Pediatric idiopathic pulmonary arterial hypertension (IPAH) refractory to maximal medical therapy is associated with high morbidity and mortality, and therapeutic options remain limited. We describe a 12-year-old girl with end-stage IPAH who developed acute decompensated right heart failure despite triple combination therapy, including high-dose prostacyclin. Escalation to intravenous epoprostenol failed to result in clinical or hemodynamic improvement. Following multidisciplinary discussion, ethical approval, and informed consent, sotatercept was initiated under compassionate use. Sotatercept therapy resulted in rapid and marked clinical recovery, including improvement in World Health Organization functional class (WHO FC) from IV to II, substantial increase in 6-min walk distance, normalization of NT-proBNP levels, and significant echocardiographic and invasive hemodynamic improvement. This case highlights the potential role of sotatercept as a rescue therapy in pediatric IPAH refractory to conventional treatment.

Coronary artery anomalies (CAAs) are rare and heterogeneous congenital abnormalities. However, some of them are associated with serious consequences, including myocardial ischemia, heart failure, and even death. This study aimed to characterize symptoms, course, treatment, and prognosis of different types of CAAs. This single-centre retrospective cohort study included 38 consecutive paediatric patients hospitalised in the Department of Paediatric Cardiology due to a diagnosis of CAAs between 01.2012–03.2025. Patients were divided into two groups: anomalous origin of the coronary arteries (Origin anomalies) and coronary artery fistulae (CAF), respectively, 20 and 18 patients. The collected data included clinical symptoms, laboratory tests, electrocardiography, echocardiographic parameters, course of treatment and long-term follow-up. The mean age of the analysed group was 3.32 ± 5.35 years (range: 1 day − 16 years; IQR0-4.25). The study group included 42

BackgroundLoeys–Dietz syndrome (LDS) is a clinically and genetically heterogeneous, autosomal dominant aortic aneurysm syndrome with widespread systemic involvement. We present the case of a 16.5-year-old girl with LDS type 2 (LDS2) caused by a heterozygous pathogenic variant, c.1582C>T (p.Arg528Cys), in the transforming growth factor-beta receptor type 2 (TGFBR2) gene who was treated with recombinant growth hormone (rGH) due to coexisting GH deficiency (GHD). This case report (observational study) presents the efficacy of rGH therapy and the safety aspects of this treatment, including aortal imaging follow-up (echocardiography, ECHO). To our knowledge, this is the first investigation of the effects of long-term rGH treatment on aortic dimensions in an LDS patient.Case summaryLDS was recognized in the patient in the 2nd year of life. After the 3rd year of life, growth deceleration was observed. At age 6, GHD was recognized [the maximum GH after stimulation 7.2 ng/ml; insulin-like growth factor-1 (IGF-1), 35 ng/ml; N: 84–447]. At age 6.5 years, rGH was initiated (height standard deviation score, htSDS −2.4), which continued for up to 14.25 years (htSDS-1.4). Her height at 16.5 years was 155 cm. The dose of rGH was 0.025–0.028 mg/kg/day. After the age of 16 months, widening of the aortic root was observed via echocardiography. At nearly 16 years, due to dilated aortic root (Z score +5.95), the girl underwent a plastic operation on the aorta, which had a satisfactory outcome. The patient's current status is stable, but the management of patients with LDS requires multidisciplinary cooperation due to the many coexisting comorbidities.ConclusionsAlthough aortic dilatation occurs in most LDS patients, the possible influence of GH therapy on aortic size must be considered. However, whether IGF-1, the main biochemical marker of GH activity, can be independently associated with increased aortic diameter has not been determined. In addition to its growth-promoting effect, the wide influence of GH on the human body, metabolic status, and muscle strength is also significant. The extremely low IGF-1 level before rGH therapy in the present patient and the strict monitoring of the IGF1/IGFBP3 ratio during rGH administration seem to be safe and beneficial for therapy.

