IntroductionAssessment of myocardial function through speckle tracking echocardiography (STE) can bring benefits to conventional echocardiography in premature newborns, a particular vulnerable group in terms of adaptation to extra-uterine life. Furthermore, it represents a non-invasive imagistic method which can guide therapeutic approach in the hemodynamically unstable newborn. This study aims to highlight the particularities of myocardial function in late premature newborns, by conducting a comparison with a group of healthy neonates, by using STE.MethodsConducted over a timespan of two years, this prospective study enrolled 64 term neonates and 21 premature newborns, with gestational ages ranging between 28 and 36 weeks, who prior to discharge underwent a cardiac ultrasound, involving two-dimensional image acquisitions of the apical four-chamber view of both ventricles. Afterwards, the images were offline analyzed, by using the autostrain function.ResultsAfter segmental strain analysis, no significant discrepancies between the two groups in terms of interventricular values were found. However, left ventricle and right ventricle strain measurements differed significantly (p < 0.01), for each of the analyzed segments (basal, medial or apical). Moreover, a linear increase in interventricular (IV) basal strain with corrected gestational age progression was noted (p = 0.04). Peak global longitudinal strain (pGLS) and EF were similar between the two study groups. Premature newborns presented significantly more negative mean values of right ventricular free wall longitudinal strain (RVFWSL), (−24.19 ± 4.95 vs. −18.05 ± 5.88, p < 0.01) and of right ventricle global four chamber longitudinal strain (RV4CSL), (−19.71 ± 3.62 vs. −15.46 ± 5.59, p < 0.01), when compared to term neonates.ConclusionsThe 2D STE is a reliable method for cardiac assessment of late preterm newborns. The evaluation of two-dimensional global longitudinal LV and RV strains might represent a useful tool in clinical practice. A better response of the right ventricle to the longitudinal deformation within premature neonates was noted. Thus, this study facilitates the identification of accurate reference values for this particular population segment, which will enable the evaluation of ventricular function in premature newborns with concurring disorders. Future longitudinal studies, assessing the fetal heart, could provide more insight into the development of myocardial function.

The aim of this systematic literature review was to assess the data regarding neuromarkers used to evaluate the impact of cardiovascular surgery on neurodevelopmental pattern of children with congenital heart defects. A systematic search was performed on PubMed and Google Scholar databases. Out of 713 publications screened, 10 studies (471 patients) met the inclusion criteria. The included studies were coded on several variables: number and heterogeneity of patients (age, congenital heart defects), exclusion of patients with conditions that predispose to neurological impairment, neuroimaging workup pre- and post-surgery, neurodevelopmental assessment, interventions (part of a different study), and follow-up period. Results were reported according to PRISMA guidelines. Findings include: neuron-specific enolase and brain-derived neurotrophic factor are not reliable neuromarkers, for protein S100B different results were reported, for activin A there is lack of evidence, and glial fibrillary acidic protein could represent a reliable neuromarker for acute brain-injury. Directions for future research are discussed.

Background: Several studies in recent years have shown the association between vitamin D levels and heart failure. Vitamin D deficiency is related to increased cardiovascular morbidity and mortality, with a higher risk of developing heart failure. In this systematic review, we aimed to assess recent studies that analyzed vitamin D deficiency and heart failure in adult and pediatric populations. (2) Methods: We conducted a systematic search for studies published in the following databases: PubMed and Scopus from January 2012 to October 2022. (3) Results: Most observational studies that were included found a significant association between hypovitaminosis D and heart failure. However, the beneficial role of vitamin D supplementation is still controversial due to the lack of randomized controlled trials. (4) Conclusions: Vitamin D may play an important role as a cardiovascular marker in heart failure patients. More well-designed studies are needed to investigate the relationship between vitamin D and heart failure and to determine if vitamin D supplementation could improve long-term outcomes.

Data regarding reference intervals for strain parameters derived from 2D speckle-tracking echocardiography in full-term newborns are limited and still under development. Our objectives were to establish the level of reproducibility and reference intervals in assessing myocardial function using 2D speckle-tracking echocardiography for longitudinal and regional strain measurements. A total of 127 full-term newborns were examined to be included in the study, of which 103 were analyzed. We used two-dimensional acquisitions from apical four-chamber view of both ventricles and analyzed the autostrain function offline. We obtained interobserver agreement between the two observers ranging from good to excellent for all speckle-tracking parameters except for the strain of the medial portion of the left ventricle (LV) lateral wall and the strain measured on the basal portion of the inter-ventricular septum, which reflected a fair interobserver reproducibility (ICC = 0.52, 95% IC: 0.22–0.72 and ICC = 0.43, 95% IC: 0.12–0.67, respectively). The reference values obtained for the LV peak longitudinal strain were between −24.65 and −14.62, those for the right ventricle (RV) free wall were from −28.69 to −10.68, and those for the RV global four-chamber were from −22.30 to −11.37. In conclusion, two-dimensional peak longitudinal LV and RV strains are reproducible with good to excellent agreement and may represent a possible alternative for the cardiac assessment of healthy newborns in the clinical practice.

