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Introduction: Assessment 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. Methods: Conducted 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. Results: After 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. Conclusions: The 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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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.

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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 threedimensional 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.

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INTRODUCTION: Interventional ablation has been demonstrated to represent an effective therapy in patients with atrial fibrillation (AF), leading to restoration and maintenance of sinus rhythm in the majority of cases. However, recurrence of AF is encountered in 35% to 40% of cases, and the causes for this frequent complication have not been elucidated so far. MATERIAL AND METHODS: Here we present the study protocol of the FIBRO-RISK trial, a prospective, single-center, cohort study which aims to investigate the impact of inflammatory-mediated myocardial fibrosis on the risk of recurrence after successful catheter ablation of atrial fibrillation. The level of systemic inflammation in the pre-ablation and immediate post-ablation period will be assessed on the basis of serum levels of inflammatory biomarkers (hsCRP, matrix metalloproteases, interleukin-6), while the level of cardiac fibrosis will be determined based on cardiac magnetic resonance imaging associated with complex post-processing techniques for mapping myocardial fibrosis at the level of left atrium and left ventricle. At the same time, the amount of epicardial fat will serve as an indirect marker of localized inflammation and will be determined at different levels in the heart (surrounding left atrium, right atrium or the entire heart), while ventricular function will be assessed on the basis of serum levels of NT-proBNP prior to the procedure. All these parameters will be investigated in patients with successful ablation of AF, who will be divided into 2 groups: group 1 - patients who develop AF recurrence at 1-year, and group 2 - patients with no recurrence of AF at 1-year. In all patients, the following biomarkers will be determined: serum levels of inflammatory biomarkers and NT-proBNP at 24 hours and 1-year post procedure, the amount of myocardial fibrosis at the level of left atrium and left ventricle at baseline +/- 7 days, and the amount of epicardial fat surrounding left atrium, right atrium and the entire heart at baseline +/- 7 days.The primary endpoint of the study will be represented by the rate of AF recurrence at 1-year post ablation, documented by either ECG or Holter monitoring. The secondary endpoints of the study will consist in:In conclusion, FIBRO-RISK will be the first CMR-based study that will investigate the impact of inflammation-mediated myocardial fibrosis and ventricular remodeling on the risk of recurrence after successful ablation of AF, aiming to validate inflammatory biomarkers and myocardial fibrosis as predictors for AF recurrence.

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We present the case of a 17-year-old girl with Ebstein anomaly and repeated episodes of reentrant tachycardia due to a right posterior accessory pathway. Catheter ablation was performed using intracardiac echocardiography. A ViewFlex Xtra probe was inserted and showed an anormal tricuspid valve with elongated anterior leaflet and low insertion of the septal leaflet towards the apex. The anatomical annulus was identified by the course of the right coronary artery. RF application on the posterior annulus stopped the reentrant arrhythmia. After ablation, programmed stimulation showed absence of both antegrade and retrograde conduction through the accessory pathway.

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Fetal common arterial trunk is an anomaly represented by a unique arterial trunk that arouses from the base of the heart, and gives birth to systemic branches, both pulmonary and coronary, frequently associated with a ventricular septal defect (VSD) and has a poor prognosis. We present a series of 17 cases diagnosed in our tertiary center with different types of fetal common arterial trunk, its associated disorders, the evolution of the pregnancies, and of the neonates. We concluded that our cases support the fact that a complete intrauterine evaluation of each case of the common arterial trunk is impossible. The postnatal prognosis of the cases from our center was fatal, similar to most reports of the literature.

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Supraventricular tachyarrhythmia represents the most frequent fetal dysrhythmia. In the lack of diagnosis and treatment these fetuses may develop hydrops and even death. For the therapeutic approach it is important to establish the diagnosis of the type of supraventricular tachycardia. In this paper we report 29 cases with different types of supraventricular tachycardia in which the diagnosis was established using our own protocol, which allowed us to make the difference between the types of tachycardia (atrioventricular reentry tachycardia due to the accessory pathway, atrial ectopic tachycardia and permanent junctional reciprocal tachycardia). We acquired the data by a series of recordings in M mode and pulsed Doppler by simultaneous recording of an artery and a vein flow. First of all, we diagnosed the supraventricular tachycardia type, with short or long ventriculoarterial interval, and afterwards, we made the difference between atrial ectopic tachycardia and permanent junctional reciprocal tachycardia using methods to decrease the atrioventricular conduction.

