Kawasaki disease (KD) is an acute vasculitis affecting medium-sized vessels and characterized by fever lasting more than 5 days. Differential diagnosis of KD is challenging. Microribonucleic acids (miRNA/miR) have been widely studied as potential biomarkers for diagnosing KD. The objective of this study is to systematically review the clinical utility of miRNAs in KD. This systematic review followed the PRISMA 2020 guidelines. We included English-language, full-text observational studies enrolling adult and/or pediatric patients with KD (complete or incomplete) which quantified miRNA expression levels or genotyped single-nucleotide polymorphisms (SNPs) in miRNA genes. We searched the following databases: Embase, Medline Ultimate, PubMed, Scopus, and Web of Science. Each database was last searched on January 12, 2026. Thirty-six studies met the eligibility criteria and were included in this review. The most reproducible finding concerns miR-223 which has been repeatedly reported to be upregulated in KD patients compared with both healthy and febrile controls. Several miRNAs have been described to distinguish specific KD phenotypes, e.g., coronary artery lesion (CAL) formation and intravenous immunoglobulin (IVIG) responsiveness. The majority of investigated SNPs in miRNA genes were not associated with KD susceptibility; however, some associations were reported, including with KD phenotypes such as CAL formation. Conclusions: Multiple studies have shown dysregulation of miRNA expression in KD patients and the potential utility of miRNAs in diagnosing KD and identifying individuals at risk of CAL formation or IVIG resistance. Nevertheless, there is a need for methodological standardization in future research and for studies including multiethnic cohorts.

The use of the six‑lead AliveCor KardiaMobile (6LACKM) has not been thoroughly evaluated in children under three years of age. This report presents our initial experience with modified 6LACKM ECG acquisition techniques tailored to this population, including hand-assisted chest placement and adhesive electrodes adaptation, in various patient states and positions. In a prospective study, we aim to assess the feasibility of 6LACKM in this population and compare its diagnostic quality to standard 12‑lead ECG. Our preliminary experience demonstrate that high-quality tracings can be obtained with the proposed approaches, supporting the potential utility of 6LACKM in early pediatric electrocardiographic monitoring.

Heart rate asymmetry (HRA) is a nonlinear measure reflecting unequal contributions of heart rate decelerations and accele­rations to heart rate variability (HRV). This scoping review, conducted in line with the PRISMA extension for Scoping Reviews (PRISMA-ScR) guidelines, aimed to summarize current evidence on HRA in pediatric populations. A systematic search of PubMed, Scopus, Web of Science, Embase, and Google Scholar (2000–2025) identified four eligible studies involving 192 children aged 3–18 years, including healthy individuals and those with attention deficit/hyperactivity disorder (ADHD), depression, or cardiac conditions. All studies were single-center and used electrocardiography (1000 Hz); one used 24-hour Holter monitoring, whereas the others used 5-minute recordings. One study also used a Pneumonitor (250 Hz). HRA was assessed using Porta’s index, Guzik’s index, and Ehlers’ index. Some studies included orthostatic testing. The findings suggest HRA may help detect neurocardiac dysregulation in both healthy children and those with specific diagnoses and may serve as a prognostic tool. Further research is needed to confirm its clinical relevance.

Gorlin-Goltz syndrome (GGS) is a rare hereditary autosomal dominant condition. Cardiac fibromas are one of the clinical manifestations of this disease. The presence of these benign heart tumors may lead to life-threatening complications. We present the case of a 5-year-old girl with GGS and a large cardiac fibroma who had ventricular fibrillation during respiratory tract infection. Several options of treatment were considered in the secondary prevention of cardiac arrest. A partial resection of the tumor was performed to prevent recurrence of malignant arrhythmia. The loop recorder implanted postoperatively didn’t record any episodes of sustained ventricular arrhythmia in a 9-month follow-up. In conclusion, cardiac fibromas, especially with the presence of infections, increase risk of ventricular arrhythmias. Partial resection of tumor can be effective and relatively safe in the secondary prevention of ventricular fibrillation in patients with large cardiac fibromas with GGS.

