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Aims In congenitally corrected transposition of the great arteries (CCTGA) the right ventricle (RV) is systemic. Atrioventricular block (AVB) and systolic dysfunction are frequently observed. Permanent pacing of the subpulmonary left ventricle (LV) may worsen RV dysfunction. The aim of this study was to seek out if LV conduction system pacing (LVCSP) guided by three-dimensional-electroanatomic mapping systems (3D-EAMs) can preserve RV systolic function in paediatric CCTGA patients with AVB. Methods and results Retrospective analysis of CCTGA patients who underwent 3D-EAM-guided LVCSP. Three-dimensional-pacing map guided lead implantation towards septal sites with narrower paced QRS. Electrocardiograms (ECGs), echocardiograms, and lead parameters (threshold, sensing, and impedance) were compared at baseline (pre-implantation) and at 1-year follow-up. Right ventricle function was evaluated by 3D ejection fraction (EF), fractional area change (FAC), RV global longitudinal strain (GLS). Data are reported as median (25th–75th centiles). Seven CCTGA patients aged 15 (9–17) years, with complete/advanced AVB (4 with prior epicardial pacing), underwent 3D-guided LVCSP (5 DDD, 2 VVIR). Baseline echocardiographic parameters were impaired in most patients. No acute/chronic complications occurred. Ventricular pacing was >90%. At 1-year follow-up QRS duration showed no significant changes compared with baseline; however, QRS duration shortened in comparison with prior epicardial pacing. Lead parameters remained acceptable despite ventricular threshold increased. Systemic RV function was preserved: FAC and GLS improved significantly, and all patients showed normal RV EF (>45%). Conclusion Three-dimensional-EAM-guided LVCSP preserved RV systolic function in paediatric patients with CCTGA and AVB after short-term follow-up.

Background Pacemakers (PMKs) and implantable cardioverter-defibrillators (ICDs) are frequently utilized in patients with specific cardiac rhythm disorders. The downfalls of these devices are several but no data exist regarding leads thrombosis. Aim of the study the analysis of pacing lead thrombosis occurrence in paediatric PMK-ICD patients. Methods single Centre retrospective study of a series of paediatric/young adult patients showing pacing lead thrombosis. Data are reported as median (interquartile range). Results from 2015 to 2021 five patients (2 males), aged 16 (12-22) years, showed transthoracic echocardiographic evidence of lead vegetations during routine follow-up after 5 (1-16) years from lead's initial implantation. Age at device implantation was 9 (5-12) years. Two patients had dual-chamber ICD, 3 had PMKs (2 DDD, 1 VVI). No patient had any early wound complications after implantation. At the time of thrombosis detection, no patient reported any recent fever or symptoms of clinical illness. Devices and leads were normally functioning in all patients. Blood examination revealed absence of infectious or inflammatory signs, and all patients had negative hemocultures for aerobic, anaerobic bacteria and fungi. Therefore, lead thrombosis was diagnosed. Transesophageal echocardiogram (TEE) confirmed the echo finding. Thrombophilia testing revealed that 4 out of 5 patients had methylenetetrahydrofolate reductase (MTHFR) mutation, homozygous and heterozygous (2 each). Patients received oral anticoagulation therapy (OAT) and TEE showed complete recovery in 4 patients after 6-12 months; one adult patient is followed-up elsewhere. Conclusions pacing lead thrombosis is a rare complication in paediatric/young adult patients with PMK/ICD, well treated with OAT. MTHFR mutation is a risk factor for this complication.

A multidisciplinary study group involving physicians and jurists was established to review and approve an informed consent about the most frequent interventional procedures for congenital heart diseases.The authors worked together with representatives of the Italian Society of Pediatric Cardiology and Congenital Heart Disease (SICP) Council and Jurist's expert in the field of health case-law. The final draft was shared with the major Italian centers involved in congenital interventional procedures and with AICCA, the Italian Patients Association of Congenital Heart Diseases - Adults and Children.At the end of this review process, a final informed consent form was developed for the most frequent procedures performed in our catheterization laboratories. All of them consist of two parts: a general statement and a procedure-related one.The work performed by this multidisciplinary study group, under the supervision of the SICP, resulted in a new dedicated informed consent about interventional procedures in the field of congenital cardiology, taking into account the new legal requests. This informed consent is intended to be both a document that can be used as such and a document from which to derive a specific document for each center. We believe that using similar informed consents in all Congenital Heart Disease Centers or at least have informed consents all inspired by the same setting, could be a further improvement in taking care of the patients and their families.

The aim of the study was to evaluate the medium-term results of aortic valve neocuspidalization according to Ozaki compared to Ross procedure for treatment of isolated aortic valve disease in pediatric age. Thirty-eight consecutive patients with congenital or acquired aortic valve disease underwent either Ozaki (n = 22) or Ross (n = 16) operation between 01/2015 and 05/2020. The primary outcome was progression of aortic valve disease and aortic ring and root dimension, whereas secondary outcome was freedom from reintervention or death by type of operation. Median age was 12.4 (8.8–15.8) years and the prevailing lesion was stenosis in 20 cases (52%) and incompetence in 18 (48%). One death occurred in the Ross group in the early postoperative period, while there were no deaths in the Ozaki group. Effective treatment of aortic valve stenosis or regurgitation occurred in both groups and remained stable over a median follow-up of 18.2 (5–32) months. In Ozaki group, 3 patients required aortic valve replacement at 4.9, 3.5, and 33 months, respectively. In Ross group, 1 patient required Melody pulmonary valve replacement, whereas none required aortic valve surgery. Finally, significantly higher aortic transvalvular gradient at follow-up was recorded in Ozaki group compared to Ross group. Overall, there was no significant difference in freedom from reoperation or death between the two groups. The medium-term outcome of Ozaki and Ross in pediatric patients is similar, despite an increased tendency of the former to develop aortic transvalvular gradient in the follow-up. Future larger multicenter studies with longer follow-up are warranted to confirm these results.

