Comparative assessment of myocardial function between late premature newborns and term neonates using the 2D speckle tracking method.

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.

The Importance of Aortic Valve Bicuspid Phenotype in Valvular Evolution in Pediatric Patients: A Case Report and Literature Mini-Review.

Bicuspid aortic valve (BAV) is the most commonly encountered congenital malformation in the pediatric population, associated with aortic leaflet degeneration and aortopathy. However, studies on BAV and its complications in children are limited. We present the case of a 16-year-old with type 1B BAV with a raphe with fusion between the right and non-coronary cusps who exhibited severe aortic stenosis, regurgitation, and progressive dilatation of the ascending aorta. Surgical intervention, including aortic valve and aortic root replacement, was performed due to the patient's deteriorating condition. Histopathological examination revealed degenerative changes and calcifications in the aortic valve and mucoid fibrosis in the ascending aorta. The results are consistent with BAV patients being predisposed to aortic stenosis and regurgitation due to increased mechanical stress and hemodynamic abnormalities. Although more common in adults and a rare complication in pediatric patients, calcification was previously observed concurrently with rapid valve degeneration in our daily practice. Further studies are needed to improve our understanding of the mechanisms underlying BAV-related complications and refine treatment strategies for pediatric patients.

Normative echocardiography data of myocardial adaptation to extrauterine life: a review of prospective studies.

Recent research has increased focus and interest in characterizing the physiology of the transition circulation using echocardiography. Critique of published normative neonatal echocardiography data among healthy term neonates has not been performed. We have performed a comprehensive literature review using the key terms: cardiac adaptation, hemodynamics, neonatal transition, term newborns. Studies were included if they had reported echocardiography indices of cardiovascular function in the presence of maternal diabetes, intrauterine growth restricted newborns and prematurity and had a comparison group of healthy term newborns within first seven postnatal days. Sixteen published studies evaluating transitional circulation in healthy newborns were included. There was marked heterogeneity in the methodologies used; specifically, inconsistency in time of evaluation and imaging techniques used makes it challenging to determine specific trends of expected physiologic changes. Some studies revealed nomograms for echocardiography indices, though limitations persist in terms of sample size, number of reported parameters and consistency of measurement technique. A comprehensive standardized echocardiography framework which includes consistent techniques for assessment dimensions, function, blood flow, pulmonary/systemic vascular resistance, and shunts pattern is warranted to ensure consistency in the use of echocardiography to guide care of healthy and sick newborns.

The Role of Galectin-3 in Predicting Congenital Heart Disease Outcome: A Review of the Literature.

Galectin-3 (Gal-3) is a novel pro-fibrotic biomarker that can predict both right and left cardiac dysfunction caused by various cardiovascular conditions. Its expression seems to be progressively altered with evolving cardiac remodeling processes, even before the onset of heart failure. Hence, Gal-3 has been found to be an individual predictor of acute and chronic heart failure or to serve as part of an integrated biomarker panel that can foresee adverse cardiac outcomes. In congenital heart disease (CHD), Gal-3 correlates with cardiac mortality and complications in both children and adults and is proposed as a therapeutic target in order to reverse the activation of pro-fibrosis pathways that lead to heart failure. Positive associations between serum Gal-3 levels, post-operatory hospitalization rates, complications and ventricular dysfunction have also been reported within studies conducted on patients with CHD who underwent corrective surgery. Thus, this review tried to address the potential utility of Gal-3 in patients with CHD and particularly in those who undergo corrective surgery. The heterogeneity of the literature data and the lack of validation of the results obtained by the current studies on larger cohorts cannot be neglected, though. Further longitudinal research is required to establish how Gal-3 can relate to long-term outcomes in pediatric CHD.

RAF-1 Mutation Associated with a Risk for Ventricular Arrhythmias in a Child with Noonan Syndrome and Cardiovascular Pathology.

Introduction: Noonan syndrome (NS) is a dominant autosomal disease, caused by mutations in genes involved in cell differentiation, growth and senescence, one of them being RAF1 mutation. Congenital heart disease may influence the prognosis of the disease. Case presentation: We report a case of an 18 month-old female patient who presented to our institute at the age of 2 months when she was diagnosed with obstructive hypertrophic cardiomyopathy, pulmonary infundibular and pulmonary valve stenosis, a small atrial septal defect and extrasystolic arrhythmia. She was born from healthy parents, a non-consanguineous marriage. Due to suggestive phenotype for NS molecular genetic testing for RASopathies was performed in a center abroad, establishing the presence of RAF-1 mutation. Following rapid progression of cardiac abnormalities, the surgical correction was performed at 14 months of age. In the early postoperative period, the patient developed episodes of sustained ventricular tachycardia with hemodynamic instability, for which associated treatment was instituted with successful conversion to sinus rhythm. At 3-month follow-up, the patient was hemodynamically stable in sinus rhythm. Conclusions: The presented case report certifies the importance of recognizing the genetic mutation in patients with NS, which allows predicting the severity of cardiac abnormalities and therefore establishing a proper therapeutic management of these patients.