Sustained fetal tachycardias are rare but represent a high risk of mortality and morbidity. Consensus has yet to be found regarding their optimal management. The aim of this narrative review is to summarize the data available in the current literature regarding the efficacy and safety of medications used in the management of intrauterine tachyarrhythmias and to provide possible treatment protocols. In this review, we would like to emphasize the importance of a thorough evaluation of both the fetus and the mother, prior to transplacental antiarrhythmic drug initiation. Factors such as the hemodynamic status of the fetus, possible mechanisms of fetal arrhythmia, and concomitant maternal conditions are of primordial importance. As a possible treatment protocol, we would like to recommend the following: due to the risk of sustained supraventricular tachycardia (SVT), fetuses with frequent premature atrial beats should be evaluated more frequently by echocardiography. A careful hemodynamic evaluation of a fetus with tachycardia is primordial in forestalling the appearance of hydrops. In the case of atrial flutter (AFL), sotalol therapy could represent a first choice, whereas when dealing with SVT patients, flecainide should be considered, especially for hydropic patients. These data require consolidation through larger scale, non-randomized studies and should be handled with caution.

Background: Children born with single-ventricle hearts require surgery in order to survive. Liver fibrosis is a known complication of Fontan surgery for univentricular hearts. Methods: In this study on 13 post-Fontan and 21 post-Glenn patients, we used elastography (shearwave and transient elastography) as well as serum biochemical fibrosis markers to evaluate the degree of liver fibrosis in comparison to 32 controls. Results: The mean E-median and V-median values determined by shear wave elastography in the Fontan Group were significantly higher than the controls (4.85 kPa vs. 3.91 kPa and 1.25 m/s vs. 1.12 m/s, respectively). Fontan patients had significantly increased Fibrotest, Actitest, AST-to-Platelet Ratio index, ALT and GammaGT levels compared to controls. For post-Glenn patients, the mean E-median and V-median values were similar to healthy controls, whereas the Fibrotest, Actitest and AST-to-Platelet Ratio index were significantly increased. Using transient elastography, we found significantly higher values for E-median and V-median in Fontan patients compared to Glenn patients. Conclusions: Elastography and biochemical fibrosis markers are valuable non-invasive tools for screening and monitoring liver fibrosis in patients with Fontan and Glenn interventions.

Our aim was to compare the global longitudinal and regional biventricular strain between infants with severe and critical pulmonary stenosis (PS), and controls; to compare pre- and post-procedural strain values in infants with severe and critical PS; and to assess the correlations between echocardiographic strain and conventional parameters. We conducted a retrospective single-center study. The comparisons of echocardiographic variables were performed using separate linear mixed models. The overall mean right ventricle (RV) regional strains measured before intervention in PS patients was significantly different when compared to the control group (p = 0.0324). We found a significant change in the left ventricle, RV, and inter-ventricular septum strain (IVS) values from basal to apical location (p < 0.05). IVS strain values showed a higher decrease in mean strain values from basal to apical in PS patients. There was no significant difference in means of baseline and post-interventional strain values in PS patients (p > 0.05). Following the strain analysis in patients with PS, we obtained statistically significant changes in the RV global-4-chamber longitudinal strain (RV4C). The RV4C, which quantifies the longitudinal strain to the entire RV, can be used in current clinical practice for the evaluation of RV function in infants with severe and critical PS. The longitudinal and segmental strain capture the pathological changes in the IVS, modifications that cannot be highlighted through a classical echocardiographic evaluation.

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.

