Search Results (1,130)

Ventricular repolarization abnormalities are among the most frequent electrocardiographic findings in pediatric athletes undergoing cardiovascular screening, yet their clinical significance remains a major source of diagnostic uncertainty. While most of them represent benign expressions of training-induced cardiac remodeling and developmental maturation, selected patterns may constitute the earliest phenotypic manifestation of cardiomyopathies or primary electrical disease. Distinguishing physiological adaptation from early pathology is therefore essential to prevent both sudden cardiac events and unnecessary restrictions on sports participation. This review integrates contemporary international electrocardiographic interpretation criteria with emerging pediatric evidence to provide a clinically oriented framework for evaluation and risk stratification of ventricular repolarization abnormalities in pediatric athletes. Early repolarization and anterior T-wave inversion are commonly benign when occurring within recognized age- and ethnicity-specific patterns and in the absence of symptoms, concerning family history, or structural abnormalities. Conversely, lateral or inferolateral T-wave inversion, atypical ST-segment morphology, complex ventricular arrhythmias, and abnormal imaging findings represent red flags requiring comprehensive investigation, including multimodality imaging when indicated. Due to the dynamic electrophysiological evolution during adolescence, longitudinal reassessment is crucial. A structured, risk-based approach integrating electrocardiographic features, demographic/familial context, clinical evaluation, imaging findings, and follow-up provides a pragmatic strategy to optimize risk detection while safeguarding appropriate athletic participation in young athletes. Full article

Introduction: Propofol is one of the most commonly used intravenous anesthetics worldwide and is considered safe for all age groups. However, there have been reports that propofol can induce severe atrioventricular block in humans, and several studies have shown that propofol hinders or prevents the inducibility of arrhythmias during electrophysiological studies (EPS) and radiofrequency (RF) ablation. Objectives: To compare arrhythmia inducibility during electrophysiological study and radiofrequency ablation in pediatric patients with Wolff–Parkinson–White syndrome undergoing propofol-based sedation versus sevoflurane-based general anesthesia. Methods: We conducted a retrospective observational cohort study including 45 pediatric patients aged 0–18 years. Patients were identified through a review and analysis of a database of individuals with Wolff–Parkinson–White syndrome who were referred for electrophysiological study and/or radiofrequency ablation at the Electrophysiology Laboratory of the Institute of Cardiology (IC/FUC) in Porto Alegre over the past five years (2019–2024). Patients with prior ablation, structural heart disease, or ongoing antiarrhythmic therapy were excluded. The patients were divided into two groups and designated as group S (who received sedation) or group G (who received general anesthesia). Sedation (group S) was performed with midazolam (0.08–0.2 mg/kg), fentanyl (0.1–0.2 μcg/kg), and propofol 50–60 µg/kg/min in continuous infusion. General anesthesia (group G), in turn, was performed with sevoflurane at an average dose of 2% (1 MAC according to age). Results: From 4874 invasive electrophysiology procedures performed during the study period, 45 involved pediatric patients with WPW. The sedation group (n = 29) had significantly older patients (14.6 ± 2.5 vs. 10.3 ± 2.8 years, p < 0.001) with higher weight (65.9 ± 16.3 vs. 41.2 ± 7.8 kg, p < 0.001) compared to the general anesthesia group (n = 16). Arrhythmia was successfully induced in 15/29 (51.7%) patients in the sedation group compared to 13/16 (81.2%) in the general anesthesia group (p = 0.062, Fisher’s exact test). Although this difference did not reach statistical significance, it represents a clinically relevant 29.5% lower induction rate in the sedation group. Post hoc power analysis revealed the study was underpowered (49.8%), suggesting a possible Type II error. Analysis of the “procedure room time” revealed a longer duration in the general anesthesia group (97.8 ± 36.7 vs. 67.8 ± 24.4 min), and this difference was statistically significant (p = 0.002). Conclusions: This study compared propofol-based sedation with sevoflurane-based general anesthesia in pediatric WPW patients. While sedation with propofol did not show a statistically significant reduction in arrhythmia inducibility, there was a concerning trend toward lower induction rates (29.5% difference) that may be clinically relevant. The study’s limited statistical power (49.8%) suggests these findings should be interpreted cautiously, and larger prospective studies are needed to definitively establish whether propofol affects arrhythmia inducibility in this population. Propofol remains a viable option for these procedures, but clinicians should be aware of the potential for reduced inducibility, particularly in cases where arrhythmia induction is critical for diagnosis and treatment. Full article

