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Background: Ventricular function assessments in Fontan patients remain challenging. Ejection fraction (EF) lacks sensitivity for early dysfunction, and the roles of strain and advanced imaging in systemic left ventricle (LV) physiology are not fully defined. We aimed to compare (i) LV and atrial strain indices between pediatric Fontan patients with preserved EF (P-LVEF) versus reduced EF (R-LVEF) and (ii) echocardiographic global longitudinal strain, segmental longitudinal strain indices, and conventional 2D and 3D echocardiographic parameters through cardiac morphology. Methods: Pediatric patients with Fontan circulation and systemic LV morphology underwent clinical, hemodynamic, and multimodality echocardiographic evaluation, including 2D/3D parameters, global and segmental LV strain, and left atrial strain. Outcomes were analyzed according to EF status and congenital morphology. Significant results from multiple comparisons were followed by post hoc analysis, where appropriate. Results: Patients with a reduced EF exhibited a worse clinical status, a higher pulmonary vascular resistance index, and greater systemic congestion compared with those with a preserved EF. Conventional 2D indices showed no significant differences between the two studied groups except for LV end-systolic volume (ESV) (p = 0.0315) and LV end-systolic longitudinal diameter (ESL) (p = 0.0024), which showed higher values in the R-LVEF group. Although the relative frequency of impaired deformation was higher in Fontan patients with an unbalanced atrioventricular canal compared with the Fontan patients with a tricuspid atresia + pulmonary stenosis + ventricular septal defect, the difference did not reach statistical significance (p = 0.1365). Most segmental longitudinal strain values were not significantly different across patients with different cardiac morphology, except for the basal anterior segment and apical inferoseptal segment (p < 0.05). Conclusions: In pediatric Fontan patients with systemic LV morphology, a reduced EF was associated with a worse clinical and hemodynamic status. Conventional echocardiographic indices showed a limited ability to differentiate between the compared groups. Although no statistically significant differences were detected between pediatric Fontan patients with preserved EF and reduced EF, LV and atrial strain indices provided complementary information on ventricular–atrial interactions and myocardial deformation. These findings are exploratory and warrant confirmation in larger, prospective studies.

Background: Multisystem inflammatory syndrome in children (MIS-C) is a severe post-infectious complication of SARS-CoV-2, often with cardiac involvement. Myocardial strain imaging may detect dysfunction missed by conventional echocardiography. The objectives of this study are to characterize cardiac manifestations of MIS-C and assess the value of strain imaging in children with preserved and reduced left ventricular ejection fraction (LV-EF). Methods: We retrospectively analyzed 22 MIS-C patients admitted between September 2020 and January 2024, all with cardiac involvement. Clinical, laboratory, and echocardiographic data—including 2D and speckle-tracking strain—were collected at the day of worst dysfunction (DWD) and discharge (DD) and compared with 22 matched controls. Results: Median age was 4.65 years; 59% male; 45% overweight/obese. LV systolic dysfunction (LV-EF < 50%) occurred in 54.5%, coronary abnormalities in 36.4%, and pericardial effusion in 95.5%. LV global longitudinal strain (LVGLS) was significantly lower than controls at the DWD (−15.45 ± 4.76%, p < 0.0001) and DD (−20.63 ± 4.66%, p = 0.014). Strain abnormalities persisted despite LV-EF recovery, and even patients with preserved LV-EF showed significant segmental strain reduction. LVGLS and apical infero-septal strain were strongest predictors of reduced LV-EF. Conclusions: MIS-C often causes systolic dysfunction and coronary changes, but strain imaging reveals persistent subclinical myocardial injury. Long-term cardiac monitoring is warranted.

Background/Objectives: Children with single left ventricle (SLV) anatomy following Fontan palliation are at high risk for subclinical ventricular dysfunction, which may not be detected by conventional echocardiographic measures. Our objectives are as follows: (1) to assess myocardial and atrial strain profiles in pediatric Fontan patients with SLV using 2-dimensional speckle-tracking echocardiography (2D-STE), (2) to compare these findings with a healthy control group, (3) to investigate correlations with conventional echocardiographic and functional parameters. Methods: A single-center study of 66 pediatric patients, who underwent echocardiographic evaluation and a 6 min walk test (6 MWT). Conventional, 3D, and strain-based echocardiographic parameters were compared between groups. Correlations with clinical and functional indices were assessed using ANCOVA, analysis, generalized additive models, and Pearson’s correlation coefficient. Results: Fontan patients showed significantly reduced 6 MWT distances compared to controls (mean difference: 201.6 m, p < 0.0001). Post-test heart rate (HR) and oxygen saturation were significantly impaired (HR: 104.6 vs. 100.8 bpm, p = 0.0012; SaO2: 90.3% vs. 99.8%, p < 0.0001). Fontan patients showed statistically significant differences in nearly all the 2D parameters. Three-dimensional echocardiography revealed significantly lower left ventricular (LV) ejection fraction (p = 0.0020), higher end-diastolic (p = 0.0275) and end-systolic volumes (p = 0.0125) in the study group. Global longitudinal strain (LV_GLS) was reduced in Fontan patients compared to controls (p < 0.0001), with significant differences across nearly all LV segments. Left atrial (LA) reservoir and conduit strain were markedly decreased, while contractile strain remained similar. LV_GLS was negatively correlated with IVCT (r = −0.50, p = 0.0175). The LA reservoir strain (LASr_AC) significantly correlated with MAPSE (r = 0.43, p = 0.0461). Conclusions: In pediatric Fontan patients, myocardial and atrial strain imaging reveals subclinical dysfunction despite preserved conventional ejection fraction.

