Background: Acute myocarditis is defined as an inflammatory process consisting of multiple complex physiopathological processes. Due to its variability, the management of this condition has been a topic of debate. Our study aimed to evaluate the efficacy and safety of intravenous immunoglobulin (IVIg). Methods: We retrospectively collected data from patients admitted to a paediatric cardiology department from 2015 to 2020. Following the inclusion and exclusion criteria, a total of 68 patients diagnosed with acute myocarditis were selected and divided into two groups: treated with IVIg and untreated. We determined clinical and paraclinical parameters, such as symptom remission, normalisation of the ejection fraction at discharge, and cardiac marker evolution. Mixed-design analysis of variance and McNemar tests were performed to determine the statistical differences between groups. Results: In the treated group, 88.2% of the patients developed symptom remission at discharge vs. 50% in the untreated group, and 61.8% of the treated patients presented normalisation of the ejection fraction (EF) vs. 8.8% in the untreated group (p < 0.05). The evolution of cardiac markers did not statistically differ between the treated and untreated groups. Regarding safety, three treated patients presented mild, temporary side effects. Conclusions: Having found a statistically significant improvement in symptomatology and left ventricular EF, our study suggests the efficacy of IVIg in the treatment of acute myocarditis. Treatment with immunoglobulins was relatively safe, with only mild adverse reactions (fever and mild chest pain).

Background: The presence of a primary cardiac tumor in a pediatric patient is a rare echocardiographic finding. Case Report: We report the case of an 11-year-old female patient with multiple peripheral embolisms, due to a gigantic left ventricular tumor, with a unique echocardiographic appearance. The patient was referred to the emergency department due to acute pain and loss of sensitivity in both of her legs. Past medical history was significant for acute lymphoblastic leukemia. Upon physical examination, suspicion of bilateral lower leg ischemia was raised. Doppler arterial ultrasound of both legs confirmed the suspicion mentioned above, as the right lower extremity suffered from partial arterial occlusion of the external iliac artery and total occlusion of the femoral arteries. Meanwhile, in the left lower extremity, the occlusion was localized in the proximal tibio-peroneal artery. Cardiac sonography revealed a massive, mobile, left ventricular intracavitary mass. Aside from its large dimensions (6.3 cm by 3 cm), its aspect was striking as well as it had very mobile and friable edges. Emergency bilateral endarterectomy and excision of the left ventricular tumor were performed alongside systemic anticoagulant therapy, with excellent results, as no tumoral residual masses could be seen in the left ventricle, and the arterial blood flow was restored completely in both lower extremities. The histopathological aspect of the excised masses was that of a myxoma. The patient recovered well after surgery and was discharged on postoperative day 14. Conclusion: Despite only a handful of cases of cardiac myxomas being reported due to their rarity in the pediatric population, clinical presentation with peripheric embolism triggered a high index of suspicion of embolic mechanism in our patient and prompted a rapid assessment and successful management.

Introduction: Dilated cardiomyopathy (DCM) is a relatively rare manifestation of pediatric heart failure. Despite recent diagnostic advancements and expanded screening modalities, the underlying cause of DCM remains elusive in over 50% of pediatric cases. We report the case of a 1-year-old girl presenting with newly diagnosed, rapidly progressive dilated cardiomyopathy, caused by a novel, rare homozygous pathogenic variant in the PPP1R13L gene. Case presentation: The patient, with no significant family history of cardiac disease, previously asymptomatic and in good health, presented with a 2-day history of nausea, vomiting, difficulty breathing, and low urine output, following a recent respiratory infection. The echocardiography identified a dilated left ventricle (Z score > +2 for age) and severe systolic left ventricular dysfunction, and a positive diagnosis of dilated cardiomyopathy was confirmed by the cardiac magnetic resonance (CMR). Genetic testing identified a homozygous pathogenic variant in the PPP1R13L gene, classified as a novel pathogenic variant that encodes the inhibitor of apoptosis-stimulating protein of p53 (iASPP). Despite targeted treatment, the severe systolic dysfunction persisted, and the general state progressively deteriorated and warranted the need for a cardiac transplant, which she underwent one year following the initial diagnosis. Conclusions: Patients diagnosed with underlying genetic DCM, particularly those harboring PPP1R13L mutations, carry an exceedingly poor prognosis in the absence of orthotopic heart transplantation. Existent case reports confirm that disruptions in this gene predictably yield highly aggressive, life-threatening cardiomyopathies.