OBJECTIVES There is growing interest in the aortic valve (AV) neocuspidalization technique for the treatment of aortic valve disease (AVD). We report our medium-term results with this procedure performed in a paediatric patient population. METHODS Between July 2016 and May 2020, 22 patients with both congenital and acquired isolated AVD were treated with neocuspidalization. The primary outcome was progression of the preoperatively assessed AVD in the immediate postoperative course and at follow-up. Secondary outcome was freedom from reintervention by material used. Potential predictors of failure were analysed in relation to the primary outcome. RESULTS The median age at operation was 13.9 (interquartile range, 9.8–16.2) years, and the prevailing AV defect was stenosis in 10 cases (45%) and incompetence in 12 (55%). Pre-treated autologous pericardium was used in 13 patients whereas bovine pericardium in 9. Effective treatment of AV stenosis or regurgitation was achieved and remained stable over a median follow-up of 11.3 (4.7–21) months. Three patients required AV replacement at 4.9, 3.5 and 33 months. At follow-up, an upward trend of both median indexed vena contracta jet widths and aortic peak and mean gradients were recorded, the latter associated with a failure to grow the aortic annulus. Predictor of such outcome turned out to be the use of bovine pericardium. A significant inverse linear correlation between AV peak gradient at follow-up and preoperative aortic annular size (P = 0.008) was also demonstrated. CONCLUSIONS The Ozaki procedure is safe and effective in paediatric patients with AV disease. The use of heterologous pericardium should probably be minimized. Moreover, preoperative small aortic annuli should probably be promptly treated by means of an associated ring enlargement procedure.

The worldwide response to the current COVID-19 pandemic has been focused on how to prevent the disease and to protect the high-risk patient from a potentially lethal infection. Several consensus and guidelines articles have been published dealing with the cardiac patient with systemic hypertension, heart transplant or heart failure. Very little is known about the patients, both in the pediatric as well as in the adult age, with congenital heart disease. The peculiar physiology of the heart with a native, repaired or palliated congenital heart defect deserves a specialized care. Hereby we describe the early recommendations issued by the Italian Society of Pediatric Cardiology and Congenital Heart Disease and how the network of the congenital cardiac institutions in Italy reacted to the threat of potential wide spread of the infection among this fragile kind of patient.

Crossed pulmonary arteries (CPAs) represent an uncommon anatomic variant, usually associated with some specific syndromes and conotruncal defects. This finding has been described in 22q11.2 Deletion Syndrome (22q11.2DS). We evaluated the correlation between CPAs and genetic diseases, in order to better define the characteristics of this variant, considered a rare anatomic pattern. An in-depth analysis of CPAs genotype-phenotype correlations was performed via a literature review. We detected 74 CPAs patients through echocardiography. Of these 74 patients, 35.1% of patients showed additional intracardiac malformations, while 29.7% showed extracardiac vascular anomalies, of which 16.2% were associated with intracardiac defects and 13.5% were not. In all, 62.2% of patients were diagnosed with genetic diseases and 52.2% of them were 22q11.2DS patients. In conclusions, CPAs represent a cardiovascular variant, which is detectable in nonsyndromic individuals, but especially in various genetic syndromes and in particular in 22q11.2DS patients. Data on the real prevalence of this morphology is lacking in literature. Knowledge of this anatomic variant is useful to interpret the unusual course of the pulmonary branches and is helpful information before cardiovascular surgical correction. Moreover, due to the strong association of CPAs with some genetic syndromes, the identification of this anatomic pattern can indicate the utility of a genetic assessment of these patients.

Background We evaluated two-dimensional speckle-tracking echocardiography longitudinal strain (L2DSE) in functionally single left ventricles (LV). Methods We retrospectively analyzed 21 patients with functionally single LV. We divided patients into two groups according to pre-Fontan cardiac catheterization data: group 1, adequate data for Fontan procedure and group 2, inadequate data. Results LV strain correlated with predicted pressure in the Fontan system (r = 0.64; P = 0.003), pressure in the Glenn system (r = 0.57; P = 0.010), and transpulmonary gradient (r = 0.59; P = 0.008), but not with left atrial pressure (r = 0.292; P = 0.226) or ejection fraction (r = 0.254; P = 0.294). In multiple regression analysis, four-chamber LV strain was correlated with predicted pressure in the Fontan (β = 0.642: P = 0.003), whereas no association was found with LV ejection fraction (β = 0.254; P = 0.294), or time from Glenn palliation (β = 0.082; P = 0.893). When dividing the population into two groups according to catheterization data, significantly lower four-chamber longitudinal 2DSE (-25.8 ± 3.2 vs.-19.5 ± 5.1; P = 0.004) was found to be not suitable for the Fontan procedure, as compared with those who underwent successful Fontan completion. Conclusion Regardless of ejection fraction, four-chamber L2DSE is associated with high predicted pressure in the Fontan system. Patients with hemodynamic data unsuitable for Fontan operation have significantly lower mean longitudinal strain parameters. 2DSE may represent a valuable tool in assessing patients with single LV physiology and might provide useful pre-Fontan information.