BACKGROUND Neurofibromatosis type 1 (NF1) and Marfan syndrome (MFS) are genetically determined systemic disorders. Their simultaneous occurrence is exceptionally rare, with only a few cases reported in the literature. CASE REPORT A 13-year-old boy was admitted to a cardiology clinic due to mitral and tricuspid valve prolapse. He was tall, with severe scoliosis and distinctive dysmorphic features typical of MFS. More than 20 cafe-au-lait spots, characteristic of NF1, were present on his skin. The family history included NF1 in his twin brother, mother, maternal uncle, and maternal grandmother. The maternal uncle also exhibited phenotypic features of MFS and died at a young age from a suspected ruptured intracranial aneurysm. Genetic testing in our patient revealed an NF1 mutation, as well as a 16p13.11 microduplication that could explain his developmental delay and speech difficulties. The diagnosis of MFS was based on characteristic dysmorphic features and the presence of aortic root dilation, despite negative findings concerning the fibrillin-1 (FBN1) mutation typically associated with MFS. CONCLUSIONS A thorough assessment of physical features is essential to detect atypical phenotypes and recognize potential coexistence of multiple genetic syndromes. This case highlights the clinical importance of systematic cardiac evaluation, given that cardiac abnormalities typical of MFS may be critical for its recognition. Reporting such an unusual coexistence of NF1 and MFS underscores the need for multidisciplinary care to improve long-term outcomes in patients with overlapping genetic disorders.

Background/Objectives: Acute lymphoblastic leukemia (ALL) treatment is effective but involves the use of cardiotoxic anthracyclines. Data on three-dimensional (3D) strain echocardiography utility in the monitoring of acute and early-onset cardiotoxicity in children with ALL are scarce. The aim was to assess the left ventricular function in 3D strain echocardiography in children with ALL treated with anthracyclines in reference to a healthy cohort and to assess risk factors of possible subclinical cardiotoxicity. Methods: For this study, medical records from between May 2023 and December 2024 were retrospectively analyzed, and the echocardiographic data were reassessed. The study group consisted of children diagnosed and treated for ALL for at least 6 months with the use of daunorubicin and doxorubicin (or only with daunorubicin) who underwent routine cardiological evaluation with 3D echocardiography in >6 months and <2 years from the beginning of treatment. The echocardiographic records were reassessed offline to analyze the left ventricle 3D global longitudinal (GLS 3D), circumferential (GCS 3D) and radial (GRS 3D) strain. The results were compared with the results of 24 apparently healthy peers matched by sex and age. A set of models was developed to test literature-based subclinical cardiotoxicity determinants in relation to the obtained 3D strain values. Results: The study group consisted of 42 children (22 males and 20 females) aged 2–17 years (mean 7.7 ± 4.1 years) treated for ALL. The median time from the first anthracycline dose was 0.59 (IQR1 = 0.5; IQR3 = 0.96) years with a cumulative equivalent anthracycline dose of 158.2 ± 78.0 (min. 48.0–max. 451.8) mg/m2. Statistically significant differences between study and control groups were observed in terms of LVEF 3D (53.9 ± 4.4% vs. 59.1 ± 5.8%, p < 0.001), GLS 3D (−21.8 ± 4.2% vs. −25.2 ± 3.6%, p < 0.001), GCS 3D (−22.3 ± 3.8% vs. −27.3 ± 4.7%, p < 0.001) and GRS 3D (37.1 ± 5.0% vs. 44.6 ± 5.7%, p < 0.001). None of the previously described cardiotoxicity risk factors—age at the diagnosis, sex, age at treatment initiation, body surface area (BSA) at treatment initiation, time since treatment initiation, ALL subtype, total anthracycline dose and doxorubicin treatment—were statistically significant in the GLS or GCS models. Conclusions: GLS 3D, GCS 3D and GRS 3D of the left ventricle may be sensitive indicators of myocardial dysfunction in children with ALL treated with anthracyclines. The relation of the previously described cardiotoxicity risk factors to the 3D strain values and myocardial dysfunction is inconclusive; further studies are warranted.