Pulmonary arterial hypertension is a severe, progressive disease in children, that causes right ventricular dysfunction over time. Tissue motion annular displacement is a novel speckle-tracking derived echocardiographic parameter used in assessing ventricular function. The aim of our study was to determine the prognostic value of this echocardiographic parameter in children with pulmonary arterial hypertension. We conducted a case-control study by assessing twenty children with pulmonary arterial hypertension (idiopathic or secondary) and twenty age- and sex-matched controls, using clinical (WHO functional class, 6-min walking test), laboratory (brain natriuretic peptide level) and echocardiographic parameters (conventional and speckle-tracking derived tissue motion annular displacement) at enrolment and after one year of follow-up. According to their WHO functional class altering after one year, the pulmonary arterial hypertension patients were divided into two groups: non-worsening (eleven) and worsening (nine). The conventional echocardiographic parameters and all measured tricuspid tissue motion annular displacement indices (lateral, septal, midpoint and midpoint fractional displacement-TMADm%) were significantly lower in both pulmonary arterial hypertension groups (non-worsening and worsening) compared to controls. Comparing the worsening and non-worsening groups, only the TMADm% and brain natriuretic peptide level was significantly lower in worsening in comparison with non-worsening pulmonary arterial hypertension children (p = 0.010 and p = 0.018, respectively). In receiver-operating characteristic curve analysis, we found a cut-off value of 16.15% for TMADm% and a cut-off value of 34.35 pg/mL for the brain natriuretic peptide level that can predict worsening in pulmonary arterial hypertension children. In conclusion, tricuspid annulus midpoint fractional displacement, an angle-dependent speckle-tracking derived parameter, could be a good additional parameter in the assessment of the longitudinal right ventricular systolic function and in prediction of clinical worsening in children with pulmonary arterial hypertension.

Aim: Congenital atrioventricular block (CAVB) is an immunological condition, secondary to the transfer of maternal Ig G antibodies from seropositive mothers. Although the presence of these antibodies is high among pregnant women, the preva-lence of this fetal pathology is low. The aim of this paper is to analyze a series of cases with intrauterine diagnosis of CAVB and to present their follow-up protocol. Material and method: In the period between 2013-2020, five fetuses were diagnosed and followed up in the Pediatric Cardiology Clinic. In each of the cases, assessment of the hemodynamic status was done by calculation of the fetal cardiovascular profile score (CVPS). In the last cases the follow-up protocol was supplemented with longitudinal speckle tracking evaluation of the ventricular function. Results: In the present series, intrauterine death occurred in one case; in another case resumption of atrioventricular conduction was observed. Epicardial pacemaker implantation was required in three of the patients. Conclusion: Completing the evaluation of ventricular function with the longitudinal speckle tracking method in fetuses and newborn patients with congenital atrioventricular block may play an important role in establish-ing therapeutic behavior.

INTRODUCTION:Coarctation of the aorta represents a narrowing of the thoracic aorta. Hypertensive patients with blood pressure differences ≥20 millimetres of mercury have an indication for surgical or interventional treatment. Implantation of a covered stent became the preferred therapy for the management of this pathology in adolescents/adults.CASE REPORT:We report the case of a 14-year-old male sportsman, who presented in the emergency room with headache, dizziness, and tinnitus. The clinical exam revealed blood pressure differences between the upper and lower limbs of up to 50 mmHg. Based on the clinical and paraclinical data, we established the diagnosis of coarctation of the aorta and severe secondary arterial hypertension. The case was discussed by a multidisciplinary team and accepted for covered stent implantation. The 24 h blood pressure Holter monitoring after the procedure indicated the persistence of stage I arterial hypertension.CONCLUSIONS:Coarctation of the aorta is a congenital cardiovascular anomaly with high morbidity and mortality rates. Arterial hypertension, heart failure, and aortic dissection are complications of this pathology, some of them being sometimes direct consequences of secondary hypertension. Periodic cardiology follow up after the procedure is mandatory to assess the hemodynamic response, to identify potential complications, and to stratify the cardiovascular risk.

Turner syndrome (TS) is the most common chromosomal abnormality affecting females and cardiac abnormalities have been described in up to 50% of patients. Although coarctation represents one of the most frequent cardiac malformation, treatment options in these patients represent an area of debate, due to associated aortopathy and risk for aortic dissection. In addition to the contradictory data found in the literature, regarding the safety profile and utility of stenting of coarctation of the aorta in TS patients, we present the case of a patient of pediatric age, who successfully underwent the procedure, being free from periprocedural and short-term complications. Beside the presentation itself, we aimed to review and summarize the data available in the literature regarding this topic. As a conclusion, we emphasize the role of minimally invasive interventional therapy and wish to underline the need of further, larger scale studies and guidelines in this patient group. Given the related aortopathy, all preventive measures should be undertaken to avoid aortic dissection during stent implantation in this vulnerable population. Although the evolution of our patient was favorable, data found in the literature is somewhat contradictory and a close follow-up is indicated to help evaluate the risk of long-term complications.

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.

