Normal Values for Echocardiographic Myocardial Work in a Large Pediatric Population.

BACKGROUND: Echocardiographic myocardial work is a new load-independent echocardiographic technique to quantify left ventricle (LV) systolic performance. Our aim was to establish normal values for echocardiographic myocardial work in a large population of healthy children. METHODS: For all the subjects 4-, 2-, and 3-chamber-view videos were stored. The following parameters were obtained by offline analysis: the global myocardial work (GMW), the global myocardial constructive work (GCW), the global myocardial wasted work (GWW), and the global myocardial work efficiency (GWE). Age, weight, height, heart rate, and body surface area (BSA) were used as independent variables in the statistical analysis. RESULTS: In all, 516 healthy subjects (age range, 1 day-18 years; median age, 8.2 ± 5.3 years; 55.8% male; body surface area (BSA) range, 0.16 to 2.12 m2) were included. GWI, GCW, and GWW increased with weight, height, and BSA (ρ ranging from 0.635 to 0.226, p all < 0.01); GWI and GCW positively correlated with age (ρ 0.653 and 0.507). After adjusting for BSA differences, females showed higher mean GWI (p = 0.002) and GCW values (p < 0.001), thus Z-score equations for gender have been presented. CONCLUSIONS: We provided MW values in a large population of healthy pediatric subjects including lower ages. MW values increased with age and body size and, interestingly, were higher in females than in men. These data cover a gap in current nomograms and may serve as a baseline for the evaluation of MW analysis in children with congenital and acquired heart diseases.

Comprehensive Two-Dimensional Pediatric Echocardiographic Nomograms for Coronary Artery Sizes in Caucasian Children and Comparison among Major Nomograms.

BACKGROUND: Although coronary artery nomograms in children have been published, data on Caucasian children are lacking. The aim of this study is to provide: (i) a full dataset of coronary artery diameters in healthy children and (ii) a comparison among major previous nomograms. MATERIALS AND METHODS: We prospectively evaluated 606 healthy subjects (age range, 1 days-<18 years; median age 8.7 years; 62.5% male). Coronary artery measurements in a short-axis view were performed. Age, heart rate, and body surface area (BSA) were used as independent variables in different analyses to predict the mean values of each measurement. To assess the accuracy of the predictive models of different studies, a Z-score calculator was created using Lopez's nomograms for comparison. RESULTS: The association with BSA was found to be stronger, and was used for normalization of our data. The best-fit models, satisfying the assumption of homoscedasticity and normality of residuals and showing the highest R2 scores, were logarithmic (ln[y] = a + b*ln[x]). Predicted values and Z-score boundaries by BSA are provided. Our ranges of normality are slightly lower than those, diverging from -0.22 to -0.59 Z-scores for the left main coronary artery and from -0.23 to -0.3 Z-scores for the right coronary artery. CONCLUSIONS: We report a complete dataset of normal echocardiography coronary artery diameter (including new measures of the proximal origin) values in a large population of healthy children. Our data were statistically like those of north American nomograms.

Neonates and Infants with Left Heart Obstruction and Borderline Left Ventricle Undergoing Biventricular Repair: What Do We Know about Long-Term Outcomes? A Critical Review.

BACKGROUND: The decision to perform biventricular repair (BVR) in neonates and infants presenting with either single or multiple left ventricle outflow obstructions (LVOTOs) and a borderline left ventricle (BLV) is subject to extensive discussion, and limited information is known regarding the long-term outcomes. As a result, the objective of this study is to critically assess and summarize the available data regarding the prognosis of neonates and infants with LVOTO and BLV who underwent BVR. METHODS: In February 2023, we conducted a review study with three different medical search engines (the National Library of Medicine, Science Direct, and Cochrane Library) for Medical Subject Headings and free text terms including "congenital heart disease", "outcome", and "borderline left ventricle". The search was refined by adding keywords for "Shone's complex", "complex LVOT obstruction", "hypoplastic left heart syndrome/complex", and "critical aortic stenosis". RESULTS: Out of a total of 51 studies, 15 studies were included in the final analysis. The authors utilized heterogeneous definitions to characterize BLV, resulting in considerable variation in inclusion criteria among studies. Three distinct categories of studies were identified, encompassing those specifically designed to evaluate BLV, those focused on Shone's complex, and finally those on aortic stenosis. Despite the challenges associated with comparing data originating from slightly different cardiac defects and from different eras, our results indicate a favorable survival rate and clinical outcome following BVR. However, the incidence of reintervention remains high, and concerns persist regarding residual pulmonary hypertension, which has been inadequately investigated. CONCLUSIONS: The available data concerning neonates and infants with LVOTO and BLV who undergo BVR are inadequate and fragmented. Consequently, large-scale studies are necessary to fully ascertain the long-term outcome of these complex defects.