We describe a case of a 3-year-old girl with neurofibromatosis type 1 presenting with arterial hypertension, in whom multimodal vascular imaging identified significant right renal artery stenosis. The patient was successfully treated with percutaneous transluminal renal angioplasty; however, post-procedural Doppler ultrasound revealed a transient vascular fistula. Changes in renal arterial inflow during the procedure may have temporarily altered pressure gradients, facilitating the opening of communication involving pre-existing compensatory collateral vessels. This case illustrates the diagnostic value of multimodal vascular imaging in pediatric hypertension and highlights a rare, self-limiting post-interventional vascular phenomenon.

In recent years, significant progress has been made in the management of pediatric arrhythmias through the use of cardiac implantable electronic devices (CIEDs) such as pacemakers (PMs) and implantable cardioverter-defibrillators (ICDs). Remote monitoring (RM) ideally enables rapid arrhythmia detection and shortens the time to clinical intervention. We herein present three pediatric cases from our institution to illustrate the clinical utility of RM in patients with complex cardiac conditions. In each case, RM facilitated timely recognition of arrhythmias, guided appropriate interventions, and contributed to improved patient outcomes. These experiences highlight the importance of daily RM as an integral component of care not only for children with CIEDs, offering both safety benefits and reduced hospitalization burden.

Abstract Noonan Syndrome can be associated with congenital heart defects and/or hypertrophic cardiomyopathy (HCM). Mutations in RAF1 gene occur in approximately 5% of patients, with HCM present in about 85% of these cases. We describe a previously unreported RAF1 gene variant associated with familial HCM and Noonan syndrome. An infant with abnormal prenatal screening (nuchal translucency of 7mm in the first trimester) was born at term, and was initially diagnosed with coarctation of the aorta, anomalous systemic venous drainage, and multiple muscular ventricular septal defects. He underwent surgery for coarctation of the aorta and an anomalous venous connection in the first month of life (a Waldhausen operation was performed along with correction of the venous anomaly). Subsequently, he required balloon angioplasty twice for re-coarctation of the aorta at 4th and 8th months of age. At the age of 12 months he was also diagnosed with hypertrophic cardiomyopathy with features of non-compaction confirmed later on cardiac magnetic resonance imaging (CMR). The recent CMR (08.2024) showed HCM with a maximal left ventricular wall thickness of 17 mm. Clinically the patient presented with Noonan syndrome features (prominent forehead, webbed neck, pectus excavatum) which got more pronounced with age. In his family history, the patient’s father had HCM, and underwent a heart transplant. His mother was treated for coarctation of the aorta. Genetic testing of the patient and his father revealed a novel, previously undescribed RAF1 c998G>T(p.Arg333Leu) variant, which was not present in his mother. Both clinical and phenotypical features of the father and child correspond with Noonan syndrome type 5, which is caused by RAF1 mutations. This variant has been reported by two laboratories as a variant of unknown significance; however, the clinical status of the affected patients was not provided. In patients with Noonan Syndrome harboring RAF1 variants, missense mutations are commonly observed, including the variant we report. The variant is located in the Intrinsically Disordered Region (IDR), a region in which mutations associated with Noonan Syndrome have been previously described as pathogenic. Furthermore, the variant we describe segregated with the disease. Therefore, our report sheds new light on this RAF1 variant in the context of its familial occurrence.