(1) Background: The clinical course of acute myocarditis in adolescents is heterogeneous, and reliable predictors of early functional changes remain limited, particularly in patients without severe systolic dysfunction. Routine hematologic parameters may reflect the early inflammatory response, but their prognostic relevance in pediatric non-fulminant myocarditis is poorly defined. This exploratory study aimed to assess whether admission inflammatory blood indices are associated with short-term changes in left ventricular systolic function in adolescents with acute myocarditis. (2) Methods: We retrospectively analyzed 44 adolescents (median age 16 years, 84% male) hospitalized with suspected acute non-fulminant myocarditis between 2020 and 2023. All patients had preserved or mildly reduced left ventricular ejection fraction (LVEF) at presentation. Clinical, laboratory, electrocardiographic, and echocardiographic data obtained at admission were analyzed. Changes in LVEF between the acute and post-acute phases during hospitalization were assessed using transthoracic echocardiography. Cardiac magnetic resonance imaging was performed in a subset of patients to support diagnosis but was not uniformly available for quantitative analysis. (3) Results: No in-hospital deaths occurred. A modest positive correlation was observed between neutrophil count at admission and improvement in LVEF during hospitalization (r = 0.348, p = 0.028). No significant associations were found between LVEF change and white blood cell count, lymphocyte count, monocyte count, neutrophil-to-lymphocyte ratio (NLR), troponin I, or NT-proBNP. (4) Conclusions: In adolescents with non-fulminant acute myocarditis and preserved or mildly reduced systolic function, admission neutrophil count was associated with short-term improvement in left ventricular ejection fraction. Given the retrospective design, limited sample size, and absence of mechanistic data, these findings should be interpreted as hypothesis-generating. Further prospective studies incorporating standardized cardiac magnetic resonance imaging and immunologic profiling are needed to clarify the clinical significance of this association.

AIMS:Paediatric acute heart failure (AHF) differs from adult cases, and less numerous population limits research aimed at improving clinical practice and prognosis. We aimed to evaluate outcomes and prognostic factors in the paediatric population of AHF patients with left ventricular ejection fraction (EF) < 50%, or systemic right ventricular fractional area change <40%. METHODS AND RESULTS:This single-centre retrospective cohort study included 162 consecutive paediatric patients hospitalized for new-onset AHF with systolic dysfunction or the first exacerbation with reported systolic dysfunction of chronic heart failure (CHF). Patients were categorized into two subgroups: those with >5% improvement in EF on follow-up echocardiography before discharge (n = 115) and those with EF deterioration, ≤5% EF improvement, or death during hospitalization (n = 47). The study contained 58% males and 42% females. The median age was 1 [interquartile range (IQR) 0-8] year, 14 (IQR 2-101) months. The mortality rate was 12.3%. Acute heart failure aetiologies included congenital heart defects (51.2%), cardiomyopathy (20.4%), infections (14.2%), arrhythmias (11.7%), and others (2.5%). Our study demonstrated independent factors of worse outcomes: cardiomyopathies aetiology [odds ratio (OR) 2.762 (1.210-6.307)], older age [OR 1.006 (1.000-1.011)], and factors of better prognosis: infective aetiology [OR 0.083 (0.010-0.664)], higher EF [OR 0.953 (0.905-0.999)], oxygen saturation (SpO2) [OR 0.951 (0.905-0.992)]. In contrast to CHF exacerbation, de novo AHF had better outcomes (P = 0.014). CONCLUSION:Acute heart failure with systolic dysfunction in children has an unfavourable prognosis, however most patients improved during hospitalization. In our study, cardiomyopathy aetiology predicted worse outcomes. In turn, infective aetiology, younger age, higher EF, and SpO2 were independent factors of better prognosis.