Background/Objectives: The development of normal fetal cardiac function, a dynamic process that has not yet been precisely documented throughout the literature, is difficult to quantify by classic echocardiography. Our aim was to analyze the function of the fetal myocardium through speckle tracking and establish reference values for global and segmental longitudinal strain for both ventricles in fetuses with a gestational age (GA) between 22 and 39 weeks. Methods: We conducted a prospective study in which 170 fetuses underwent echocardiographic evaluation and those 150 that were eligible for the study underwent offline speckle tracking analysis. Results: A mixed-design ANOVA model with Greenhouse–Geisser correction showed no significant differences in regional strain measurements among GA groups (F [2, 147] = 1.25, p = 0.289) but showed significant differences in regional strain measurements among the right ventricle (RV), left ventricle (LV), and interventricular free wall (Greenhouse–Geisser F [1.3, 195.2] = 45.70, p < 0.001, GG ε = 0.66, original df = 2, 294). The wall-by-segment interaction term of the model was statistically significant for regional strain (Greenhouse–Geisser F [2.7, 394.2] = 27.00, p < 0.001, GG ε = 0.67, original df = 4, 588), while the segment-by-gestational age group term had a tendency toward statistical significance (Greenhouse–Geisser F [3.0, 221.4] = 2.21, p = 0.088, GG ε = 0.75, original df = 4, 294). The results of Welch’s ANOVA model showed no significant difference in right-ventricle peak global longitudinal strain (pGLS) between GA groups (F [2.0, 92.2] = 0.52, p = 0.5972) and global longitudinal strain measurements (F [2.0, 89.6] = 27.00, p = 0.3733). Conclusions: The reference values for longitudinal strain, represented by the pGLS for LV, ranged from −20.79 to −8.05 for fetuses with a GA between 22 and 27 weeks, from −20.14 to −8.99 for fetuses with a GA between 28 and 33 weeks, and from −20.19 to −8.88 for fetuses with a GA between 34 and 39 weeks. For RV pGLS, the reference values were between −18.99 and −6.35, also depending on GA. Reference ranges for the large gestational groups studied can help us to recognize subtle changes in fetal cardiac function.

Hypertrophic cardiomyopathy (HCM) is a myocardial disease characterized by abnormal thickening of the ventricular myocardium. It is most commonly inherited as an autosomal dominant disorder caused by mutations in sarcomere or sarcomere-associated protein genes. We report the case of a 15-year-old female with HCM and a strong family history (mother, sister, and maternal grandfather). Despite this background, her diagnosis was made incidentally following the onset of cardiac symptoms. Genetic testing confirmed a pathogenic MYH7 mutation. Based on elevated risk scores (HCM-Risk Kids = 8.6%, Primacy Risk Score = 13.09), she was considered at high risk for sudden cardiac death and underwent implantation of an implantable cardioverter-defibrillator (ICD) for primary prophylaxis. This case highlights the importance of a comprehensive approach to pediatric and adolescent HCM, including family history, genetic testing of at-risk relatives, early diagnosis, and multidisciplinary management. It also emphasizes the urgent need for systematic family screening of first-degree relatives using echocardiography and electrocardiography. Although genetic testing confirmed the diagnosis in our patient, it could not be extended to relatives due to financial limitations. Expanding access to genetic screening at a national level should be a priority. Future research should focus on optimizing genetic testing protocols and improving quality-of-life interventions for young patients with HCM and ICDs.