Mid-Term Electrical Remodeling after Percutaneous Atrial Septal Defect Closure with GCO Device in a Pediatric Population.

BACKGROUND AND AIM: The GORE® CARDIOFORM (GCO) septal occluder is an atrial septal defect/patent foramen ovale closure device with theoretical advantages over other commercialized devices thanks to its softness and anatomical compliance. Our aim was to evaluate the short- and medium-term electrocardiographic changes after percutaneous ASD closure with GCO in a pediatric population. METHODS: We enrolled 39 patients with isolated ASD submitted to trans-catheter closure from January 2020 to June 2021. ECG was performed before, at 24 h and 6 months after the procedure. P wave dispersion, QTc and QTc dispersion were calculated. ECG Holter was recorded at 6 months after implantation. RESULTS: Patients' age and body surface area (BSA) were 8.2 ± 4.2 years and 1.0 ± 0.3 m2 respectively. At the baseline, mean P wave dispersion was 40 ± 15 msec and decreased at 24 h (p < 0.002), without any further change at 6 months. At 24 h, PR conduction and QTc dispersion significantly improved (p = 0.018 and p < 0.02 respectively), while the absolute QTc value considerably improved after 6 months. During mid-term follow-up, QTc dispersion remained stable without a significant change in PR conduction. The baseline cardiac frequency was 88.6 ± 12.6 bpm, followed by a slight reduction at 24 h, with a further amelioration at 6 months after the procedure (87.3 ± 14.2, p = 0.9 and 81.0 ± 12.7, p = 0.009, respectively). After device deployment, two patients developed transient, self-limited junctional rhythm. One of them needed a short course of Flecainide for atrial ectopic tachycardia. No tachy/brady-arrhythmias were recorded at the 6-month follow-up. ASD closure resulted in a marked decrease in right heart volumes and diameters at 6 months after percutaneous closure. CONCLUSIONS: Percutaneous ASD closure with the GCO device results in significant, sudden improvement of intra-atrial, atrio-ventricular and intraventricular electrical homogeneity. This benefit persists unaltered over a medium-term follow-up. These electrical changes are associated with a documented positive right heart volumetric remodeling at mid-term follow-up.

[Arrhythmias in adult congenital heart disease at the emergency department: ANMCO Tuscany clinical pathway]. / I clinical pathways dell'ANMCO Toscana: la gestione in urgenza delle aritmie nel cardiopatico congenito adulto.

Arrhythmias are a common complication in the adult population with congenital heart disease (ACHD). Arrhythmias often lead to hemodynamic instability and, on the other hand, may be a marker of hemodynamic impairment in ACHD patients, both in natural history and after cardiac surgery. Treatment requires knowledge of basic anatomy and any previous cardiac surgery; the availability of patient's health records, if possible, is therefore crucial for therapeutic choices. In the emergency setting, the first target is represented by the patient's hemodynamic stabilization; mainly in moderate or high complexity ACHD, the connection with the referral center is recommended, to which patients should be entrusted for follow-up. A regional epidemiological observatory, aiming to assess the number, type and outcomes of emergency admissions of ACHD patients could be a useful tool for analyzing the effectiveness of the collaboration network between the different structures involved and for implementing organizational pathways.

Rescue atrial septal defect closure with the new GORE® cardioform atrial septal defect occluder.

Atrioventricular block (AVB) is an infrequent but life-threatening complication of transcatheter closure of atrial septal defect (ASD), accounting for 0.1%-6.2% of cases in large series. It has been related to unfavorable defect anatomy as well as size and intrinsic stiffness of the occluding device. In this setting, the new GORE® cardioform ASD occluder (GCA) device could be an appealing technical advance in ASD treatment. We report a case of complete AVB after ASD closure with an Amplatzer septal occluding (Abbott, Plymouth MN, USA) device successfully treated by its percutaneous retrieval and "rescue" deployment of GCA device few months later.

Screening of Coronary Artery Origin by Echocardiography: Definition of Normal (and Abnormal) Take-Off by Standard Echocardiographic Views in a Healthy Pediatric Population.