Purpose: Mitral valve surgery in children involves correcting congenital and acquired pathologies, with a reported mortality rate of 0.9%. Low cardiac output syndrome (LCOS) is a serious complication with the incidence of 20-25%. The aim of the study was to estimate possible prognostic factors of LCOS in children undergoing mitral valve procedure. Material and method: This single-center retrospective analysis enrolled children aged <18 years who underwent mitral valve surgery during 24 year period. Preoperative clinical and laboratory parameters, and operative factors were analyzed. Results: Thirty consecutive pediatric patients (11 (37%) males and 19 (63%) females) in median (Q(1) - Q(3)) age of 57 (25-115) months, who underwent mitral valve replacement, were included. The 30-day mortality was 7% (2 patients) and was related to postoperative multiorgan failure. LCOS occurred in 8 (27%) children. The receiver operator curve (ROC) analysis established parameters that have predictive value for LCOS occurrence: cardiopulmonary bypass (CPB) time, with 89 min as optimal cut-off point (AUC = 0.744, p = 0.011) yielding sensitivity of 100% and specificity of 42.9%; left ventricular ejection fraction (LVEF) < 60 % (AUC = 0.824, okp = 0.001) with sensitivity of 62.5% and specificity of 93.75%; and red blood cell distribution width (RDW) above 14.5 % (AUC = 0.840, p < 0.001; sensitivity of 87.5% and specificity of 75%). Conclusions: In mitral valve replacement in pediatric patients, CPBtime above 89 min, preoperative LVEF below 60% and preoperative RDW above 14.5% can be regarded as the potential predictors of LCOS.

BACKGROUND:Remote monitoring (RM) of cardiac implantable electronic devices for adults offers improved treatment efficacy and, consequently, better patient clinical outcomes. There is scant data on the value and prognosis of RM in the pediatric population.AIMS:The goal of this study was to determine the efficacy of RM by analyzing the connectivity of bedside transmitters, adherence to planned automatic follow-ups, and occurrence of alert-based events.METHODS:We evaluated the pediatric population with implanted pacemakers for congenital AV block or after surgically corrected congenital heart diseases.RESULTS:A total of 69 patients were included in our study. The median (Q1-Q3) patient age was 6.0 (2.0-11.0) years. All patients received bedside transmitters and were enrolled in the RM system. Among them, 95.7% of patients had their first scheduled follow-up successfully sent. Patients were followed up remotely over a median time of 33.0 (13-45) months. Only 42% of patients were continuously monitored, and all scheduled transmissions were delivered on time. Further analysis revealed that 34.8% of patients missed transmissions between June and September (holiday season). Alert-based events were observed in 40.6% patients, mainly related to epicardial lead malfunction and arrhythmic events. Overall compliance was also compromised by socioeconomic factors.CONCLUSIONS:Our findings are in concordance with recently published results by PACES regarding a high level of compliance in patient enrollment to RM and time to initial transmission. However, a lower level of adherence was observed during the holiday season due to interrupted connectivity of bedside transmitters. Importantly, a relatively low occurrence of alert transmissions was observed, mainly related to epicardial lead malfunction and arrhythmic events.

Background: To our knowledge, no studies have assessed Quality of life (QoL) in asymptomatic children with a preexcitation electrocardiogram pattern. Aim: To evaluate the QoL of children with asymptomatic Wolff-Parkinson-White (WPW) syndrome. Methods: This study involved QoL assessment of 31 children with asymptomatic preexcitation and 82 healthy children using the WHOQOL-BREF and the Pediatric Arrhythmia Related Score (PARS), a specific questionnaire that we have developed, which is related to patients' feelings and observations concerning arrhythmia. Results: There were no significant differences between the two groups in all the measured domains; however, there were significant differences regarding general satisfaction with their health condition (P = 0.01). There were no differences in general satisfaction with the QoL, but WPW children more often experienced palpitations than the control group (P <0.001) and were more likely to feel sad (P = 0.046) and nervous (P = 0.04) compared to healthy children. Conclusions: The children with WPW were more dissatisfied with their health compared to healthy children. Although both groups of children had similar levels of satisfaction with their QoL, some areas of physical and psychological parameters of QoL were worse in WPW children. The PARS questionnaire is a useful tool as a disease-specific QoL instrument, which supplements the general questionnaire and aids in clinical practice and decision-making.