Background: Chronic hypoxemia in cyanotic congenital heart disease triggers well-recognized hematological adaptation; however, whether hypoxemia also drives systemic inflammatory activation remains uncertain. This study aimed to evaluate hematological parameters and inflammatory indices in cyanotic and acyanotic congenital heart disease (CHD) to better characterize the relationship between hypoxemia and systemic inflammatory status. Methods: In this single-center retrospective study, 260 children with congenital heart disease were classified as cyanotic (n = 158) or acyanotic (n = 102). Preoperative clinical data and laboratory parameters were analyzed, including oxygen saturation, hemoglobin, hematocrit, leukocyte indices, C-reactive protein (CRP), and procalcitonin (PCT). Inflammatory indices derived from complete blood counts were calculated, including the neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), and systemic immune–inflammation index (SII). Results: Oxygen saturation was significantly lower in cyanotic patients than in acyanotic patients (75 ± 9% vs. 95 ± 4%, p < 0.001). Consistent with hypoxemia-driven hematological adaptation, hemoglobin and hematocrit levels were significantly higher in the cyanotic group (16.1 ± 2.9 g/dL vs. 13.1 ± 2.0 g/dL and 50.8 ± 9.7% vs. 39.7 ± 5.5%, respectively; p < 0.001). In contrast, inflammatory indices (NLR, PLR, and SII) were similar between cyanotic and acyanotic patients, and no significant associations were observed between oxygen saturation and these inflammatory indices. Conclusions: While cyanotic congenital heart disease demonstrates marked hematological adaptation secondary to chronic hypoxemia, systemic inflammatory indices appear similar in cyanotic and acyanotic patients. These findings suggest a relative dissociation between hypoxemia-driven hematological responses and the evaluated systemic inflammatory indices, indicating that inflammatory burden in congenital heart disease may not be solely explained by cyanosis and may reflect additional underlying mechanisms not captured by these markers. Full article

Background/Objectives: Children with congenital heart disease are exposed to ionizing radiation, which may induce cancer. This study aimed to reassess cancer risk after cardiac catheterization (CC) between 1999 and 2013, with follow-up until 15 years of age, cancer diagnosis, or death. Methods: We studied 2762 children who underwent at least one CC before eight years of age between 1999 and 2013. Cancer diagnoses were obtained from the German Childhood Cancer Registry. For patients with tumors and 60 randomly selected control patients, cumulative effective radiation doses (Deff) were calculated. Results: During 344,80 person-years of follow-up, ten patients developed cancer, whereas 5.3 cases were expected (standardized incidence ratio [SIR] 1.88; 95% CI 0.90–3.46; p = 0.0449). Eight tumors occurred in patients who underwent CC during the first year of life, compared with 3.5 expected (SIR 2.26; 95% CI 0.98–4.46; p = 0.0282). Patients with cancer had a median of 2.0 (1–11) CCs and a median D_eff of 14.6 mSv (2.4–94.3) compared with 1.0 (1–10) CCs and 9.7 mSv (0.7–171.5) in controls. Neither parameter differed significantly. No specific malignancy was predominant. Conclusion: Cardiac catheterization early in life remains associated with an increased cancer risk; however, compared with our previously published 1980–1998 cohort, a reduction in risk was observed. Full article