Background: Echocardiographic screening of anomalous coronary artery origin is of increasing interest for children participating in sport activities. However, criteria to define normal coronary artery origins in children are poorly defined. Thus, the aim of the present investigation is to define the normal origin and angle of emergence of coronary arteries by echocardiography in healthy children. Materials and methods: The distances of the left main and right coronary artery (LMCA, RCA) origins from the aortic annulus were measured in the parasternal long-axis view (LAX). The angle of coronary artery emergence was measured in the parasternal short-axis view (SAX). Results: A total of 700 healthy subjects (mean age: 9.53 ± 5.95 years; range: 1 day−17.98 years) were prospectively enrolled. The distance of the RCA and LMCA from the aortic annulus correlated with body surface area, and nomograms (Z-scores) were generated. The RCA origin was below the sinotubular junction (STJ) in 605 patients (86.43%), at the STJ in 66 patients (9.43%), and above the STJ in 29 patients (4.14%). The LMCA origin was below the STJ in 671 patients (95.86%), at the STJ in 12 patients (1.71%), and above the STJ in 17 patients (2.43%). With respect to the RCA, an emergence angle < 18.5° in the SAX predicted a high take-off. with a sensitivity of 98.3% and a specificity of 93.1% (AUC 0.998). With respect to the LMCA, an emergence angle > 119.5° in the SAX predicted a high take-off, with a sensitivity of 70.6% and a specificity of 82.4% (AUC 0.799). Conclusion: This study establishes nomograms for LMCA and RCA origin in standard echocardiographic projections in healthy children.

Lung Ultrasound Findings in Healthy Children and in Those Who Had Recent, Not Severe COVID-19 Infection.

Background: Lung ultrasound (LUS) is gaining consensus as a non-invasive diagnostic imaging method for the evaluation of pulmonary disease in children. Aim: To clarify what type of artifacts (e.g., B-lines, pleural irregularity) can be defined normal LUS findings in children and to evaluate the differences in children who did not experience COVID-19 and in those with recent, not severe, previous COVID-19. Methods: LUS was performed according to standardized protocols. Different patterns of normality were defined: pattern 1: no plural irregularity and no B-lines; pattern 2: only mild basal posterior plural irregularity and no B-lines; pattern 3: mild posterior basal/para-spine/apical pleural irregularity and no B-lines; pattern 4: like pattern 3 plus rare B-lines; pattern 5: mild, diffuse short subpleural vertical artifacts and rare B-lines; pattern 6: mild, diffuse short subpleural vertical artifacts and limited B-lines; pattern 7: like pattern 6 plus minimal subpleural atelectasis. Coalescent B-lines, consolidations, or effusion were considered pathological. Results: Overall, 459 healthy children were prospectively recruited (mean age 10.564 ± 3.839 years). Children were divided into two groups: group 1 (n = 336), those who had not had COVID-19 infection, and group 2 (n = 123), those who experienced COVID-19 infection. Children with previous COVID-19 had higher values of LUS score than those who had not (p = 0.0002). Children with asymptomatic COVID-19 had similar LUS score as those who did not have infections (p > 0.05), while those who had symptoms showed higher LUS score than those who had not shown symptoms (p = 0.0228). Conclusions: We report the pattern of normality for LUS examination in children. We also showed that otherwise healthy children who recovered from COVID-19 and even those who were mildly symptomatic had more "physiological" artifacts at LUS examinations.

Left Ventricular Systolic Impairment after Pediatric Cardiac Surgery Assessed by STE Analysis.

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.

Neo-aortic posterior sinus of Valsalva reduction and closed coronary artery transfer as a method to approach aortopulmonary mismatch in transposition of great arteries.

OBJECTIVE: In transposition of great arteries (TGA), aortopulmonary mismatch (APM) can determine postoperative neo-aortic insufficiency after arterial switch operation (ASO). The distortion of sinu-tubular junction may be the geometric mechanism responsible. We developed a strategy able to reduce the mismatch at the timing of ASO, and in this study, we aimed to describe our indications and results. METHODS: Preoperative root circumferences at the level of the mid-portion of sinus of Valsalva and ascending aorta circumference were used to define APM. Indication to surgery was a neo-aortic root (NAR) to ascending aorta ratio ≥ 1.4. Along with standard ASO, posterior neo-aortic sinus inverted conal resection and punch technique for coronary reimplantation was used in all patients to re-establish the more geometric ratio possible between the two components. Hypoplastic aortic arch (HAA) and aortic coarctation (CA) were managed by aortic arch enlargement with an autologous pericardial patch. RESULTS: Twenty patients (20 male), 19 with diagnoses TGA (17 with ventricular septal defect, 85%) and 1 with Taussig-Bing anomaly underwent ASO. HAA was present in three (15%) and CA in two (10%). The mean preoperative neo-aortic to ascending aorta ratio was 1.8 versus 1.1 postoperatively (p < .01). No moderate or severe neo-aortic insufficiency was observed before discharge and at a mean follow-up of 4.3 years (interquartile range = 0.5-12 years). CONCLUSION: Neo-aortic reduction plasty with coronary reimplantation by punch technique is an effective strategy to approach preoperative APM in TGA. This technique confers a more harmonious geometry to NAR that can improve neo-aortic valve function.