Our objectives are to compare speckle-tracking peak global longitudinal (pGLS) and regional strain values in neonates with coarctation of aorta (CoA) and control groups. Echocardiographic parameters measured by speckle-tracking were studied in a retrospective single-center study. A comparison of pGLS and segmental deformation between neonates with CoA and control group was performed using a three-way mixed ANOVA model. There was a significant difference in the means of segmental strain values between CoA and control group at the apical (p = 0.018) and basal segments (p = 0.031) of the interventricular septum and at the apical segment (p = 0.026) of the left ventricle (LV). After correcting for multiple comparisons, the results had a tendency toward statistical significance (adjusted-p < 0.10). There was significant difference in the mean values of pGLS [F(1, 39) = 7.61, p = 0.009, adjusted p = 0.018] between the studied groups. The results of ROC analysis showed that a cut-off value of −16.60% for pGLS provided an estimated sensitivity of 92.31% (95% CI: [63.97, 99.81]) and 71.43% specificity (95% CI: [51.33, 86.78]) for the diagnosis of CoA in neonates (AUC = 0.794, 95% CI: [0.66, 0.93]). pGLS can be regarded as a feasible and reproducible parameter reflecting LV dysfunction in newborns with CoA when compared to newborns with a false-positive diagnosis.

Background: Multimodality imaging improves the accuracy of cardiac assessment in patients with prior myocardial infarction. The aim of this study was to investigate the association between coronary plaque vulnerability (PV) and myocardial viability in the territory irrigated by the infarct-related artery (IRA). Secondary objectives include evaluation of the systemic inflammation but also different cardiac risk scores (SYNTAX score, Duke jeopardy score, or calcium score) using hybrid imaging models of coronary computed tomography angiography (CCTA) and cardiac magnetic resonance (CMR) in patients who have suffered a previous myocardial infarction (MI). Material and methods: The study included 45 subjects with documented MI in the 30 days prior to study enrolment, who underwent CCTA and CMR examinations. Computational postprocessing of CCTA and CMR images was used to generate fused imaging models. Based on the vulnerability degree of the associated non-culprit lesion located proximally in the IRA, the study population was divided into 3 groups: Group 1 – subjects with no sign of vulnerability (n = 7); Group 2 – subjects with 1 or 2 CT vulnerability features (n = 28); and Group 3 – subjects with >2 features of vulnerability (n = 12). Results: CCTA features indicative for the severity of coronary artery disease were not different between groups in terms of calcium scoring (460 ± 501 vs. 579 ± 430 vs. 432 ± 494, p = 0.7) or SYNTAX score (25 ± 9.2 vs. 24.9 ± 8.3 vs. 20.2 ± 11.9, p = 0.4). However, after 1 month, infarct size and the Duke jeopardy score were associated with increased PV (infarct size 8.77 ± 3.4 g in Group 1, compared to 20.87 ± 8.3 g in Group 2 and 27.99 ± 11.8 g in Group 3 (p = 0.007), while the Duke jeopardy score was 4.4 ± 1.6 in Group 1, vs. 7.07 ± 2.1 in Group 2 vs. 7.5 ± 1.73 in Group 3 (p = 0.01). Inflammatory biomarkers were directly associated with coronary plaque vulnerability (p = 0.007 for hs-CRP and p = 0.038 for MMP-9). Conclusion: In patients with prior myocardial infarction, the size of myocardial scar was directly correlated with the vulnerability degree of coronary plaques and with systemic inflammation quantified during the acute phase of the coronary event. Hybrid imaging may help to identify the hemodynamically significant plaques with superior accuracy.

Introduction. Coarctation of the aorta (CoA) occurs in 6-8% of patients with congenital heart disease, with an incidence of 4/10,000 live births. It can be present with various clinical pictures, in an age range between newborn and adolescence. Infants with birth weights ≤ 2 kg with CoA have increased risk of severe intraventricular hemorrhage (IVH) compared to their birth weight-matched cohorts. Case presentation. We present a case of a premature, male newborn, with low birth weight, who at the age of 3 weeks developed signs and symptoms of cardiogenic shock. Clinical examination, echocardiography and angio-CT confirmed the diagnosis of a severe CoA, without any flow through the ductus arteriosus. Treatment with prostaglandin E1 was initiated, to promote ductal patency. The systolic blood pressures were above 99 percentile for his gestational age in the upper extremities, with a noninvasive pressure gradient around 86 mmHg between the arm and leg. Transfontanellar ultrasonography evaluation revealed a grade II/III bilateral IVH, in resorption, with cystic transformation. At the age of 6 weeks definitive surgical repair was performed. Postoperatively, at the 3 month follow-up, the clinical suspicion of recoarctation of the aorta arised, confirmed by echocardiography and angio-CT, with arterial hypertension and gradient around 37 mmHg between the arm and leg. Prosthetic patch aortoplasty was performed. The postoperative evolution was marked by the persistence of arterial hypertension, the infant requiring continued treatment with angiotensin-converting enzyme inhibitor. Conclusions. Premature neonates with low birth weight have always constituted a medical management challenge, with short and long term implications. A strong point in solving such cases is a multidisciplinary medical for assessment, management and outcomes.