Background: Congenital heart disease (CHD) represents a major cause of perinatal morbidity and mortality, and fetal echocardiography is essential for its early diagnosis and management. Maternal smoking has been suggested as a potential teratogenic factor affecting fetal cardiovascular development; however, findings regarding its association with CHD remain inconsistent. This study aimed to evaluate the relationship between maternal smoking during pregnancy and the risk of CHD. Methods: A total of 2715 pregnant women and 2784 fetuses who underwent fetal echocardiography at ≥20 weeks’ gestation between 1 January 2024 and 1 November 2025 were analyzed. Pregnancies complicated by known chromosomal or syndromic abnormalities, significant teratogenic exposure, duplicate assessments, or nonstandard examinations were excluded. Maternal smoking status during pregnancy was recorded and categorized according to daily cigarette consumption. The prevalence of CHD and the distribution of CHD subtypes were evaluated and compared according to smoking status. Fetal cardiac diagnoses were classified based on the classical morphological classification system. Results: A total of 2715 pregnancies (2784 fetuses) were analyzed, including 2530 fetuses in the non-smoking group and 254 in the smoking group. Congenital heart disease was detected in 12.5% of fetuses in the non-smoking group and 14.2% in the smoking group, with no statistically significant difference (p = 0.442). According to the classical morphological classification, the distribution of fetal echocardiographic pathologies did not differ significantly between groups (p = 0.607). Septal defects were the most common subtype in both groups. Although conotruncal defects were proportionally more frequent in the smoking group, this difference did not reach statistical significance. After reclassifying daily cigarette consumption into four exposure categories, no association was detected between maternal smoking and CHD risk (OR = 1.04; 95% CI: 0.86–1.26; p = 0.691). Conclusion: In this cohort referred for fetal echocardiographic evaluation, no association was detected between maternal smoking during pregnancy and the risk of congenital heart disease or alterations in CHD subtype distribution. No consistent dose–response relationship was observed. These findings suggest that no association was detected between maternal smoking exposure and CHD. Further large-scale prospective studies are needed to clarify phenotype-specific associations. Full article

Background: Neonatal Ebstein’s anomaly (EA) is a severe condition with significant hemodynamic instability and early myocardial dysfunction, where abnormal right-heart geometry limits conventional echocardiography and highlights the value of myocardial deformation imaging. Methods: We conducted a single-center retrospective observational study including 16 neonates with EA and 26 healthy neonates. All subjects underwent comprehensive transthoracic echocardiography during the neonatal period. Conventional two-dimensional imaging and speckle-tracking echocardiography (STE) were used to assess biventricular and biatrial myocardial deformation. Deformation parameters were compared between groups, and receiver operating characteristic (ROC) curve analysis evaluated diagnostic performance. Results: Neonates with EA demonstrated significant structural remodeling and severe biventricular and biatrial dysfunction compared with controls. Speckle-tracking showed markedly reduced right ventricular longitudinal strain (LS) in all segments (all, p < 0.001), particularly in free-wall and four-chamber views. Left ventricular (LV) global LS (GLS) was significantly reduced in neonates with EA compared with controls (−14.53% vs. −22.32%, p < 0.001), indicating early involvement of LV myocardial function in the neonatal period. Atrial reservoir, conduit, and contractile strain were severely impaired in both atria (all, p < 0.001). ROC analysis revealed excellent diagnostic accuracy, especially for LVGLS (AUC 0.919) and right atrial contractile strain (AUC 0.958). Conclusions: STE enables the early detection of extensive biventricular and biatrial myocardial dysfunction in neonatal EA, including abnormalities not fully captured by conventional echocardiographic parameters, thereby providing significant incremental diagnostic value. Full article