Background/Objectives: Acute lymphoblastic leukemia (ALL) is the most common childhood malignancy with promising survival. ALL treatment involves the use of cardiotoxic anthracyclines. The data for children on new methods of echocardiographic detection of early-onset anthracycline-related left ventricle dysfunction is lacking. Methods: Consecutive children with ALL were prospectively enrolled. The echocardiography was performed after ALL diagnosis and before initiation of anthracyclines (first assessment) and after induction and intensification therapy completion (second assessment). The left ventricle echocardiographic assessment involved conventional two-dimensional (2D) echocardiography, 2D speckle tracing, and three-dimensional (3D) echocardiography with offline analysis for 3D speckle tracing. Results: The preliminary group of 32 children presented with mean time between the first and second assessment of 7.3 ± 1.5 months (min.5.3–max.11.4). All children were treated by the same treatment protocol and received doxorubicin and daunorubicin. The mean cumulative equivalent anthracycline dose was 165.6 ± 54.0 mg/m2. Statistically significant differences between the first and the second echocardiography were observed in LV-GLS-2D −24.6 ± 3.3% vs. −21.0 ± 3.3%; p < 0.001, LVEF-3D 59.7 ± 7.3% vs. 55.1 ± 3.0%; p = 0.010, LV-GLS-3D −23.3 ± 5.3% vs. −20.4 ± 2.8%; p = 0.031 and LV-GCS-3D −26.3 ± 5.9% vs. −21.9 ± 3.2%; p = 0.017. The differences in LVEF-2D and LV-GRS-3D were not statistically significant. The decrease of >15% from the first assessment was observed in 9 (28%) in LV-GLS-2D, 8 (25%) in LV-GLS-3D, 11 (34%) in LV-GCS-3D and only in 4 (13%) patients in LV-GRS-3D. Conclusions: Two-dimensional and three-dimensional speckle tracing and 3D-LVEF may be sensitive indicators of subclinical left ventricular function impairment in children treated for ALL with anthracyclines. However, this is a preliminary analysis of the planned cohort; our results should be interpreted with caution.

Kawasaki disease is an acute systemic vasculitis of unknown aetiology. The diagnosis and treatment of the typical course of the disease have already been well described. However, in atypical, recurrent, or complicated Kawasaki disease cases, diagnostic and therapeutic decisions may be challenging. We present the case of a 3.5-year-old boy who presented with typical Kawasaki disease symptoms. Complete Kawasaki disease was diagnosed, and the boy was initially assigned as a low-risk patient, but he developed persisting fever and finally giant coronary aneurysms despite treatment according to the recommendations. Shortly after discharge, the boy was re-admitted due to limping. Two similar relapses were observed over the period of three months and the boy was ultimately diagnosed with recurrent Kawasaki disease complicated by arthritis. The initial typical presentation of Kawasaki disease may not predict its further course, recurrence, or potential complications.

Childhood cancer and its therapy, especially that involving potentially cardiotoxic cancer treatment, can structurally affect muscle strength and heart function, and therefore may result in lowered physical activity (PA). This study protocol aims to find the significant determinants of PA levels in pediatric oncological patients 1–5 years after heart-toxic chemotherapy and/or radiation of the heart region. The study group will include children 1–5 years after completing cancer therapy involving cardiotoxicity risk factors. The primary outcome of interest is the PA measured with an ActiGraph GT3X Accelerometer for 14 consecutive days and the assessment of WHO pediatric age-adjusted PA norms achievement. Assessed PA levels will be evaluated according to the possible determinants of PA: disease/treatment related risk factors, treatment complications, possible complications after the treatment, cardiac function with echocardiography including 2D and 3D strain imaging, physical function and muscle strength in ALPHA (Assessing Levels of Physical Activity) health-related fitness test battery and exercise capacity in cardiopulmonary exercise testing. The self-efficacy and motivation to PA, quality of life (QoL), lifestyle, socio-demographic, and anthropogenic factors as well as knowledge about the positive impact of PA will be evaluated with original and validated questionnaires. The PA determinants of the study group will be compared to the results of the control group of children in the same follow-up period (> 1 year < 5 years) after completing cancer therapy without cardiotoxic methods. The results may contribute to the development of future recommendations on prophylactic and therapeutic approaches, as well as proper lifestyle recommendations for children in long-term follow-up after cardiotoxic cancer therapy. The determinants will be used to develop targeted exercise prescriptions and exercise programs. NCT06256068.