Evolving reconstructive techniques have progressively become the preferred approach for treatment of pediatric mitral valve regurgitation. We present our experience in a cohort of patients undergoing surgical correction for severe mitral regurgitation. Fifty-five patients (age 1 month–18 years; median 5 years) were included in the present analysis. Different surgical techniques were used (posterior leaflet augmentation in 25, isolated cleft closure in 12, Alfieri-type procedure in 10, annuloplasty in 5, with artificial chordae in 2, and quadrangular resection with chordal transposition in 1). Follow-up time ranged from 1 to 192 months (median 38[IQR 12–54] months). Operative and follow-up mortality was 0%. Reintervention in the whole population occurred in 31% of patients. However, when first surgery was performed under 2 years of age (no = 17), reintervention reached nearly 50%. The degree of residual mitral regurgitation at follow-up remained stable after surgery, while a significant increase in mean transmitral gradient was observed over time (paired t test = 0.03). In multivariable Cox-regression analysis, post-surgical transmitral gradient was the only independent predictor for reintervention (p = 0.017; HR 2.4; 95%CI 1.2–5.1), after correcting for differences in age at surgery, type of reintervention, mitral annulus dimension, and BSA at the first surgery. ROC curve demonstrated that a post-surgical transmitral mean gradient value > 5 mmHg, was predictive for reintervention (AUC = 0.89; Youden index = 0.44). Our study suggests that the use of conservative technique strategy achieves satisfactory functional results in infants and children with severe MR, although the rate of reoperation in younger patients remains substantial. Post-operative moderate mitral stenosis was the strongest predictor for reoperation.

The Coronavirus disease 2019 (COVID-19) pandemic has thoroughly and deeply affected the provision of healthcare services worldwide. In order to limit the in-hospital infections and to redistribute the healthcare professionals, cardiac percutaneous intervention in Pediatric and Adult Congenital Heart Disease (ACHD) patients were limited to urgent or emergency ones. The aim of this article is to describe the impact of the COVID-19 pandemic on Pediatric and ACHD cath laboratory activity during the so-called 'hard lockdown' in Italy. Eleven out of 12 Italian institutions with a dedicated Invasive Cardiology Unit in Congenital Heart Disease actively participated in the survey. The interventional cardiology activity was reduced by more than 50% in 6 out of 11 centers. Adolescent and ACHD patients suffered the highest rate of reduction. There was an evident discrepancy in the management of the hard lockdown, irrespective of the number of COVID-19 positive cases registered, with a higher reduction in Southern Italy compared with the most affected regions (Lombardy, Piedmont, Veneto and Emilia Romagna). Although the pandemic was brilliantly addressed in most cases, we recognize the necessity for planning new, and hopefully homogeneous, strategies in order to be prepared for an upcoming new outbreak.

The aim here was to describe the role of speckle tracking echocardiography (STE), in identifying impairment in systolic function in children and adolescents with focal myocarditis and without reduction in ejection fraction. We describe data from 33 pediatric patients (age 4–17 years) admitted for focal myocarditis, confirmed by cardiac magnetic resonance (CMR), and without impaired ejection fraction and/or wall motion abnormalities. All children underwent Doppler echocardiography examination with analysis of global (G) and segmental longitudinal strain (LS) and CMR for the quantification of edema and myocardial fibrosis. Reduction in LS was defined according to age-specific partition values. At baseline, impaired GLS was present in 58% of patients (n = 19), albeit normal ejection fraction. LS was also regionally impaired, according to the area of higher edema at CMR (i.e. most impaired at the level of the infero-lateral segments as compared to other segments (p p = 0.01). At follow-up, GLS improved in all patients (p

Background: No previous study has defined the prevalence of cardiac geometric and mechanical function abnormalities through the analysis of advanced echocardiographic parameters in children with autosomal recessive polycystic kidney disease (ARPKD). Aim: The purpose of this study was to evaluate cardiac geometry and function through advanced echocardiography in a well-characterized sample of pediatric patients with ARPKD. Methods: Standard echocardiograms were obtained in 27 children with ARPKD (0–18 years) and in 88 healthy children of similar age, gender distribution, and body build. Left ventricular (LV) hypertrophy was defined as LV mass > 45g/(m2.16 + 0.09) and cardiac remodeling was defined by age-adjusted relative wall thickness (RWT). Systolic function was assessed by ejection fraction, midwall fractional shortening (mFS), and global longitudinal (GLS) and circumferential strain (GCS). Results: Patients with ARPKD exhibited a higher LV mass index as compared to controls, and a more concentric LV geometry (both p < 0.001). Accordingly, the prevalence of abnormal LV geometry was significantly higher in ARPKD (33 vs. 0%; p < 0.005). No differences could be observed in the two groups for ejection fraction or GLS (both p = n.s.), while a significantly lower mFS (p < 0.05) as well as GCS (p < 0.001) could be observed. In the analysis of covariance, both LV mass index and RWT remained significantly higher in the ARPKD group, while mFS and GCS remained significantly lower (all p < 0.05). The prevalence of subclinical systolic dysfunction was significantly higher in patients with ARPKD as compared with control subjects (33 vs. 0%; p < 0.001). Conclusions: Children with ARPKD show significantly impaired cardiac phenotype, characterized by high rates of LV abnormal geometry paired with systolic mechanical dysfunction.

Background The purpose of this study was to evaluate the accuracy of the pulmonary flow study in (1) predicting the feasibility of concomitant intracardiac repair after one-stage unifocalization; and in (2) predicting long-term survival and the onset of right ventricular dysfunction after surgery. Methods Between October 1996 and July 2015, a flow study was obtained in 95 patients undergoing complete one-stage unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. The ability to achieve 100% flow (approximately 2.5 L · min −1 · m −2 ) into the pulmonary bed at a mean pressure of 30 mm Hg or less was utilized as an indicator for acceptability of ventricular septal defect closure. Results Overall survival was 78% ± 6% at 15 years. Sixty-four patients underwent successful one-stage intracardiac repair. The flow study accurately predicted suitability for VSD closure (area under the curve = 0.855). After one-stage ventricular septal defect closure, no difference in survival was observed after stratification according to flow study pressures (25 mm Hg or less versus greater than 25 mm Hg, log rank p = 0.20). At a median follow-up of 7 years, no association was found between flow study pressure and the onset of right ventricular dysfunction ( p = 0.21). Overall, the inability to achieve final intracardiac repair was a strong predictor of death (hazard ratio 9.14, 95% confidence interval: 1.98 to 42.07, p Conclusions Suitability for ventricular septal defect closure is reliably defined by the flow study with a cutoff of 30 mm Hg. Flow study pressure values do not affect long-term outcomes. The ability to obtain intracardiac repair (in either one or more stages) is the strongest predictor of survival.