Objectives: The objective of this study was to compare fetal MAPSE and TAPSE values in preeclamptic pregnancies with those in healthy pregnancies and to examine the changes in these parameters according to the severity of preeclampsia. Methods: This prospective case–control study enrolled 77 women with preeclampsia and 81 healthy pregnant controls. Fetal MAPSE and TAPSE values were obtained under standardized conditions by experienced operators using M-mode ultrasonography. Results: Fetal mitral annular plane systolic excursion (MAPSE) and tricuspid annular plane systolic excursion (TAPSE) values were found to be significantly lower in the preeclampsia group compared with the control group (p < 0.001). In analyses evaluating preeclampsia cases within themselves, fetal MAPSE and TAPSE values were found to be more significantly reduced in the preeclampsia with severe features group compared to the preeclampsia without severe features group. Conclusions: Fetal MAPSE and TAPSE values, measured by M-mode ultrasonography, were found to be significantly lower in the preeclampsia group compared to the control group. The more pronounced decrease in these values, particularly in preeclampsia with severe features cases, suggests that MAPSE and TAPSE measurements may be early indicators of fetal cardiovascular adaptation to the impaired intrauterine environment. Full article

Background/Objectives: A known disadvantage of extracardiac Fontan is the absence of growth potential and potential late flow stagnation compared to lateral tunnel Fontan. This study investigates the differences in changes in the cross-sectional area and impact on liver fibrosis. Methods: The anteroposterior and lateral diameters of the Fontan pathways were measured using angiograms. Cross-sectional area and the indexed cross-sectional area were calculated, and their relation to Fibrosis-4 index was analyzed. Results: A total of 334 angiograms of 224 patients (212 extracardiac and 12 lateral tunnel Fontan) were evaluated. The median age at Fontan was 2.2 (Interquartile Range: 1.8–2.9) years. The median period from Fontan to angiogram was 3.3 (0.04–10.8) years. Cross-sectional areas remained unchanged in extracardiac Fontan patients and increased in lateral tunnel Fontan patients. The indexed cross-sectional areas in extracardiac Fontan patients decreased over time. The smallest indexed-cross-sectional areas were 200 mm²/m² in extracardiac Fontan patients at 10 years postoperatively, whereas indexed cross-sectional areas in lateral tunnel Fontan patients were larger and more variable. Fibrosis-4 index increased time-dependently in both groups. Indexed cross-sectional area at the smallest level <156 mm²/m² was identified as a risk factor for liver fibrosis. Conclusions: The Fontan pathway expanded in patients after lateral tunnel Fontan, whereas indexed cross-sectional area decreased over time after extracardiac Fontan. Importantly, in patients after extracardiac Fontan, narrowing of the Fontan pathway might be one of the risk factors for progression of liver fibrosis. Full article

Soluble growth stimulation protein form of interleukin-1 receptor-like 1 (ST2) may signal myocardial stress, and elevated ST2 blood levels are associated with adverse outcomes in adult heart disease. Data on ST2 in children with congenital heart disease (CHD) is limited. This study explored ST2 in newborns and older children with atrial septal defect (ASD), as this represents a common CHD type that remains clinically challenging to recognize in childhood with slowly evolving symptoms. A case–control study was carried out in newborn ASD cases versus controls measuring ST2 on dried blood spot samples and additionally in pediatric ASD cases versus controls on venous blood together with cardiac magnetic resonance before and after treatment. ST2 was higher in newborns with ASD (n = 19) compared to controls (n = 93); (p < 0.01). Receiver operating characteristics to diagnose newborn ASD by ST2 showed an area under the curve of 0.848. Levels of ST2 decreased in pediatric ASD (n = 16) after treatment (p = 0.014). Lower left ventricular ejection fraction correlated with higher ST2 levels before (r = −0.348) and after treatment (r = −0.497). Elevated ST2 in newborns may aid early ASD diagnosis. Levels of ST2 in pediatric ASD decrease after treatment, and higher levels are associated with lower left ventricular ejection fraction, warranting further study. Full article

