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

ObjectivesTo study changes in heart function and hemodynamics during the transitional period in small for gestational (SGA) infants and appropriate (AGA) healthier counterparts.DesignA 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.ResultsEighteen 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].ConclusionsSGA 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

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-β) 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

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-β) 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

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

Total Anomalous Pulmonary Venous Connection in Children

Introduction: The aim of this study was to study the anatomical types of total anomalous pulmonary venous connection (TAPVC), the associated cardiac and extracardiac congenital malformations, clinical manifestations, and postoperative evolution

A Novel Speckle-Tracking Echocardiography Derived Parameter That Predicts Clinical Worsening in Children with Pulmonary Arterial Hypertension

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

A Novel Speckle-Tracking Echocardiography Derived Parameter That Predicts Clinical Worsening in Children with Pulmonary Arterial Hypertension

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

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

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

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

A rare case of cerebral hemorrage in a pre-term infant with coarctation of the aorta

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