Right ventricular (RV) evaluation represents one of the major clinical tasks in the follow-up of repaired tetralogy of Fallot patients (rToF) with pulmonary valve regurgitation, as both severe RV dilatation and dysfunction are key factors in defining the need of pulmonary valve replacement. The aim of our study was to report the diagnostic accuracy of echocardiography in the identification of rToF patients with severely dilated and/or depressed RV as compared to cardiac magnetic resonance (CMR). Among our patients with rToF, a subgroup of 95 (17.6 ± 6.8 years; 60% male), who underwent right ventricular qualitative and quantitative evaluation with CMR following echocardiographic suspicion of severe dilation/dysfunction, were included in the analysis. When comparing echocardiographic RV functional parameters to CMR findings, we found no association between CMR-ejection fraction (EF) and either tricuspid annulus plane systolic excursion (TAPSe) nor tissue Doppler systolic tricuspid excursion velocity (all p = ns). In contrast RVFAC was strongly associated with CMR-EF (r = 0.44; p

BACKGROUND Strain echocardiography is a reliable echocardiographic modality to measure myocardial mechanical deformation and evaluate regional and global myocardial systolic function, and has been demonstrated to be a useful tool for the evaluation of cardiac function in children with acquired and congenital heart diseases. Recent advancements in echocardiographic technology allow to analyze myocardial strain in multiple layers. To our knowledge, there are no published reference values for echocardiographic multilayer-specific strain measurement. AIM OF THE STUDY Aim of our study was to establish normal reference values for left ventricular (LV) and right ventricular (RV) longitudinal strain (LS) in a wide population of Italian children and to provide normal reference values of layer-specific LS in a healthy pediatric population. METHODS AND RESULT At present we have analyzed data from two-hundred (target: n = 500) healthy, consecutive subjects (age CONCLUSIONS We provide preliminary data on 200 children to establish normal reference values for echocardiographic multilayer-specific strain measurement in a wide population of healthy italian children. Abstract P1542 Figure.

Aim To evaluate the ability of Speckle Tracking Echocardiography (STE), as compared to Cardiac Magnetic Resonance (CMR), in identifying acute and sub-acute abnormalities in systolic function occurring with focal myocarditis in children and adolescents without evident wall motion abnormalities. Methods: We analyzed data from 33 consecutive patients (age 4-17year) with CMR-confirmed focal myocarditis and without regional motion abnormalities and/or reduced ejection fraction. Patients underwent echocardiography with analysis of regional and global longitudinal strain and CMR for the identification of focal edema and myocardial fibrosis. Impaired longitudinal strain was defined according to previously reported age-specific reference values. Results: Despite normal ejection fraction at admission, prevalence of impaired systolic function by STE was present in 58% of patients (n = 19). Reduction in longitudinal strain was regionally related to CMR-identified edema areas, with lowest values found at the level of the infero-lateral segments as compared to the mean of the other segments (p Abstract P828 Figure.

We report a case of a three months old baby, with no history of heart disease, referred to our center for cardiological screening. EKG was normal and a physiologic 1/6 systolic murmur was present. Conversely, echocardiography revealed a thin-walled, echo-free cystic intrapericardic structure adjacent to the posterior wall of the left ventricle; no signs of compression on cardiac structures were evident. CAT scan confirmed the presence of echo-free cystic over-diaphragmatic structure at the crux cordis level. The cyst was described as located between visceral pericardial layer and muscolar ventricular wall and appeared to cause compression on the midbasal wall of the left ventricle, of the interventricular septum and less extensively on the right ventricle. The dimension of the cyst was 2.7x2.5x1.8 cm. Worthy of note, posterior interventricular and posterolateral coronary arteries were very close to the cyst and partially pushed apart one from the other by the mass. Eight months after the diagnosis we detected an increase in mass size, confirmed also by cardiac MRI showing a mass of 4.5x3.4x3 cm with an extimated volume of 23 cm2. Because of significant increase of dimension of the cyst we decided for surgical excision of the mass. The cyst contained clear and translucent fluid and was successfully removed. Pleuropericardial window was created to prevent pericardial effusion. Histopathological report suggested the diagnosis of bronchogenic cyst because of the presence of smooth muscle. Bronchogenic cyst are closed epithelial-lined sacs developed from the respiratory system as the result of an abnormal budding process during the early development of the foregut. These congenital malformations are usually located in the mediastinum or in the lung parenchyma, but atypical locations such as neck, intramedullary part of the spine, diaphragm or intraabdominal region have been reported. Only a few cases occurring in the heart have been described, but their location in the left ventricle is extremely rare. Bronchogenic cyst are usually asymptomatic: symptoms are related to the interaction with the adjacent structures and therefore depend on the dimensions and the localization of the mass. Surgical approach is mandatory if a complication occurs. Conversely there is not a unique approach for asymptomatic mass as in our case. The young age of the baby and the absence of symptoms argued in favor of a "watchful waiting" strategy. Nevertheless, the particular localization of the cyst at the crux cordis level, its relatively rapid growth and above all the evidence of a close relationship with the coronary arteries, suggesting a concrete risk of compression with further enlargement, were determinant for the final decision for surgical excision. Abstract P268 Figure 1