Development of interventional methods has revolutionized the treatment of structural cardiac diseases. Given the complexity of structural interventions and the anatomical variability of various structural defects, novel imaging techniques have been implemented in the current clinical practice for guiding the interventional procedure and for selection of the device to be used. Three- dimensional echocardiography is the most used imaging method that has improved the three-dimensional assessment of cardiac structures, and it has considerably reduced the cost of complications derived from malalignment of interventional devices. Assessment of cardiac structures with the use of angiography holds the advantage of providing images in real time, but it does not allow an anatomical description. Transesophageal Echocardiography (TEE) and intracardiac ultrasonography play major roles in guiding Atrial Septal Defect (ASD) or Patent Foramen Ovale (PFO) closure and device follow-up, while TEE is the procedure of choice to assess the flow in the Left Atrial Appendage (LAA) and the embolic risk associated with a decreased flow. On the other hand, contrast CT and MRI have high specificity for providing a detailed description of structure, but cannot assess the flow through the shunt or the valvular mobility. This review aims to present the role of modern imaging techniques in pre-procedural assessment and intraprocedural guiding of structural percutaneous interventions performed to close an ASD, a PFO, an LAA or a patent ductus arteriosus.

Abstract Introduction: The incidence of cardiac arrhythmia is approximately 1% in the neonatal period and 1–3% in late pregnancy. Atrial flutter (AF), a rhythm disorder based on the mechanism of reentry, represents approximately 32% of all neonatal cardiac arrhythmias. In the majority of cases, the flutter is converted to sinus rhythm using antiarrhythmic drugs, transesophageal overdrive pacing, or synchronized electrical cardioversion. Case presentation: We present a case of a born on term, female infant who was noted to be tachycardic on the fetal monitor. Clinical examination revealed tachypnea and tachycardia with dysrhythmia, at a heart rate of 250 bpm. Electrocardiography showed AF with 2–3 : 1 atrioventricular conduction (atrial and ventricular rates were 350 bpm and 250–275 bpm, respectively). Echocardiography revealed no relevant structural disease. Therapy with amiodarone and prophylactic anticoagulant was initiated. As no control of ventricular rate was obtained and a succession of three ventricular extrasystoles was noted on the surface ECG, propranolol therapy was initiated, without success. Therefore, synchronized electrical cardioversion was applied, with conversion to sinus rhythm at a heart rate of 136 bpm. The neonate was discharged in good condition. Conclusions: AF is one of the most common high ventricular rate arrhythmias during fetal age. Uncontrolled AF may precipitate heart failure, and prompt restoration to sinus rhythm may require electrical cardioversion in cases refractory to anti-arrhythmic drugs, in order to prevent possible complications.

This study aimed to investigate possible associations of the susceptibility to congenital heart defects (CHDs) with AXIN1 rs1805105, rs12921862 and rs370681 gene variants and haplotypes, and AXIN2 rs2240308 gene variant. Significant associations were identified for AXIN1 rs370681 and AXIN2 rs2240308 variants. AXIN1 rs370681 variant was significantly associated with decreased odds of CHDs (adjusted OR varying from 0.13 to 0.28 in codominant, dominant and recessive gene models), while the AXIN2 rs2240308 variant was associated with increased odds of CHD in the dominant model. The haplotype-based generalized linear model regression of AXIN1 rs1805105, rs12921862 and rs370681 variants revealed that C-C-C and C-C-T haplotypes significantly increased the risk of CHDs (p < 0.05). No significant second order epistatic interactions were found between investigated variants (AXIN1 rs1805105, rs12921862, rs370681, and AXIN2 rs2240308). Our conclusion is that AXIN1 rs1805105, rs12921862, and rs370681 (C-C-C and C-C-T) haplotypes and AXIN2 rs2240308 contribute to CHDs susceptibility.

Pulmonary arterial hypertension (PAH) is a rare but severe complication that should be investigated in patients diagnosed with transposition of the great arteries who have undergone neonatal arterial switch operation. Early diagnosis and aggressive combination therapy for PAH could help to improve survival in these patients. We report a favorable clinical response 6 months after the initiation of vasodilator therapy in a pediatric case with transposition of the great arteries, successfully repaired in the neonatal period, who developed pulmonary arterial hypertension at the age of 5 years.