Anomalous aortic origin of coronary arteries (AAOCA) represents a rare congenital heart disease. However, this disease is the second most common cause of sudden cardiac death in apparently healthy athletes. The aim of this systematic review is to analyze the feasibility and the detection rate of AAOCA by echocardiography in children and adults. A literature search was performed within the National Library of Medicine using the following keywords: coronary artery origin anomalies and echocardiography; then, the search was redefined by adding the keywords: athletes, children, and high take-off. Nine echocardiographic studies investigating AAOCA and a total of 33,592 children and adults (age range: 12–49 years) were included in this review. Of these, 6599 were athletes (12–49 years). All studies demonstrated a high feasibility and accuracy of echocardiography for the evaluation of coronary arteries origin as well as their proximal tracts. However, some limitations exist: the incidence of AAOCA varied from 0.09% to 0.39% (up to 0.76%) and was lower than described in computed tomography series (0.3–1.8%). Furthermore, echocardiographic views for the evaluation of AAOCA and the definition of “minor” defects (e.g., high take-off coronary arteries) have not been standardized. An echocardiographic protocol to diagnose the high take-off of coronary arteries is proposed in this article. In conclusion, the screening of AAOCA by echocardiography is feasible and accurate when appropriate examinations are performed; however, specific acoustic windows and definitions of defects other than AAOCA need to be standardized to improve sensitivity and specificity.

Lung ultrasound (LUS) is increasing in its popularity for the diagnosis of pulmonary complications in acute pediatric care settings. Despite the high incidence of pulmonary complications for patients with pediatric cardiovascular and congenital heart disease, especially in children undergoing cardiac surgery, the use of LUS remains quite limited in these patients. The aim of this review is to provide a comprehensive overview and list of current potential applications for LUS in children with congenital heart disease, post-surgery. We herein describe protocols for LUS examinations in children, discuss diagnostic criteria, and introduce methods for the diagnosis and classification of pulmonary disease commonly encountered in pediatric cardiology (e.g., pleural effusion, atelectasis, interstitial edema, pneumothorax, pneumonia, and diaphragmatic motion analysis). Furthermore, applications of chest ultrasounds for the evaluation of the retrosternal area, and in particular, systematic search criteria for retrosternal clots, are illustrated. We also discussed the potential applications of LUS, including the guidance of interventional procedures, namely lung recruitment and drainage insertion. Lastly, we analyzed current gaps in knowledge, including the difficulty of the quantification of pleural effusion and atelectasis, and the need to differentiate different etiologies of B-lines. We concluded with future applications of LUS, including strain analysis and advanced analysis of diaphragmatic mechanics. In summary, US is an easy, accurate, fast, cheap, and radiation-free tool for the diagnosis and follow-up of major pulmonary complications in pediatric cardiac surgery, and we strongly encourage its use in routine practice.

Background. Despite current trends toward early primary repair, the surgical systemic-to-pulmonary shunt is still considered the first-choice palliation in patients with critical tetralogy of Fallot (TOF) and duct-dependent pulmonary circulation unsuitable for primary repair. However, stenting of the right ventricular outflow tract (RVOT) is nowadays emerging as an effective alternative to surgical palliation in selected patients. Methods and results. RVOT stenting is usually performed from a venous route, either femoral or, in selected cases, the right internal jugular vein. Less frequently, mostly in pulmonary infundibular/valvar atresia, this procedure can be performed using a hybrid surgical/interventional approach by surgical exposure of the RVOT, puncture of the atretic valve, and stent deployment under direct vision. The size and type of the most appropriate stent may be chosen, based on ultrasound measurements of the RVOT, to cover the right ventricular infundibulum completely and, at the same time, sparing the pulmonary valve, unless significant pulmonary valve annulus hypoplasia and/or supra-valvular stenosis is a significant component of the obstruction. In the large series so far published, early mortality of RVOT stenting is less than 2%, comparing favourably with either Blalock-Thomas-Taussig shunt or early primary repair. In addition, morbidity and clinical sequelae of this approach do not significantly differ from surgical palliation, even if RVOT stenting shows lesser durability and a higher rate of trans-catheter re-interventions over a mid-term follow-up. Finally, similar but more balanced pulmonary artery growth than surgical palliation following RVOT stenting is reported over a mid-term follow-up. Conclusions. RVOT stenting is a technically feasible, well-tolerated, and effective palliation in critical TOF. This approach is cost-effective with respect to surgical palliation either in high-risk neonates or whenever a short-term pulmonary blood flow source is anticipated due to the early surgical repair. It effectively increases pulmonary blood flow, improves arterial saturation, and promotes balanced pulmonary artery growth over a mid-term follow-up.