Echocardiographic quantification is crucial for the diagnosis and management of patients with acquired and congenital heart disease (CHD). In neonatal and pediatric age, the echocardiogram begins with subxiphoid, or subcostal, imaging instead of left parasternal views. This allows for the determination of visceral situs (site or location) at the beginning of an examination. Regardless of where the examination starts, the segmental approach is used to describe all of the major cardiovascular structures in sequence. Patent foramen ovale is a normal interatrial communication during fetal life. Complete anatomic closure of the foramen ovale occurs in 70-75% of adults, which means that almost 25% of the population has a patent foramen ovale. Atrial septal defects are a common congenital disorder with a prevalence of approximately 2 per 1000 live births. The reported rate of spontaneous atrial septal defect closure in the first year of life ranges from 4% to 96%. The most important predictor for spontaneous closure is the size of the defect, with smaller defects more likely to close. Systemic-to-pulmonary collateral arteries can occur in premature infants without chronic lung disease and may represent a transient phenomenon. They may be present normally after birth and then gradually disappear. Physiological valvular regurgitation is most commonly observed in the tricuspid valve among children (32.8%), followed by pulmonary regurgitation (17.2%). The ductus arteriosus usually is functionally closed within 48 h of birth, although some authors consider the patent ductus to be abnormal only after 3 months of age. Prematurity clearly increases the incidence of patent ductus arteriosus.

Newer parameters of cardiac mechanics provide additional insights on cardiac dysfunction in adult patients with CKD. The aim of this study was to identify prevalence of subclinical abnormalities in cardiac function through the analysis of novel indices of cardiac mechanics in a large population of children with CKD.Between 2009 and 2011, the prospective observational Cardiovascular Comorbidity in Children with CKD Study enrolled patients with CKD ages 6-17 years old with eGFR=10-45 ml/min per 1.73 m(2) in 14 European countries. Cardiac morphology and function were assessed through echocardiography. The analysis presented encompasses global radial, longitudinal, and circumferential strains as well as time to peak analysis. Data were compared with 61 healthy children with comparable age and sex.Data on 272 patients with CKD with complete echocardiographic assessment are reported (age =12.8±3.5 years old; 65% boys). Patients with CKD showed mildly higher office BP values and higher prevalence of left ventricular hypertrophy, but no differences were observed among groups in left ventricular ejection fraction. Strain analysis showed significantly lower global radial strain (29.6%±13.3% versus 35.5%±8.9%) and circumferential strain components (-21.8%±4.8% versus -28.2%±5.0%; both P0.05) in patients with CKD without significant differences observed in longitudinal strain (-15.9%±3.4% versus -16.2%±3.7%). Lower values of global radial strain were associated with lower circumferential endocardial-to-epicardial gradient (r=0.51; P0.01). This association remained significant after adjusting for BP, eGFR, and presence of left ventricular hypertrophy. Eventually, patients with CKD also showed higher delay in time to peak cardiac contraction (58±28 versus 37±18 milliseconds; P0.05).A significant proportion of children with CKD show impaired systolic mechanics. Impaired systolic function is characterized by lower radial strain, transmural circumferential gradient, and mild cardiac dyssynchrony. This study suggests that analysis of cardiac strain is feasible in a large multicenter study in children with CKD and provides additional information on cardiac pathophysiology of this high-risk population.

Background Patients with an open ventricular septal defect (VSD) after repair of pulmonary atresia (PA), VSD, and major aortopulmonary collaterals (MAPCAs) are the most vulnerable subgroup. We analyzed the impact of concomitant versus delayed VSD closure on survival and intermediate-term right ventricular (RV) function. Methods Between October 1996 and February 2017, 96 patients underwent a pulmonary flow study–aided repair of PA/VSD/MAPCAs. For patients who underwent either concomitant or delayed intracardiac repair, echocardiographic RV systolic function was retrospectively calculated to assess (1) RV fractional area change (RVFAC) and (2) two-dimensional RV longitudinal strain (RVLS) of the free wall of the right ventricle. QLAB cardiac analysis software version 10.3 (Philips Medical Systems, Andover, MA) was used for analysis. Results A total of 64 patients underwent concomitant VSD closure at the time of unifocalization, and 16 patients underwent delayed VSD closure at a median of 2.3 years (range: 3 days to 7.4 years). At a median follow-up of 8.1 years (range: 0.1 to 19.5 years) for the concomitant repair group versus 7.4 years (range: 0.01 to 15.3 years) for the delayed repair group, no differences in RVFAC and RVLS were observed (RVFAC: 41.0% ± 6.2% versus 41.2% ± 7.6%, p = 0.91; RVLS: −18.7 ± 4.3 versus −18.9 ± 4.0, p = 0.87). Conclusions Patients (83%) with PA/VSD/MAPCAs underwent complete repair at intermediate-term follow-up with preserved RV function. Delayed VSD closure was accomplished in 50% of the patients initially deemed unsuitable for repair. Delayed VSD closure did not affect survival and did not portend impaired RV systolic function.