The Predictive Role of Plasma Biomarkers in the Evolution of Aortopathies Associated with Congenital Heart Malformations.

Dilatation of the aorta is a constantly evolving condition that can lead to the ultimate life-threatening event, acute aortic dissection. Recent research has tried to identify quantifiable biomarkers, with both diagnostic and prognostic roles in different aortopathies. Most studies have focused on the bicuspid aortic valve, the most frequent congenital heart disease (CHD), and majorly evolved around matrix metalloproteinases (MMPs). Other candidate biomarkers, such as asymmetric dimethylarginine, soluble receptor for advanced glycation end-products or transforming growth factor beta have also gained a lot of attention recently. Most of the aortic anomalies and dilatation-related studies have reported expression variation of tissular biomarkers. The ultimate goal remains, though, the identification of biomarkers among the serum plasma, with the upregulation of circulating MMP-1, MMP-2, MMP-9, tissue inhibitor of metalloproteinase-1 (TIMP-1), asymmetric dimethylarginine (ADMA), soluble receptor for advanced glycation end-products (sRAGE) and transforming growth factor beta (TGF-ß) being reported in association to several aortopathies and related complications in recent research. These molecules are apparently quantifiable from the early ages and have been linked to several CHDs and hereditary aortopathies. Pediatric data on the matter is still limited, and further studies are warranted to elucidate the role of plasmatic biomarkers in the long term follow-up of potentially evolving congenital aortopathies.

Assessment of Biventricular Myocardial Function with 2-Dimensional Strain and Conventional Echocardiographic Parameters: A Comparative Analysis in Healthy Infants and Patients with Severe and Critical Pulmonary Stenosis.

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.

Comparative evaluation of echocardiography indices during the transition to extrauterine life between small and appropriate for gestational age infants.

Objectives: To study changes in heart function and hemodynamics during the transitional period in small for gestational (SGA) infants and appropriate (AGA) healthier counterparts. Design: A hospital based prospective observational study was performed at a perinatal center. Echocardiograms were performed on the first postnatal day and again at 48 h age. Term SGA infants were compared with those AGA newborns matched for the GA and mode of delivery. Results: Eighteen SGA infants were compared with 18 AGA infants [gestation 38 ± 1.5 vs. 38 ± 1.2 weeks, p > 0.05 and birthweight 2331 ± 345 vs. 3332 ± 405 grams, p < 0.05, respectively]. Maternal weight and body mass index was higher among non-affected pregnancies, 61% infants were born vaginally, and no differences in cord blood pH at birth were noted. SGA infants had higher systolic and mean blood pressure at both time points, lower indices of right ventricular (RV) performance [TAPSE (tricuspid annular peak systolic excursion) 7.4 ± 2.8 vs. 9.3 ± 0.7 on day 1, 7.2 ± 2.8 vs. 9.2 ± 0.5 on day 2, p = 0.001], lower pulmonary acceleration time (PAAT) suggestive of elevated pulmonary vascular resistance [56.4 ± 10.5 vs. 65.7 ± 13.2 on day 1, 61.4 ± 12.5 vs. 71.5 ± 15.7 on day 2, p = 0.01] and higher left ventricular (LV) ejection fraction [62.1 ± 7.8 vs. 54.9 ± 5.5 on day 1, 61.9 ± 7.6 vs. 55.8 ± 4.9 on day 2, p = 0.003]. Conclusions: SGA infants had evidence of higher pulmonary vascular resistance, and lower RV performance during the postnatal transition. The relevance and impact of these changes to hemodynamic disease states during the postnatal transition requires prospective investigation.

Diagnostic Difficulties in a Case of Fetal Ventricular Tachycardia Associated with Neonatal COVID Infection: Case Report.

The clinical course of COVID in the pediatric population is considered to be much milder when compared to adults; however, the occurrence of severe and fatal forms of the disease in children is non-negligible, especially in patients with comorbidities such as prematurity or cardiac disease. We report a case of a newborn with sotalol-controlled fetal ventricular tachycardia, who was postnatally diagnosed with COVID infection. The myocardial injury was sustained on the basis of pericardial effusion, left ventricular dysfunction, rapid progression to coronary artery dilation, and an arrhythmic storm. We believe that, in our case, there is a significant overlap between fetal ventricular tachycardia, associated with impaired left ventricular function, and COVID infection, diagnosed after birth; both factors contribute to the myocardial dysfunction with a fulminant clinical evolution. To our knowledge, this is the first case describing neonatal myocardial dysfunction associated with SARS-CoV infection complicating the clinical course of rare fetal tachyarrhythmia.

Implantation of Covered Stent for Coarctation of the Aorta and Secondary Hypertension in Adolescents-Case Report.

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.

Speckle-Tracking Global Longitudinal and Regional Strain Analysis in Neonates with Coarctation of Aorta: A Case-Control Study.

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.