Background/Objectives: Portable and wearable ECG technologies are increasingly used in adult cardiac monitoring. However, evidence supporting their feasibility and diagnostic performance in pediatric populations remains limited. This systematic review evaluates the diagnostic accuracy, usability, artifact susceptibility, and user acceptance of mobile ECG technologies in pediatric cardiology. Methods: A systematic literature search was performed in the Embase, PubMed, Scopus, and Web of Science databases. The review was conducted in accordance with the PRISMA 2020 guidelines and was registered in the PROSPERO database. Results: A total of 30 publications were included in the final analysis. Portable ECG devices demonstrated good feasibility diagnostic utility in children. Handheld systems provided high-quality tracings with strong agreement with standard 12-lead ECGs and higher adherence, as well as user satisfaction compared with conventional event recorders. However, automated rhythm classification frequently misidentified pediatric arrhythmias. Smartwatch-based ECG recordings showed high diagnostic accuracy when manually interpreted, but automated algorithms were unreliable, particularly for tachyarrhythmias and conduction abnormalities. Alternative electrode placement strategies improved smartwatch performance, and patient acceptance was consistently high. ECG patch monitoring, particularly with extended-wear devices, achieved the highest diagnostic yield, detecting arrhythmias often missed by short-duration Holter monitoring while maintaining comparable signal quality. Conclusions: Mobile ECG technologies represent a promising adjunct for pediatric rhythm surveillance, offering diagnostic performance comparable to standard modalities when interpreted by clinicians and improved usability and patient acceptance. Persistent limitations include the poor reliability of adult-oriented automated algorithms and the underrepresentation of younger children and the predominantly off-label use of these devices in pediatric populations, underscoring the need for pediatric-specific algorithm development and age-adapted device design. Full article

Background: Arrhythmogenic cardiomyopathy (ACM) is an inherited disorder characterized by fibrofatty replacement of cardiomyocytes. The inflammatory episodes of ACM, known as the “hot phase”, can mimic acute myocarditis. It was seldom observed in a DES-associated ACM as a “hot-phase” presentation. Case Presentation: The proband, a 13-year-old female, initially presented with a series of clinical manifestations of fulminant myocarditis. Although recommendation-guided anti-immunotherapy had been provided, this patient still developed into an aggressive cardiomyopathy with biventricular dilation and severe systolic heart failure. Additionally, cardiac magnetic resonance demonstrated circumferential late gadolinium enhancement in left ventricular myocardium with diffuse fibrosis. Whole-exon sequencing identified a de novo missense variant, as c.335T>A (p.L112Q) of the DES gene, resulting in protein dysfunction. And a diagnosis of ACM due to a DES variant had been identified. Finally, this patient received heart transplantation, and biventricular fibrofatty infiltration was confirmed by pathological analysis. Conclusions: This case presented a de novo genetic variant that can induce severe and aggressive heart failure. This finding emphasizes the importance of comprehensive genetic analysis in patients suspected of having fulminant myocarditis, which would greatly benefit the precise clinical management and outcomes. Full article

Background/Objectives: Cardiovascular manifestations in pediatric Marfan syndrome (MFS) exhibit substantial heterogeneity. Early identification of patients at elevated risk of requiring cardiac surgery is essential to optimizing outcomes. This study aimed to determine phenotypic features associated with cardiovascular surgery in genetically confirmed pediatric MFS. Methods: Among the 1006 children evaluated, 214 with genetically verified MFS were included in the analysis. We categorized patients by the presence or absence of cardiac surgery during childhood. Systemic and cardiovascular features were assessed. We applied binary logistic regression to identify independent associated manifestations with surgical intervention. Results: 20/214 patients (9.3%, 11.5 ± 5.5 years) underwent cardiac surgery (50% aortic root replacement, 20% mitral valve surgery, 30% combined interventions). Extracardiac features—Marfan-type facial features, pectus carinatum, pes planus, hindfoot deformity, and myopia of ≥3 diopters—were significantly associated with an increased probability of surgery (OR 3.0–4.6). Tricuspid valve prolapse and pulmonary artery dilatation were more prevalent in surgical patients. Surgical patients exhibited higher systemic manifestation scores (9.2 vs. 5.2; p ≤ 0.05) per revised Ghent criteria (RGC). Conclusions: A higher systemic score (RGC) correlates with increased risk for surgery. Marfan-type facial features, pectus carinatum, pes planus, hindfoot deformity, and myopia ≥ 3 diopters were strongly associated with the need for early cardiac surgery. Comprehensive phenotypic assessment, including systemic manifestation scoring, enables risk stratification and supports timely surgical planning in pediatric MFS. Full article

