe the clinical and genetic characteristics of paediatric cardiomyopathy patients and identify mortality predictors in a metropolitan region of Brazil.

Prospective observational study of paediatric patients with cardiomyopathies. Clinical data, genetic findings, and survival were analysed using Kaplan–Meier curves.

A total of 45 cases, male predominance (55.6%), and mean age at diagnosis of 6.5 years. Dilated and hypertrophic cardiomyopathy were the most common (33.3%). The main reason for diagnosis was the investigation of cardiovascular symptoms (60.9%). Genetic investigation occurred in 66.6%, a positivity rate of 60%. Multi-organ/system involvement was significantly associated with a positive genetic result (77.7%, p = 0.017). Mortality was 11.1%; survival was significantly lower in the following conditions: ejection fraction < 30% (p < 0.0001), functional class III/IV (p < 0.0001), heart failure (p = 0.0091), use of three or more cardiovascular medications (p < 0.001), N-Terminal Pro-B-Type natriuretic peptide >1000pg/mL (p = 0.004), and heart transplant indication (p < 0.001).

These findings provide novel data in Brazil, highlight a high rate of positive genetic test, particularly among patients with systemic involvement and identify key clinical predictors of mortality to guide risk stratification and care.

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.

Central venous catheter use is not standardised in paediatric cardiology, yet it is associated with additional morbidity. We aimed to characterise variation in central venous catheter use and complications across paediatric acute care cardiology units.

This retrospective, multi-centre, and registry-based study examined all unique acute care cardiology encounters from February 2019 through September 2021 in the Paediatric Acute Care Cardiology Collaborative registry. Descriptive and comparative statistics were assessed for differences based on central venous catheter use. Multivariate logistic regression identified factors associated with increased line duration and use frequency.

About 35,379 encounters from 24 institutions were assessed. About one in five encounters had at least one central venous catheter (n = 7,524, 21.3%). Neonates and post-operative cardiothoracic surgery patients were more likely to have central venous catheters than not (28.9% of neonates with, versus 11.4% without; 58.4% of post-operative patients with, versus 35.7% without; p < 0.001). Most patients after STAT 4 procedures retained central venous access for over half of the acute care cardiology stay. Institutions with overall “low” central venous catheter utilisation rates (<20%) also used central venous access most often on STAT 4 patients (p < 0.0001). Complication rates for venous thrombus and central line-associated bloodstream infection were low (1.9% and 0.2%).

There is variable utilisation of central venous catheters across participating acute care cardiology units, though overall they are common vascular access modalities. Acute care cardiology units use central venous catheters more often in neonates, those after cardiac surgery, and in their higher-risk patients (i.e. after STAT 4 procedures).

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.