Background: Little is known about the clinical value of late gadolinium enhancement (LGE), in children affected by dilated cardiomyopathy (DCM). Materials and methods: We retrospectively evaluated 15 patients (8 ± 6 years, 6 males) with diagnosis of DCM who underwent cardiac magnetic resonance since 2014. All scans were performed with a 1.5 T system (Aera, Siemens). Study protocol included cine steady-state free precession sequences, followed by administration of 0.2 mmol/kg of gadolinium-based contrast agent. Inversion recovery Turbo Flash sequences, in the same position of cine images, were acquired 10-15 min after the injection of contrast agent, in order to assess the presence of LGE. The latter was considered positive with a signal intensity > 6 SD from normal myocardial tissue. Indexed end-diastolic volume (EDVi) and end-systolic volume (ESVi), and left ventricle (LV) ejection fraction (EF) were calculated by using dedicated software on off-line workstation. Global longitudinal strain and diastolic function were evaluated by echocardiography. Clinical follow-up, including death, transplant, and listing for heart transplant [major adverse cardiac events (MACE)], were evaluated. Patients were divided into two different subgroups: negative (Group A) and positive (Group B) for presence of LGE. Statistical analysis was performed by using Mann-Whitney U test (p < 0.05 considered as statistically significant). results: Seven patients (47%) showed LGE. A global diffuse subendocardial pattern was evident in all patients presenting LGE (7/7, 100%). The following main LV indexes were observed in the two subgroups. Group A: EDVi = 96 ± 33 ml, ESVi = 56 ± 29 ml, LV EF = 45 ± 10%, global longitudinal strain = -16 ± 5%, E/e' ratio = 10 ± 3, MACE = 1. Group B: EDVi = 130 ± 60 ml, ESVi = 89 ± 43 ml, LV EF = 31 ± 6%, global longitudinal strain = -13 ± 4%, E/e' ratio = 9 ± 3, MACE = 3. There was no statistically significant difference between the two groups, in terms of EDVi (p: 0.2), ESVi (p: 0.2), and E/e' ratio (0.9), whereas a significant difference of LV EF, presence of significative mitral regurgitation, and global longitudinal strain were observed (respectively, p: 0.03, p: 0.009, and p: 0.03). conclusion:In our population of children with DCM, LGE shows a global diffuse subendocardial pattern. Presence of LGE seems to play a role in these patients determining a worst global systolic function.

Aim/objectiveSeveral studies have demonstrated a significantly increased risk of specific patterns of congenital anomalies in infants born to diabetic mothers. In particular, caudal dysplasia sequence has been linked to pregnancy complicated by maternal diabetes. In addition, several cases of infants born to diabetic mothers presenting with features of DiGeorge anomaly have been reported. Infants with DiGeorge anomaly can display additional manifestations within the spectrum of caudal dysplasia sequence, including vertebral anomalies and renal agenesis.MethodsWe report a neonate presenting with the co-occurrence of features of both DiGeorge anomaly and caudal dysplasia sequence, born to a mother with poorly controlled insulin-dependent diabetes.ResultsThe patient was affected by truncus arteriosus type A1 and hypertrophic cardiomyopathy.ConclusionMaternal diabetes can cause a spectrum of manifestations, expressing with isolated DiGeorge anomaly or caudal dysplasia sequence, with intermediate phenotypes or with the co-occurrence of both the congenital anomalies in the same patient. The present observations argue for a feasible link between truncus arteriosus with hypertrophic cardiomiopathy, DiGeorge anomaly, and maternal diabetes.

International audience; Computer models of the heart are of increasing interest for clinical applications due to their discriminative and predictive power. However the personalisation step to go from a generic model to a patient-specific one is still a scientific challenge. In particular it is still difficult to quantify the uncertainty on the estimated parameters and predicted values. In this manuscript we present a new pipeline to evaluate the impact of fibre uncertainty on the personalisation of an electromechanical model of the heart from ECG and medical images. We detail how we estimated the variability of the fibre architecture among a given population and how the uncertainty generated by this variability impacts the following personalisation. We first show the variability of the personalised simulations, with respect to the principal variations of the fibres. Then discussed how the variations in this (small) healthy population of fibres impact the parameters of the personalised simulations.

Transplanted heart dysfunction is a serious life-threatening condition in pediatric transplant recipients. Several studies have focused on echocardiographic detection of early signs of cardiac dysfunction in this population. We evaluated advanced echocardiographic indices of cardiac function in a large sample of children and young adults with heart transplants with apparently normal cardiac function.The study included 60 patients with normal ejection fraction with transplantation performed at pediatric age between 1986 and 2014 and 60 healthy control subjects. All patients and control subjects underwent a complete transthoracic echocardiographic examination including tissue Doppler analysis, 2-dimensional speckle tracking, and 3-dimensional echocardiography. Two-dimensional speckle tracking analysis was used to obtain measures of left ventricular (LV) radial, circumferential, and longitudinal strain and to derive LV twist and torsion from basal and apical rotation. Three-dimensional echocardiography was used to measure LV volumes and ejection fraction and to evaluate LV systolic synchrony.No differences were observed between groups in LV volumes, left atrial diameters, LV ejection fraction, or right ventricular fractional area change. However, patients showed lower values of longitudinal systolic excursion of valvular planes at both the mitral and the tricuspid valve level as well as higher mitral E/E' ratio. Cardiac radial strain was similar between groups; a significant net reduction in both global left and right ventricular longitudinal strain and LV global circumferential strain could be observed between the 2 groups (all p0.05). In addition, reduced LV twist and torsion was found in patients compared with healthy control subjects (p0.01) mainly owing to a significant reduction in basal rotation (-2.4° vs -3.8°; p0.05). none of the control subjects demonstrated LV dyssynchrony, whereas systolic dyssynchrony was observed in 20% of heart transplant recipients.Even in the absence of signs of graft failure and in the presence of a normal ejection fraction, transplanted hearts show a significant reduction in biventricular function. Additional prognostic studies are needed to establish whether these abnormalities predict development of overt heart failure.

Objective To determine a simplified method to identify presence of left ventricular hypertrophy (LVH) in pediatric populations because the relationship between heart growth and body growth in children has made indexing difficult for younger ages. Study design Healthy children (n = 400; 52% boys, 0-18 years of age) from 2 different European hospitals were studied to derive a simplified formula. Left ventricular mass (LVM) was calculated according to the Devereux formula. The derived approach to index LVM was tested on a validation cohort of 130 healthy children from a different hospital center. Results There was a strong nonlinear correlation between height and LVM. LVM was best related to height to a power of 2.16 with a correction factor of 0.09. Analysis of residuals for LVM/[(height 2.16 ) + 0.09] showed an homoscedastic distribution in both sexes throughout the entire height range. A partition value of 45 g/m 2.16 was defined as the upper normal limit for LVM index. As opposed to formula suggested by current guidelines (ie, LVM/height 2.7 ) when applying the proposed approach in the validation cohort of 130 healthy participants, no false positives for LVH were found (0% vs 8%; P Conclusions Our data support the possibility to have a single partition (ie, 45 g/m 2.16 ) value across the whole pediatric age range to identify LVH, without the time-consuming need of computing specific percentiles for height and sex.