Introduction: Food insecurity (FI) is a social driver that profoundly affects the health of children. Nutritional optimization is essential in patients with congenital heart disease (CHD). Material and Methods: We performed a cross-sectional survey screening for FI among patients aged 0–21 years at an outpatient pediatric cardiology clinic between September 2023 and December 2024. Sociodemographic and clinical data from encounters were collected, and diagnostic codes were used to classify CHD severity. The zip code-level median household income was determined using data from the U.S. Census. The Childhood Opportunity Index categorization was used to determine neighborhood-level resources. Univariate and multivariate logistic regression were used to assess sociodemographic associations with FI. Results: There were 955 encounters with completed FI screening. Positive screens were demonstrated in 200 surveys (20.9%). Compared to English-speaking White families, those with FI were more likely to be of Hispanic ethnicity (66% vs. 45.2%) and primarily speak Spanish (42.5% vs. 15.0%). Families with FI also lived in areas with lower median household income and fewer available resources. In multivariable analysis, after adjusting for ethnicity, income, and neighborhood-level resource availability, Spanish primary language was the only independent risk factor associated with FI (OR 2.7, 95% CI 1.7–4.2, p < 0.0001). There were no differences in FI status by CHD severity. Conclusions: FI was highly prevalent in this cohort and was associated with low-income and low-resource neighborhoods, Hispanic ethnicity, and a Spanish primary language. These results may have implications for targeting future FI interventions. Full article

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. Full article

Background: Manganese (Mn) is an essential trace element with antioxidant properties; however, excessive exposure may contribute to inflammation and vascular dysfunction. Hair analysis provides an indicator of long-term Mn exposure. This study evaluated the relationship between hair Mn levels, acute coronary syndrome (ACS), coronary artery disease (CAD) severity, and cardiovascular risk factors, with particular emphasis on metabolic status in a cardiometabolic population. Methods: Hair Mn concentration was measured using inductively coupled plasma optical emission spectrometry (ICP-OES) in 80 patients (mean age 67 ± 11 years; 28.8% women) undergoing coronary angiography for suspected ACS. Final diagnoses included stable CAD (N = 42) and ACS [ST-elevation myocardial infarction (STEMI) N = 17, non-ST-elevation myocardial infarction (NSTEMI) N = 12, and unstable angina (UA) N = 9]. CAD severity was quantified using the SYNTAX score and the Coronary Artery Surgery Study Score (CASSS). Associations with clinical variables were assessed using non-parametric tests and Spearman correlations. The median SYNTAX score was 13.8 (range 0.0–68.5), and the median hair Mn concentration was 0.22 ppm (range 0.01–1.65). Results: SYNTAX scores were higher in ACS than in stable CAD (p = 0.027), with the highest values observed in NSTEMI. Hair Mn levels did not differ among diagnostic groups and showed no association with CASSS or SYNTAX (R = −0.11; p = 0.348). No differences were detected with respect to sex, smoking, prior myocardial infarction, hypertension, hyperlipidemia, or type 2 diabetes. A modest inverse correlation was observed between hair Mn and body mass index (BMI) in unadjusted analysis (R = −0.25; p = 0.03), but this association was not robust after correction for multiple comparisons, suggesting a potential exploratory link between manganese homeostasis and cardiometabolic status. Conclusions: Although hair Mn concentration was not associated with angiographic indices of CAD severity or ACS subtypes, the observed relationship with BMI may indicate a role of Mn homeostasis in cardiometabolic regulation. Larger prospective studies are required to clarify these associations. Full article