Heart failure (HF) is a complex clinical syndrome that represents an increasingly relevant health issue, associated with substantial morbidity and mortality in pediatric patients. While considerable therapeutic advances have transformed the prognosis of adult patients with heart failure over the past three decades, progress in pediatric heart failure has been considerably slower. In adult patients, large randomized controlled trials (RCTs) have established angiotensin-converting enzyme inhibitors (ACE inhibitors), beta-blockers, mineralocorticoid receptor antagonists (MRAs), angiotensin receptor-neprilysin inhibitors (ARNIs), and, more recently, sodium-glucose cotransporter-2 (SGLT2) inhibitors as the cornerstone of guideline-directed medical therapy. Evidence supporting pharmacological therapy in pediatric heart failure remains scarce and frequently inconclusive, with most available studies being limited by small sample sizes and heterogeneous patient populations. Therefore, heart failure treatment in children is mostly extrapolated from adult studies and guidelines. However, developmental differences in myocardial structure and function, neurohormonal signaling, pharmacokinetics and pharmacodynamics may substantially influence therapeutic response. In addition, the rarity and the etiological heterogeneity of pediatric HF create major methodological challenges for adequately powered clinical trials. This review compares the pharmacological management of HF in adult and pediatric populations, examines the strength of evidence for contemporary therapies and discusses the biological and methodological limitations of translating adult evidence into pediatric practice. Multicenter collaborative studies and well-designed pediatric randomized controlled trials are essential for establishing truly evidence-based pharmacological strategies for children with HF.

Introduction: Determining the prognosis and probable evolution of dilated cardiomyopathy (DCM) in pediatric patients has been an area of interest in the literature over the years. The aim of this study was to investigate baseline clinical, laboratory, and echocardiographic parameters associated with early ventricular recovery during hospitalization in children with dilated cardiomyopathy. Materials and methods: A retrospective, observational, single-center study was conducted including 56 pediatric patients diagnosed with DCM between 2015 and 2025. Baseline clinical and paraclinical variables were collected at admission. Changes in the left ventricular ejection fraction were assessed during hospitalization, and patients were categorized into recovery and non-recovery groups. Statistical analysis was performed to identify independent predictors of early ventricular recovery. Results: Early ventricular recovery occurred in 25 (44.64%) patients. In multivariate logistic analysis, Ross functional class was the only independent marker associated with early ventricular recovery (OR 2.547, 95% CI 1.179–5.501; p = 0.017). Bootstrap validation confirmed the robustness of the association. Troponin, NT-proBNP, mitral regurgitation and treatment strategy were not independently associated with recovery. ROC analysis demonstrated a modest discriminatory performance for the Ross functional class. Discussion: In this cohort of children with DCM, symptom severity was the only baseline variable consistently associated with early ventricular recovery. Clinical assessment was more useful than laboratory biomarkers and echocardiographic parameters in predicting short-term improvement in ventricular function. Conclusions: The Ross functional class was the only baseline variable independently associated with early ventricular recovery. These findings support the use of the Ross functional classification as a simple bedside tool, useful in early prognostic stratification in children with dilated cardiomyopathy, although its discriminatory performance was modest.

Short QT syndrome (SQTS) is a rare, inherited cardiac channelopathy characterized by an abnormally shortened QT interval, accelerated ventricular repolarization, and an increased risk of atrial and ventricular tachyarrhythmias, including sudden cardiac death (SCD). We report the case of a 14-year-old girl diagnosed with SQTS presenting with persistent atrial fibrillation and a complex independent neurological background. The patient, with no significant family history of cardiac disease or SCD, was incidentally found to have atrial fibrillation and a markedly shortened QT interval during a routine medical evaluation. Although she remained entirely asymptomatic from a cardiovascular perspective, her medical history was notable for maternal Toxoplasma gondii infection during pregnancy, extreme prematurity, and delayed psychomotor development. Electrocardiographic (ECG) findings consistently demonstrated a short QT interval, and genetic testing revealed a likely pathogenic variant in the KCNJ2 gene, consistent with type 3 short QT syndrome (SQTS3). Despite the initiation of antiarrhythmic therapy, atrial fibrillation persisted and the QT interval remained significantly shortened throughout the 24-month follow-up. This case highlights the diagnostic and therapeutic challenges of managing short QT syndrome in pediatric patients, particularly in those who are asymptomatic yet exhibit sustained atrial arrhythmias. It also highlights the coexistence of cardiac channelopathy and neurological comorbidities, emphasizing the importance of a multidisciplinary approach for these distinct clinical entities.