OBJECTIVESThe goal of this study was to evaluate percutaneous interventional and minimally invasive surgical closure of secundum atrial septal defect (ASD) in children.BACKGROUNDConcern has surrounded abandoning conventional midline sternotomy in favor of the less invasive approaches pursuing a better cosmetic result and a more rational resource utilization.METHODSA retrospective analysis was performed on the patients treated from June 1996 to December 1998.RESULTSOne hundred seventy-one children (median age 5.8 years, median weight 22.1 kg) underwent 52 device implants, 72 minimally invasive surgical operations and 50 conventional sternotomy operations. There were no deaths and no residual left to right shunt in any of the groups. The overall complication rate causing delayed discharge was 12.6% for minimally invasive surgery, 12.0% for midline sternotomy and 3.8% for transcatheter device closure (p < 0.01). The mean hospital stay was 2.8 ± 1.0 days, 6.5 ± 2.1 days and 2.1 ± 0.5 days (p < 0.01); the skin-to-skin time was 196 ± 43 min, 163 ± 46 min and 118 ± 58 min, respectively (p < 0.001). Extracorporeal circulation time was 49.9 ± 10.1 min in the minithoracotomy group versus 37.2 ± 13.8 min in the sternotomy group (p < 0.01) but without differences in aortic cross-clamping time. Sternotomy was the most expensive procedure (15,000 € ± 1,050 € vs. 12,250 € ± 472 € for minithoracotomy and 13,000 € ± 300 Marie for percutaneous devices).CONCLUSIONSWhile equally effective compared with sternotomy, the cosmetic and financial appeal of the percutaneous and minimally invasive approaches must be weighed against their greater exposure to technical pitfalls. Adequate training is needed if a strategy of surgical or percutaneous minimally invasive closure of ASD in children is planned in place of conventional surgery.

Background Granulicatella spp. is a fastidious bacteria responsible for bacteremia and endocarditis which are fatal in about 20% of the cases. These severe infections are uncommon in children under 17 years of age and have proven extremely difficult to treat. Cases presentation We report a brief review of the literature and two cases of NVS bacteremia by Granulicatella complicated by infective endocarditis (IE). The first one is that of a 7-year-old Caucasian female with Shone syndrome and IE involving the pulmonary valve homograft, confirmed by echocardiography. The second case is that of a 5-year-old Caucasian male. In this patient echocardiogram was negative for signs of IE; however, a “possible” IE was suspected on the basis of a cardiac catheterization 3 weeks before the onset of fever. Since in both our patients clinical failure of first line antibiotic treatment was observed, we used a combination of meropenem with another anti-streptococcal drug with excellent results. Conclusion In Granulicatella bacteremia in the pediatric population, combination antimicrobial therapy including meropenem should be considered as a second line treatment in non-responding patients.

BACKGROUND: Infants with cyanotic congenital heart disease palliated with placement of a systemic-to-pulmonary-artery shunt are at risk for shunt thrombosis and death. We investigated whether the addition of clopidogrel to conventional therapy reduces mortality from any cause and morbidity related to the shunt. METHODS: In a multicenter, double-blind, event-driven trial, we randomly assigned infants 92 days of age or younger with cyanotic congenital heart disease and a systemic-to-pulmonary-artery shunt to receive clopidogrel at a dose of 0.2 mg per kilogram of body weight per day (467 infants) or placebo (439 infants), in addition to conventional therapy (including aspirin in 87.9% of infants). The primary efficacy end point was a composite of death or heart transplantation, shunt thrombosis, or performance of a cardiac procedure due to an event considered to be thrombotic in nature before 120 days of age. RESULTS: The rate of the composite primary end point did not differ significantly between the clopidogrel group (19.1%) and the placebo group (20.5%) (absolute risk difference, 1.4 percentage points; relative risk reduction with clopidogrel, 11.1%; 95% confidence interval, -19.2 to 33.6; P=0.43), nor did the rates of the three components of the composite primary end point. There was no significant benefit of clopidogrel treatment in any subgroup, including subgroups defined by shunt type. Clopidogrel recipients and placebo recipients had similar rates of overall bleeding (18.8% and 20.2%, respectively) and severe bleeding (4.1% and 3.4%, respectively). CONCLUSIONS: Clopidogrel therapy in infants with cyanotic congenital heart disease palliated with a systemic-to-pulmonary-artery shunt, most of whom received concomitant aspirin therapy, did not reduce either mortality from any cause or shunt-related morbidity. (Funded by Sanofi-Aventis and Bristol-Myers Squibb; ClinicalTrials.gov number, NCT00396877.).