Background: Speckle-tracking echocardiography (STE) has gained increasing value in the evaluation of congenital heart diseases (CHD); however, its use in pediatric cardiac surgery is limited. Aim: To evaluate left ventricular (LV) systolic impairment after biventricular pediatric cardiac surgery by STE strain (ε) analysis. Methods: We prospectively enrolled 117 children undergoing cardiac surgery for CHD. Echocardiography was performed at four different times: pre-operatively, 12–36 h (Time 1), 3–5 days (Time 2), and 6–8 days (Time 3). Images were obtained in the 4-2-and 3 apical chamber’s views to derive LV global and regional (basal/mid/apical) ε values. Results: At different postoperative times, we performed 320 examinations in 117 children (mean age: 2.4 ± 3.9, range: 0–16 years); 117 age-matched healthy children served as controls. All global, basal, and mid LVε values decreased after surgery; the lowest values being at Time 1 (p < 0.0001), which increased thereafter. At discharge, all global, basal, and mid LVε values remained lower than in pre-operative and healthy children (p < 0.05). Instead, apical segments (lowest at baseline) increased after surgery (p < 0.0001) but remained lower compared to controls. LV ejection fraction (LVEF) decreased at Time 1 (p = 0.0004) but promptly recovered to Time 2 and normalized at Time 3. Conclusions: STE ε analysis revealed a significant LV systolic impairment after surgery with amelioration thereafter but incomplete normalization at discharge. Base-apex differences emerged with apical segments that, contrary to all the other regions, showed relative hypercontractility after surgery. The slower recovery of LVε values compared to LVEF suggests that STE ε analysis may be more accurate for the follow-up of mild LV post-surgical impairment.

The aim of the present review is to highlight the strengths and limitations of echocardiographic parameters and scores employed to predict favorable outcome in complex congenital heart diseases (CHDs) with borderline right ventricle (RV), with a focus on pulmonary atresia with intact ventricular septum and critical pulmonary stenosis (PAIVS/CPS). A systematic search in the National Library of Medicine using Medical Subject Headings and free-text terms including echocardiography, CHD, and scores, was performed. The search was refined by adding keywords “PAIVS/CPS”, Ebstein’s anomaly, and unbalanced atrioventricular septal defect with left dominance. A total of 22 studies were selected for final analysis; 12 of them were focused on parameters to predict biventricular repair (BVR)/pulmonary blood flow augmentation in PAIVS/CPS. All of these studies presented numerical (the limited sample size) and methodological limitations (retrospective design, poor definition of inclusion/exclusion criteria, variability in the definition of outcomes, differences in adopted surgical and interventional strategies). There was heterogeneity in the echocardiographic parameters employed and cut-off values proposed, with difficultly in establishing which one should be recommended. Easy scores such as TV/MV (tricuspid/mitral valve) and RV/LV (right/left ventricle) ratios were proven to have a good prognostic accuracy; however, the data were very limited (only two studies with <40 subjects). In larger studies, RV end-diastolic area and a higher degree of tricuspid regurgitation were also proven as accurate predictors of successful BVR. These measures, however, may be either operator and/or load/pressure dependent. TV Z-scores have been proposed by several authors, but old and heterogenous nomograms sources have been employed, thus producing discordant results. In summary, we provide a review of the currently available echocardiographic parameters for risk prediction in CHDs with a diminutive RV that may serve as a guide for use in clinical practice.

Background/Objectives: While speckle-tracking echocardiography (STE) is increasingly gaining acceptance in the medical community, establishing normal pediatric values and interpreting data derived from software provided by various vendors can pose significant challenges. This review aims to present an updated compilation of nomograms pertinent to speckle-tracking echocardiography. Methods: A review of research using three medical engine searches (National Library of Medicine, Science Direct, and Cochrane Library) for Medical Subject Headings (MeSH) and the free text terms “echocardiography”, “STE”, “normal values”, and ”children” was performed and refined by adding the keywords “nomograms”, “z-scores”, and “healthy children”. Results: A total of twenty-five studies were selected for the final analysis. Our research indicated that current nomograms provide adequate coverage of most strain parameters; however, those pertaining to the right ventricle and the atria are less numerous than those for the left ventricle. A noted trend suggests a decrease in strain values with advancing age and increasing body surface area; nevertheless, the relationships observed were weak and nonlinear. The absence of robust correlations between strain values and age and body size parameters hindered the generation of a Z-score possessing sufficient statistical power. Consequently, normal values are primarily represented as mean values accompanied by standard deviation. A comparative analysis of vendors demonstrated good agreement between different versions of the same platform for Philips (except for QLAB 5) and, similarly, between General Electric (GE) and TomTec. The limited data available regarding the comparison between GE and Philips revealed significant findings that warrant further investigation of differences. Conclusions: A comprehensive review and an updated list of current pediatric nomograms for STE measurements have been presented. This may serve as a valuable guide for accurately interpreting STE in pediatric patients with congenital and acquired heart disease.