A statistical comparison of reproducibility in current pediatric two-dimensional echocardiographic nomograms.

BACKGROUND: The aim of this study is to compare new pediatric nomograms for clinical parameters from 2D echocardiography. METHODS: 2D pediatric echocardiographic parameters from four recent nomograms were used for statistical analysis. To assess the accuracy of the predictive models from each study, namely multivariate, linear, and nonlinear regression, mean values and 5th and 95th percentiles (µ ± 1.65σ) were calculated. A Z-score calculator was created. RESULTS: Mean values and 5th and 95th percentiles have been provided for a range of BSA (0.15-2.20 m2) for each nomogram assessed in this study. Moreover, plots of Z-scores over the same range of BSA have been generated to assess trends among different studies. For most measurements from the two most recent nomograms, namely Lopez et al. and Cantinotti et al., differences were within a Z-score of 0.5 (Z-score range: 0.001-1.26). Measurements from Sluysmans and Colan and Pettersen et al. were observed to diverge from Lopez et al. at the upper extremities of BSA. Differences among various nomograms emerged at lower extremes of BSA. CONCLUSIONS: The two most recent echocardiographic nomograms were observed to have the most statistically similar ranges of normality. Significant deviations in ranges of normality were observed at extremes of BSA. IMPACT: Echocardiographic nomograms for pediatric age are discordant. Comparison of current pediatric echocardiographic nomograms. A Z-score calculator was created. Clinical relevance of differences among nomograms is highlighted.

Prognostic Value of a New Lung Ultrasound Score to Predict Intensive Care Unit Stay in Pediatric Cardiac Surgery.

BACKGROUND: Lung ultrasound (LUS) in pediatric cardiac surgery is gaining consensus. We (1) evaluated the prognostic value of a new LUS-score in pediatric cardiac surgery, and (2) compared LUS-score to conventional risk factors including age, The Society of Thoracic Surgeons/European Association of Cardio-Thoracic Surgery (STAT) score, cardiopulmonary bypass time, and prognostic biomarkers including brain natriuretic peptide and cystatin-C. METHODS: LUS examinations were performed in 237 children (median age, 0.55 years; interquartile range, 0.09-4.15 years) at 12 to 36 hours after surgery. For each hemithorax, 3 areas (anterior/lateral/posterior) were evaluated in the upper and lower halves, constituting 12 total scanning areas. For each site a score was assigned: 0 (rare B lines), 1 (separated B lines), 2 (coalescent B lines), 3 (loss of aeration), and total LUS score was calculated as sum of all sites. The primary endpoints were intensive care unit length of stay and extubation time. RESULTS: The mean total LUS score was 12.88 ± 6.41 (range, 0-26) and was higher in newborns (16.77 ± 5.25) compared with older children (5.36 ± 5.57; P < .001). On univariate analysis, LUS score was associated inversely with age (beta 0.26; P = .004) and body surface area (beta 3.41 P = .006) and positively with brain natriuretic peptide (beta 1.65; P < .001) and cystatin-C (beta 2.41; P < .001). The LUS score, when added as continuous predictor to a conventional risk model (age, STAT score, and cardiopulmonary bypass time) emerged significant both for intensive care unit length of stay (beta 0.145, P = .047) and extubation time (beta 1.644; P = .024). When single quadrants were analyzed, only anterior LUS score was significant (intensive care unit length of stay beta, 0.471; P = .020; extubation time beta 5.530; P = .007). CONCLUSIONS: Our data show the prognostic incremental value of a new LUS score over traditional risk factors in pediatric cardiac surgery.

Retrosternal Clots After Fontan Surgery by Systematic Evaluation With Transthoracic Ultrasound.