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INTRODUCTION: Total anomalous pulmonary venous connection is an uncommon congenital heart disease. Four types are described based on the site of pulmonary venous drainage: supracardiac, cardiac, infradiaphragmathic, and mixed connection. In most cases of supracardiac type, the common venous confluence drains through an ascending left vertical vein into the brachiocephalic vein, right superior vena cava, and then into the right atrium. Anomalous connection of the pulmonary venous confluence directly to the right SVC, especially the obstructed form is an unusual and severe supracardiac variant. The prenatal diagnosis is challenging. PATIENT CONCERNS: We present a case report of a fetus diagnosed with TAPVC at 23 gestational weeks. DIAGNOSIS INTERVENTIONS: The 4-chamber view showed a small left atrium, with a "smooth" posterior wall and the absence of pulmonary vein connection. This is the first case of prenatally diagnosed isolated, obstructed supracardiac type with drainage directly into the right superior vena cava. CONCLUSION: The obstetrician and fetal cardiologist should be cautious at the direct and indirect echocardiographic signs of this condition. A prenatal diagnose of isolated, obstructed form is important for adequate planning of delivery and postnatal surgery in a tertiary center.

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Congenital heart disease is the most common congenital anomaly, representing an important cause of infant morbidity and mortality. Congenital heart disease represents a group of heart anomalies that include septal defects, valve defects, and outflow tract anomalies. The exact genetic, epigenetic, or environmental basis of congenital heart disease remains poorly understood, although the exact mechanism is likely multifactorial. However, the development of new technologies including copy number variants, single-nucleotide polymorphism, next-generation sequencing are accelerating the detection of genetic causes of heart anomalies. Recent studies suggest a role of small non-coding RNAs, micro RNA, in congenital heart disease. The recently described epigenetic factors have also been found to contribute to cardiac morphogenesis. In this review, we present past and recent genetic discoveries in congenital heart disease.

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BACKGROUND: Right ventricular function has been identified as an important prognostic factor in children with pulmonary arterial hypertension. The aim of the study was to assess the deformation pattern and prognostic value of right ventricular longitudinal strain in children with pulmonary arterial hypertension. METHODS: We prospectively evaluated 46 children (25 with pulmonary arterial hyperetension and 21 age and sex matched controls) using conventional and speckle-tracking echocardiography, brain natriuretic peptide levels and clinical status expressed by WHO functional class and 6-min walking test. According to the clinical status after 12 months of follow-up, the pulmonary arterial hypertension patients were divided in two groups: non-worsening (13) and worsening (12). RESULTS: Right ventricular free wall longitudinal strain and strain rate were significantly lower in children with PAH compared with controls (-24.72 ± 3.48 vs -15.60 ± 3.40, p = 0.0001 and -1.44 ± 0.09 vs -1.09 ± 0.15, p = 0.0001, respectively). There was a more expressed decrease of basal than apical region of right ventricular free wall longitudinal strain/strain rate in pulmonary arterial hypertension patients compared with controls (strain: p = 0.0001 vs p = 0.07 and strain rate: p = 0.0001 vs p = 0.002). Comparing worsening and non-worsening pulmonary arterial hypertension patients there was a significant difference in Mid right ventricular free wall longitudinal strain (-14.00 ± 3.13 vs. -20.76 ± 4.62, p = 0.0001). In multivariable logistic regression analysis Mid right ventricular free wall longitudinal strain was an independent predictor of worsening in pulmonary arterial hypertension children (OR 0.45; 95 % CI: 0.21-0.96, p = 0.041). In ROC curve analysis a cut-off value of Mid right ventricular free wall longitudinal strain of -18.50 % predicted clinical worsening in pulmonary arterial hypertension children, with a sensitivity and specificity of 91.7 and 30.8 %, respectively (area under curve = 0.88 ± 0.06, 95 % CI: 0.75-1.00, p = 0.001). CONCLUSIONS: Two-dimensional speckle-tracking echocardiography is a complementary non-invasive tool for assessment of right ventricular function in children with severe pulmonary arterial hypertension, allowing also clinical prediction and segmental analysis of right ventricular myocardial performance in these patients.

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