Background/Objectives: Bicuspid aortic valve (BAV) is a prevalent congenital heart defect that continues to present a significant challenge in the management of paediatric patients. The assessment of left ventricle systolic function is typically conducted through the measurement of the left ventricular ejection fraction. Currently, left ventricle global longitudinal strain (LV GLS) is regarded as a more sensitive indicator, enabling the quantitative assessment of global and segmental ventricular function through the determination of myocardial deformation. Methods: A prospective study was conducted between 10 January 2023 and 10 January 2024 in a tertiary paediatric cardiology referral centre. The study enrolled children aged 6 to 17 years with BAV who were undergoing periodic evaluation, as well as a control group. The primary objective was to analyse the systolic function (global and segmental LV) using the classical method (LV EF) and speckle tracking echocardiography (STE). Results: The study group comprised 73 patients with a mean age of 13 years and was predominantly male. The control group comprised 55 patients. The phenotype IB with aortic regurgitation (AR) was the most prevalent. The results of the STE evaluation in the control group demonstrated mean GLS values between −22.1% and −22.8%. A comparison of the BAV group and the control group revealed a significant difference in GLS for the apical four-chamber view (p = 0.022). Conclusions: Although the analysis of global LV function demonstrated normal values of EF in patients with BAV, the strain analysis revealed significantly reduced strain in the inferior segment and in the apical four-chamber view, as well as in the anterior segment. Further investigation is required to determine whether reduced LV GLS in paediatric patients with BAV will ultimately result in the development of clinical heart failure. Additionally, it is necessary to ascertain whether this can identify patients with subclinical heart failure and whether early detection can result in a reduction in morbidity.

This study focuses on two of the most prevalent congenital heart defects: bicuspid aortic valve (BAV) and coarctation of the aorta (CoA). In severe forms, CoA is an emergency that can become life-threatening. Both diseases are linked to aortic vasculopathy, which represents a significant contributor to morbidity and mortality in young patients with congenital heart disease. The aim of this study was to investigate whether there are differences between patients with CoA and BAV, and patients with CoA and without BAV in terms of outcome and echocardiographic parameters, and to evaluate the efficacy of different echocardiographic parameters in predicting CoA. A retrospective and prospective observational analytic study was conducted between January 2018 and October 2024 at a tertiary pediatric cardiology referral center enrolling patients aged between 0 and 17 years, diagnosed with CoA, who underwent surgical CoA repair. The common carotid-subclavian artery index (CSAI) and the aortic isthmus-descending aorta index (I/D) were calculated based on echocardiographic measurements. A total of 101 patients underwent surgical or interventional treatment for CoA in the studied period, 58.4% being neonates. Based on echocardiographic examination, 68.3% of patients had BAV with CoA. The mean value of the carotid-subclavian index was 0.95 in neonates, compared to 1.11 in children (p = 0.035). No statistically significant difference was observed in the mean value of the aortic isthmus-descending aorta index between the two groups (p = 0.237). The implications of the coexistence of the two conditions are not yet fully understood. This study did not find significant echocardiographic differences between patients with CoA and BAV and patients with CoA and without BAV. Further long-term, multicenter, multi-arm studies with larger groups are required in these areas.

We present the case of a neonatal patient with atrial tachycardia with unusual characteristics – abrupt onset and cessation, non-responsiveness to overdrive, termination by direct cardio-version – in association with an ostium secundum-type atrial septal defect and a large inter-atrial septum aneurysm. Electrocardiographic recordings showed P waves with characteristics suggesting a right low septal origin, which suggested a possible involvement of the septal aneurysm. In dealing with arrhythmias, a thorough echocardiographic evaluation is mandatory to identify underlying structural malformations and to evaluate the hemodynamic status. Further research should be made concerning mechanisms of arrhythmias in patients with an associated atrial septum aneurysm.

Scimitar syndrome is a rare congenital cardiovascular malformation that includes a partially anomalous drainage of the pulmonary veins in the inferior vena cava, right pulmonary hypoplasia, and systemic-pulmonary collaterals originating from various segments of the aorta. We present a case of Scimitar syndrome with associated intracardiac lesions and a large arterial conduct supplying the right lung, originating from the abdominal aorta and draining in the inferior vena cava.

Congenital aortic stenosis (AS) occurs in around 0.2–0.5% of newborns, and its clinical severity is quite variable. Some of the newborns with AS require urgent medical care: prostaglandin infusion, balloon aortic valvuloplasty, or surgical intervention. Despite having a severe clinical evolution in neonates, the prenatal diagnosis of congenital AS is quite low. We present the case of a fetus with critical AS, who had been prenatally diagnosed at 35 weeks of gestation, via fetal cardiac ultra-sound. The echocardiographic parameters revealed a severely depressed left ventricular systolic function, with dilated chambers, and a severe aortic stenosis. Offline speckle-tracking analysis was performed in order to aid in deciding the optimal methods and timing of delivery. Left ventricular analysis revealed a severely impaired global longitudinal strain of 2.1%, left ventricular ejection fraction 18.4%, increased LV volumes, while the right ventricular function was only mildly depressed. Therefore, the decision was to delay the premature delivery, and the fetus was born at a gestational age of 38 weeks, in a hospital with a neonatal cardiovascular surgery department. The patient had undergone surgical repair of the cardiac anomaly at 3 days after birth.