OBJECTIVES: Bleeding is a common, serious, and often subtle complication after total cavopulmonary connection surgery. The aim of the present study was to assess the incidence of retrosternal clots after surgery, which were searched for systematically with transthoracic ultrasound. DESIGN: Retrospective study. SETTING: Single center. PARTICIPANTS: Total cavopulmonary surgeries were reviewed from January 2016 to May 2019. INTERVENTIONS: Thoracic ultrasound with careful evaluation of the retrosternal area was performed at different postoperative times (12-36 hours, 5-7 days, and before discharge) as completion of routine echocardiography. MEASUREMENTS AND MAIN RESULTS: Among 37 children undergoing total cavopulmonary connection (mean age 5.5 ± 1.8 years [range 2.4-11.7]; mean body surface area 0.7 ± 0.1 m2 [range 0.3-1.6 m2]), retrosternal clots were detected in 18 (48.6%). Of these, 7 (13.5%) had small clots (<1 cm), 2 (5.4%) small to moderate sized clots (>1 cm-<2 cm), 3 (8.1%) moderate sized clots (>2-<3 cm), and 6 (16.2%) large clots (>3 cm). Four of the 6 detected large clots required surgical revision, and in the other 2 patients, the clots were not treated because the patients' conditions were clinically stable. When 3 major groups (group 1-no or small clots, group gropu 2 are small to moderate or moderate, group 3-large clots) were evaluated, no significant differences were noted in age, body surface area, CPB time, conduit type, or the number of previous surgeries. CONCLUSIONS: With thoracic ultrasound diagnosis, existence of retrosternal clots was found to be very common after total cavopulmonary connection. Most clots were small or moderate with no clinical effect; however, large clots that required redo surgery also were detected.

Normative Data for Left and Right Ventricular Systolic Strain in Healthy Caucasian Italian Children by Two-Dimensional Speckle-Tracking Echocardiography.

BACKGROUND: There is an increasing interest in echocardiographic strain (ε) measurements for the assessment of ventricular myocardial function in children; however, pediatric nomograms remain limited. Our aim was to establish pediatric nomograms for the left ventricular (LV) and the right ventricular (RV) ε measured by two-dimensional speckle-tracking echocardiography (2D-STE) in a large cohort of healthy children prospectively enrolled. METHODS: Echocardiographic measurements included STE LV longitudinal and circumferential and RV longitudinal global end-systolic ε. Age, weight, height, heart rate (HR), and body surface area (BSA) were used as independent variables in different analyses to predict the mean values of each measurement. Echocardiograms were performed by Philips-iE33 systems (Philips, Bothell, WA) and offline measurements on Philips-Q-Lab-9. RESULTS: In all, 721 subjects (age 31 days to 17 years; 48% female) were studied. Low coefficients of determination (R2) were noted among all of the ε parameters evaluated and adjusted for age, weight, height, BSA, and HR (i.e., R2 all ≤ 0.10; range, 0.01-0.088). This hampered the possibility of performing z-scores with a sufficient reliability. Thus, we are limited to presenting data as mean values (±SD) stratified for age groups and divided by gender. LV longitudinal ε values decreased with age (P < .001), while no significant age-related variations were noted for RV longitudinal ε. A significant base-to-apex (lowest to highest) gradient in circumferential LV ε values was noted at all ages (P < .001). CONCLUSIONS: We report pediatric echocardiographic normative data for 2D-STE for the LV and RV ε by using vendor-specific software. Our results confirm previous observations, showing only little variations of strain parameters with age and gender.

Electrocardiograms of Children and Adolescents Practicing Non-competitive Sports: Normal Limits and Abnormal Findings in a Large European Cohort Evaluated by Telecardiology.

OBJECTIVE: The objective of this study was to derive normal electrocardiographic values and to report the abnormal findings in a large contemporary European cohort of physically active children and young adolescents. METHODS: In a 3-month period, data derived from subjects aged between 3 and 14 years and referred to the Telecardiology Centre (Genoa, Italy) for electrocardiogram (ECG) evaluation as pre-participation screening for non-competitive sports were analyzed. RESULTS: A total of 2060 ECGs were recorded. Of those, 1962 did not show any morphological abnormality and were used to derive normality ranges for heart rate, PR interval, QRS duration, corrected QT interval, and voltage of R wave as measured in V1 according to age and sex. Findings and clinical implications of the 98 ECGs with abnormal findings were also reported. Abnormal ECG findings were not as uncommon as expected in this population, being manifest in about 5 % of subjects. However, major ECG anomalies (diffuse negative T-waves, pre-excitation) were present in just ten subjects (0.5 %). Lower mean heart rate values (from 90-100 bpm at 3 years of age to 80-85 bpm at 14 years of age) and lower rates of the prevalence of negative T-waves in the V3 lead (from 55-60 % at 3 years of age to 8-10 % at 14 years of age) were observed with increasing age. CONCLUSIONS: This is the first work reporting derived normal limits and abnormal ECG findings in a large contemporary European cohort of children and adolescents aged 3-14 years practicing non-competitive sports. Clear pathological alterations are extremely uncommon, deserving, when encountered, additional examinations. Even in a physically active population, the common features of an adult athlete's ECG are absent.