A 1-day-old term baby has been referred to our unit because of respiratory distress. A transthoracic echocardiography ( Panel A ; see Supplementary data online, Movie S1 ) was performed to rule out a congenital heart disease and showed a large thrombus (red arrow) at the pulmonary site of the patent ductus arteriosus, with no shunt. There was an associated secundum …

Although 2-dimensional, Doppler, color flow echocardiography is accepted as a safe diagnostic method to guide the surgical treatment of certain heart defects, cardiac catheterization remains mandatory for patients with tetralogy of Fallot. Based on the excellent diagnostic correlation between echocardiography and angiocardiography, a policy of echo-guided primary repair of uncomplicated, selected cases of tetralogy of Fallot was introduced at Ospedale Bambino Gesù. In the last 5 years, of 139 patients who had surgery for tetralogy of Fallot, 105 underwent primary repair. In 56 patients (53.3%), surgery was guided only by echocardiography (group I). In the remaining 49 patients (46.7%) (group II), unclear imaging of cardiovascular anatomy (n = 23), or echo-suspected associated malformations (n = 26) prompted cardiac catheterization. The 2 groups did not differ in age and weight at surgery. Intraoperative examination did not show diagnostic errors in patients of group I; cardiac catheterization and surgery confirmed the suspected associated anomalies in 16 of 26 patients of group II (38.4% false-positive). Echocardiography showed an overall sensitivity of 100% and specificity of 85% for detection of associated malformations. In conclusion, echocardiography proved to be adequate for selection of patients with uncomplicated forms of tetralogy of Fallot for primary repair.

A right aortic arch with a left descending aorta and an aberrant left innominate artery is a rare but recognized vascular anomaly that can result in compression of the trachea and the esophagus. This vascular anomaly has been diagnosed using cardiac catheterization and angiography. Recently, computed tomography and magnetic resonance imaging have been used for noninvasive diagnosis. The aim of this report is to highlight the possibility of echocardiographic diagnosis.

AimsWe investigated the prevalence, type, and course of congenital cardiac defects and systemic hypertension in our patients with Williams-Beuren-Beuren syndrome.Methods and resultsWe reviewed the clinical records of all patients with Williams-Beuren syndrome examined between 1981 and 2006. We identified 150 patients, aged from 7 months to 45 years, with a follow-up from 6 months to 25 years, the mean being 6.4 years. A cardiac anomaly was present in 113 of the 150 patients (75%). Defects were typical in over four-fifths of the group. We found supravalvar aortic stenosis in 73 of 113 patients (64.6%), isolated in 43. Pulmonary stenosis, isolated in 18 cases, was found in 51 of 113 (45.1%), while aortic coarctation and mitral valvar prolapse were each found in 7 (6.2%), 3 of the lesions is isolation. Atypical defects were found in 19 patients, tetralogy of Fallot in 2, atrial septal defects in 4, aortic and mitral valvar insufficiencies in 1 each, bicuspid aortic valves in 2, and ventricular septal defects in 9, 4 of the last being isolated. Systemic hypertension, observed in 33 patients (22%), was poorly controlled in 10. Diagnostic and/or interventional cardiac catheterization was undertaken in 24 patients, with 30 surgical procedures performed in 26 patients. Of the group, 3 patients died.ConclusionCardiac defects were present in three-quarters of our patients. Pulmonary arterial lesions generally improved, while supravalvar aortic stenosis often progressed. Atypical cardiac malformations, particularly ventricular septal defects, occurred frequently. Systemic hypertension was found in one-fifth, even in the absence of structural cardiac defects. The short-term mortality was low.

Other authors have demonstrated the ability of three-dimensional (3D) echocardiography to produce “en face” views of anomalies such as atrioventricular valve disease and atrial and ventricular septal defects. Few data exist about the usefulness of 3D images for more complex congenital heart defects and the surgical impact of this relatively new technology. This study, covering a period of 8 months and including 43 young patients affected by complex congenital heart defects, demonstrated that the routine use of 3D echocardiography is feasible and valuable for some types of cardiac defects. In fact, 3D images have provided more detailed anatomic definition of interrelations between structures in about one-third (15/43) of our cases, yielding new insight into the anatomy analogous to what can be derived from examining a heart specimen. Our surgeons found the 3D images particularly helpful for providing a realistic and almost specimen-like preview of the surgical anatomy that facilitates planning of the surgical program.

A 9-day-old neonate weighing 3 kg was referred to our institution for cyanosis and a systolic murmur. A 2-dimensional echocardiogram suggested SDL, levocardia, criss-cross heart with double-outlet right ventricle, hypoplasia of the tricuspid valve and right ventricle, and severe pulmonary stenosis. A real-time 3-dimensional echocardiogram (Sonos 7500, Philips Medical Systems, Andover, Mass) demonstrated the complex anatomy of the criss-cross heart as clearly as if one were viewing an anatomic specimen. The atria were situated in relatively normal positions, with normal venous connections. The left atrium was posterior and communicated via the mitral valve with the morphologically left ventricle situated inferiorly and extending to the right …

Background: The aim of this prospective study was to evaluate the effect of the closed loop stimulation (CLS) on the ejection fraction in pediatric patients, affected by complete atrioventricular block (CAVB) or CAVB and sinus node dysfunction (SND), with a previously implanted pacemaker (PM) and ventricular dysfunction. The role of electrical therapy in the treatment of pediatric patients with congenital atrioventricular (AV) blocks has been shown. Conventional right ventricular pacing seems to affect ventricular function. Up to now, the feasibility and the long-term results of biventricular pacing in pediatric patients were not entirely clear. Methods: In eight pediatric patients with a previously implanted single or dual chamber PM, ventricular dysfunction, and CAVB or SND and CAVB, a dual chamber PM INOS 2+ -CLS (Biotronik GmbH, Berlin, Germany) was implanted. The effect of the physiological modulation of CLS pacing mode on the ejection fraction was evaluated by Echo-Doppler examination. Measurements were performed before the substitution of the old PM and for up to 2 years of follow-up. Results: All patients showed correct electrical parameters at implantation and during follow-up. The mean value of the ejection fraction measured before the replacement of the old PM was 36 ± 7%, while after 2 years it was 47 ± 1% (P < 0.003). No patient showed any worsening of the ejection fraction, while only one showed no improvement. Conclusions: DDD-CLS pacing seems to improve ventricular function in pediatric patients with CAVB and/SND in spite of the use of the apical right conventional stimulation. (PACE 2007; 30:33‐37)