Nursing can be a rewarding profession as it involves helping others. However, it is demanding, particularly for Cardiovascular nurses who may be exposed to stressful situations, patient suffering, and death. Additionally, work-related issues such as shift work, work overload, and excessive bureaucracy can pose challenges. It is necessary to consider that caring for children presents unique challenges compared to caring for adults, which can impact the Professional Quality of Life (ProQoL) and its domains. In this study, we aimed to investigate differences in ProQoL between nurses working in pediatric cardiology and adult cardiology units. Nurses working in pediatric cardiology and adult cardiology units were enrolled in the study. The Polish version of the ProQoL 5 questionnaire was collected using a paper form. The specific domains of satisfaction, job burnout, and stress were calculated according to the questionnaire key. Moreover, data on age, additional places of employment and working hours were collected. The study examined the relationship between these factors and ProQoL domain scores using the R-Spearman correlation. Scores from each domain were compared between clinics using U-Mann-Whitney tests and a two-sided Fisher's exact test. The study involved 13 pediatric cardiology staff: six midwives, six nurses, one paramedic, and 29 adult cardiology staff: 26 nurses, two paramedics and one medical caretaker. No differences were found in any of the ProQoL domains between those working with children and those working with adults: p=0.74 for satisfaction, p=0.90 for burnout and p=0.77 for stress. A trend of lower age was observed among those working in the Paediatric Cardiology Department. The majority of nurses, regardless of ward, reported high or intermediate job satisfaction, as well as low or moderate levels of stress and burnout. Only one person employed in the adult Cardiology department reported a low level of job satisfaction. Older age was associated with higher job satisfaction (R=0.32; p=0.04) and lower levels of burnout (R=-0.31; p=0.048). Additionally, a higher number of working hours per month was associated with burnout (R=0.41; p=0.007). Satisfaction was negatively associated with burnout (R=-0.51; p<0.001) and positively correlated with stress (R=0.35; p=0.04). However, burnout was not associated with stress (p=0.56). Regardless of the different work specifications in departments, related to working with children or adults, the ProQoL does not differ significantly. Older age may be positively associated with ProQoL. It may be the result of greater work experience. A higher number of working hours per month is associated with burnout, so it is therefore appropriate to emphasize the importance of occupational health and encourage not to overwork. A multicentre study could further define the differences in ProQoL and the influencing factors.

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Danon disease is the only X-linked dominant storage disease and is one of the few glycogenoses concerning cardiac muscle.It is caused by a mutation that impairs

Familial thoracic aortic aneurysms and dissections may occur as an isolated hereditary trait or as part of connective tissue disorders with Mendelian inheritance, but severe cardiovascular disease in pediatric patients is extremely rare. There is growing knowledge on pathogenic variants causing the disease; however, much of the phenotypic variability and gene–gene interactions remain to be discovered. We present a case report of a 5.5-year-old girl with an aortic aneurysm and concomitant polycystic kidney disease. Whole exome sequencing was performed, followed by family screening by amplicon deep sequencing and diagnostic imaging studies. In the proband, two pathogenic variants were identified: p.Tyr257Ter in the LOX gene inherited from her mother, and p.Thr2977Ile in the PKD1 gene inherited from her father. All adult carriers of either of these variants showed symptoms of aortic disease. We conclude that the coexistence of two independent genetic variants in the proband may be the reason for an early onset of disease.

Simple whole blood analysis can effectively demonstrate complex changes in inflammatory responses to cardiovascular disorders in adults and enable the prediction of adverse outcomes or diminished survival. Such inflammatory activation has also been detected in the pediatric population. Blood analysis results are repeatable and readily available, which gives the method an advantage over others. Inflammatory phenomena such as a high leukocyte count and an increased neutrophil-to-lymphocyte ratio (NLR) are related to a poor prognosis of advanced heart defects and worse outcomes after pediatric cardiac surgery in the advanced stages of the disease. Surgery-associated inflammation exacerbates these diseases, and the inflammatory response may further complicate the postoperative period. Simple blood cell counts and indices may be beneficial for evaluating cardiac surgery outcomes and cardiovascular disorder prognosis in infants and children. This review summarizes current knowledge on inflammatory markers in pediatric cardiovascular diseases and surgery.