The predominant malignancy in the pediatric population is acute lymphoblastic leukemia(ALL). Although survival rates are encouraging, the ALL therapeutic regimen includes cardiotoxic anthracyclines. The cardiotoxicity related to anthracycline use is detected in speckle tracing echocardiography among adult childhood cancer survivors and adult oncological patients. The data for children on detection of early-onset anthracycline-related cardiac dysfunction is lacking. To assess left ventricle function in 3D strain echocardiography among children treated for ALL and detection of early-onset anthracycline cardiotoxicity after induction and intensification therapy completion. In this prospective study, consecutive pediatric patients diagnosed with ALL were enrolled. Transthoracic echocardiography was conducted following diagnosis and prior to the initiation of anthracycline therapy (first assessment), and after the completion of induction and intensification therapy (second assessment). Three-dimensional echocardiographic recordings were analyzed offline using the 4D LV-Analysis software to evaluate left ventricular ejection fraction(3D-LVEF), global longitudinal strain(3D-LV-GLS), global circumferential strain(3D-LV-GCS), and radial strain(3D-LV-GRS). Results from the second assessment of the study group were compared with a control group consisting of age-matched healthy children. Interobserver reliability of the data was assessed by two echocardiographers. The study cohort comprised 26 children(12 females, 14 males) aged between 1-16 years(mean age 5,9±3,7years). Among these, 23(85%) children were diagnosed with ALL type C, and 25(96%) were treated according to the AIEOP BMF 2017 protocol. The mean interval between the first and second echocardiographic assessments was 212 days(7 months). The cumulative dose of doxorubicin administered was 181,7± 62mg/m², with 8 children receiving a dose >200 mg/m² and one child >300 mg/m². Statistically significant differences between the first and the second echocardiography were observed 3D-LVEF(59,7±7,3% vs. 53,2±4,2%,p=0,004), 3D-LV-GLS(-23,3±5,3% vs -20,2±3,2%,p=0,031) and 3D-LV-GCS(-26,4±6,0% vs. -21,8±3,2%,p=0,012), the difference in 3D-LV-GRS was not statistically significant. The decrease of >15% from first assessment was observed in 9(35%) in 3D-LVGLS, 12(46%) in 3D-LVGCS and only in 3(12%) patients in 3D-LVGRS. In comparison with the control group the differences were observed in 3D-LVEF(53,2±4,2% vs. 60,7±5,8%,p<0,001), 3D-LV-GLS(-20,2±3,6% vs. -24,9±3,9%,p<0,001) and 3D-LV-GCS (21,8±3,2% vs. -28,2±4,5%, p<0,001) as well as in 3D-LV-GRS(-35,4±4,6% vs. -45,1±5,9%,p<0,001). 3D echocardiography with speckle tracing may be sensitive method in detection of early anthracycline-related cardiac dysfunction in children treated for ALL. Early detection of subclinical cardiotoxicity may enable implementation of cardioprotective measures and treatment.Table 1- results Figure 1- protocol

BACKGROUND:Low voltage bridge mapping (LVBM) is an approach to atrio-ventricular nodal reentry tachycardia (AVNRT) ablation that has been postulated to facilitate the procedure. In our center, AVNRT ablations were performed with the traditional radio frequency current (RF) current approach using the CARTO mapping system. The use of LVBM in children has been well described with cryoablation but not with RF. LVBM was introduced in our center in April 2023 and is now routinely used for all AVNRT RF ablations. The aim of this study was to analyze how the introduction of the LVBM influenced the results of the pediatric AVNRT ablations using the RF current. METHODS:We analyzed all AVNRT ablations performed between January 1, 2019 and June 30, 2024, and compared traditional and LVBM approaches. RESULTS:The study group (LVBM) consisted of 40 and the control group of 61 children. Both groups did not differ significantly regarding age, body weight, and sex distribution. In the LVBM group, the total time of RF applications was significantly shorter (median of 120 s vs 191 s) and the number of RF applications was smaller than in the control group (median of: 4.5 vs 8). Total procedural time did not differ significantly between the groups. The success rate at 3 months follow-up was higher in the LVBM (92% vs 85%) group but failed to differ statistically. No complications were reported in either of groups. CONCLUSIONS:The introduction of the LVBM in RF AVNRT ablations in children allows for limiting the number and time of RF applications required, without prolonging the total procedure time. Our experience shows LVBM can also be effectively used in RF ablations of AVNRT.