Heart rate asymmetry (HRA) is a physiological phenomenon characterized by an unequal contribution of heart rate decelerations and accelerations to different heart rate variability (HRV) features. While HRA has been demonstrated in adults’ ECGs of different duration, a similar investigation in healthy children has not been conducted. This study investigated the variance- and number-based HRA features in 96 healthy children (50 girls and 46 boys, aged 3–18 years) using 24-h ECGs. Additionally, we studied sex differences in HRA. To quantify HRA, variance-based and relative contributions of heart rate decelerations to short-term (C1d), long-term (C2d), and total (CTd) HRV, and the number of all heartbeats (Nd) were computed. Heart rate decelerations contributed more to C1d, but less to C2d and CTd, and were less frequent than heart rate accelerations. Short-term HRA was better expressed in boys. The majority of children (93.7%) had short-term HRA, 88.5% had long-term HRA, 88.5% had total HRA, and 99.0% had more accelerations than decelerations. No sex differences were observed for the rate of various HRA features. Heart rate asymmetry is a common phenomenon in healthy children, as observed in 24-h ECGs. Our findings can be used as reference data for future clinical studies on HRA in children.

Pulmonary arterial hypertension (PAH) is a rare heterogeneous disorder in the paediatric population which is mostly associated with congenital heart disease. The management of paediatric idiopathic PAH (IPAH) is difficult due to insufficient comparative data and depends on the results of evidence-based adult studies with several pulmonary vasodilators, as well as the clinical experiences of paediatric experts. Our aim was to present the case of a 9-year-old girl who underwent several methods of treatment, including pharmacotherapy with a significant reaction to treprostinil, as well as bilateral lung transplantation. The patient's treatment was distinguished by the fact that the dose escalation was as rapid as that observed in the adult population. Due to the limited current evidence and knowledge, the initiation of treatment for these patients remains an individual choice. On the grounds of the number of non-specific symptoms, the diagnosis of this patient was a long process and based mainly on the differential diagnosis. The purpose of this paper is to study this example in order to highlight the importance of early symptoms and the quick implementation of intensive treatment. The applied methods may be useful in doubtful diagnosis processes and treatment procedures in the paediatric population.

COVID-19 pandemic is the biggest epidemiologic problem of the 21st century. A severe course of SARS-CoV-2 infection in children is rare. Sometimes, especially in patients with chronic disease, COVID-19 may be insidious and life-threatening. This article presents the course of COVID-19 in a 17-year-old boy with Friedreich's ataxia-induced hypertrophic cardiomyopathy. Although, the main symptoms of COVID-19 (i.e., fever, cough) were moderate at the beginning of the illness, the patient's condition deteriorated rapidly due to cardiac problems, atrial fibrillation, and heart failure. The patient required antiarrhythmic treatment and pharmacological and electrical cardioversion. Moreover, because of pneumonia requiring supplemental oxygen, remdesivir and convalescent plasma therapy was given to the patient., The administration of the antiviral treatment was crucial to the patient's recovery.

CLINICAL IMAGE Post COVID -19 vaccination myocarditis in teenagers 1 and SARS -CoV -2.However, antibody testing revealed high titers of immunoglobulin (Ig) G and IgA against the SARS -CoV -2 spike protein in all 3 patients.Based on the clinical presentation, a diagnosis of myocarditis associated with COVID -19 vaccination was made.Bed rest and thromboprophylaxis with enoxaparin were ordered.A complete resolution of the symptoms and troponin level normalization were observed within a few days, and all the patients were discharged home.Recent data show that the risk of myocarditis in patients vaccinated against COVID -19 is 3 times higher than in the nonvaccinated ones. 1,2Male adolescents are affected more than female teenagers, and myocarditis is more likely to develop following the second dose. 2 However, in our report, 2 patients developed the symptoms after the first dose of the vaccine.In a majority of papers published so far, the diagnosis of myocarditis was based on CMR and the modified Lake Louise Criteria. 3,4In our case, CMR showed no signs of edema in any of the patients, which might indicate a milder course of the disease.However, a longer observation period is required to confirm this.The specific mechanism of COVID -19 vaccine-induced myocarditis and individual predispositions to cardiovascular events remain unknown.All our patients had a high titer of anti-SARS -CoV -2 antibodies (measured 12-23 days after the vaccination).The published data usually indicate only a modest increase in IgA antibodies after the vaccination. 5Some authors reported higher anti spike protein IgA antibodies in males than in females.A possible correlation between IgA antibody concentration and susceptibility to postvaccination myocarditis requires further investigation.