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Malan syndrome (MALNS) is an ultra-rare genetic disorder caused by heterozygous chromosomal microdeletions involving the 19p13.2 region or loss-of-function variants in the NFIX gene. It is characterized by specific phenotypical features, intellectual disability (ID), and limitations in adaptive functioning and behavioral problems. In a previous work, we defined the cognitive, adaptive, linguistic and visuomotor ability profiles in a group of 15 MALNS individuals, providing quantitative data from standardized evaluations. Here, we further extend the characterization of MALNS by analyzing the behavioral and psychopathological comorbidities of the same cohort, administering standardized tests. Children were evaluated from October 2020 to January 2022. Retrospective data analysis was also performed. Assessment consisted of clinical observations, structured parent interviews, and parent-reported questionnaires. For each scale, comparisons between subtests were performed. Results of our analysis show that the most prevalent psychiatric comorbidities are represented by anxiety symptoms (including GAD, separation anxiety and specific phobias), ADHD, autistic symptoms, and social and attention problems. Of note, minimal or no signs of ASD were observed. In conclusion, our findings indicate that the psychopathological and behavioral comorbidities, together with cognitive impairment, language problems and sensory difficulties interfere with development, daily activities and social participation, therefore contributing to the severity of the disability associated with MALNS. Awareness of this profile by professionals and caregivers can promote prompt diagnosis and support cognitive and behavioral development.

Cardiovascular involvement has a great impact on morbidity and mortality in sickle cell disease (SCD). Currently, few studies are available regarding the paediatric setting and, moreover, current guidelines for the echocardiogram screening program in the asymptomatic paediatric population are controversial. We performed a retrospective observational monocentric study on 64 SCD patients (37 male and 27 female, median age 10) at the Bambino Gesù Childrens’ Hospital, who had undergone a routine transthoracic echocardiogram. In total, 46 (72%) patients had at least one cardiac abnormality. Left atrial dilatation (LAD) was present in 41 (65%) patients and left ventricular hypertrophy (LVH) was found in 29 (45%) patients. Patients with LAD showed lower median haemoglobin levels (p = 0.009), and a higher absolute reticulocyte count (p = 0.04). LVH was negatively correlated with the median haemoglobin value (p = 0.006) and positively with the reticulocyte count (p = 0.03). Moreover, we found that patients with cardiac anomalies had higher transfusion needs and a lower frequency of pain crises. In our setting, cardiac involvement has a high prevalence in the paediatric cohort and seems to be associated with specific laboratory findings, and with a specific clinical phenotype characterized by complications related to high haemodynamic load.

OBJECTIVES The aim of this study was to identify the predictors of death and of reintervention after mitral valve replacement (MVR) in children. METHODS A single-centre retrospective study was performed including 115 patients under the age of 18 undergoing MVR between 1982 and 2019. For all patients, the ratio of prosthetic valve size (diameter in mm) to weight (kg) at surgery was calculated and long-term result was assessed. The primary outcome was freedom from mitral valve (MV) re-replacement. The composite secondary outcome was freedom from death or transplant. RESULTS Fifty-four patients had a previous surgical attempt to MV repair. The median age at surgery was 5.5 years (interquartile range 1.21–9.87). Death/transplant-free survival was 77 ± 4% at 5 years and 72 ± 5% at 10 years. Univariate analysis showed a size/weight ratio higher than 2 and age CONCLUSIONS MVR is a viable strategy in children with unrepairable MV disease. Mortality can be predicted based on size/weight ratio and age

OBJECTIVES The aim of this study was to elucidate predictors of death and reintervention after mitral valve (MV) surgery in children. METHODS A single-centre retrospective study was performed enrolling 142 patients younger than 18 years who underwent primary index surgical mitral repair or replacement at Bambino Gesù Children’s Hospital in Rome from July 1982 to April 2020. Patients with complete, transitional or partial atrioventricular septal defect and patients with single ventricle physiology were excluded. Patients were stratified according to the age group: group 1 (5 years old). The composite primary outcome was freedom from death or transplant. The secondary outcome was freedom from redo MV surgery. RESULTS Transplant-free survival was 89% at 5 years and 88% at 10 years. Stratified by age, group 1 had poorer outcome in comparison with other groups (log-rank test P = 0.105). Both univariate and multivariate analyses showed that age CONCLUSIONS The overall survival and freedom from reoperation in children undergoing MV surgery still need improvements. Younger age was a significant risk factor for death and reintervention both after repair and replacement of the MV. In particular, infants and neonates have a three-fold risk for death compared to children.

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.

Myxomas are slowly growing benign neoplasms which are rare in children. Up to 80% can be located in the left atrium and generate symptoms such as embolism, cardiac failure, fever and weight loss. Rarely, myxomas can be detected in the right ventricle outflow tract, causing arrhythmias, pulmonary emboli and sudden death. We report the case of a 13-year-old healthy child brought to the Emergency Department (ED) of the Children’s Hospital Bambino Gesù, Rome, for recent dyspnea, chest pain on exertion and new onset cardiac murmur. Patient underwent medical examination and echocardiogram with the finding of a rounded and lobulated voluminous mass in the right ventricle outflow tract (RVOT) which caused severe obstruction. The contrast computed tomography (CT) scan confirmed the presence of a heterogeneously enhancing soft-tissue mass occupying the RVOT with no evidence of pulmonary embolization. The mass was surgically excised, and the pathologic examination confirmed our suspicion of myxoma. Our experience suggests that myxoma can have mild clinical symptoms, the presentation may be non-specific, and diagnosis can be a challenge Careful examination and a diagnostic imaging workup, primarily with the transthoracic echocardiogram, are needful to make a rapid differential diagnosis and to better manage surgical treatment and follow-up.

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.

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.

Funding Acknowledgements Type of funding sources: None. Background In heart transplant (HT) patients, high LV filling pressure is considered a marker of rejection and predictive of increased mortality. Purpose Our study aims to correlate echocardiographic parameters to left-ventricular end diastolic pressure (LVEDP) at cardiac catheterization in transplant recipients. Methods This was a retrospective study of 50 HT patients (54% male) who underwent heart transplantation in paediatric age (0-18 years-old). The echocardiographic evaluation was performed within three weeks from the left heart cardiac catheterization. From apical view, we measured: left atrial strain (LAS) indices [atrial contraction (εac), LA filling (reservoir phase, εres), and LA passive emptying (conduit phase, εcon)], mitral doppler E/A, E/e’, global longitudinal strain (LVGLS) and strain rate. Results Median LVEDP was 10 mmHg (IQR 8.25-12 mmHg) and had the best correlation with decreased εres (r= -0.56, p Conclusions Non-invasive εres seems to be a good surrogate of invasive LVEDP. Monitoring εres may be of value in HT patients to survey for rejection and graft disfunction. Abstract Figure. Scatter plots LVEDP-εres correlation

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

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

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.

This manuscript describes the first report of takotsubo cardiomyopathy in a young heart transplant recipient following angry debate. Our patient is a 21-year-old woman with cardiac transplant performed owing to right ventricular failure in congenital heart disease. Positive echocardiography with typical asymmetry of regional function, positive enzymes, and negative biopsy and angiography met the criteria for the diagnosis of takotsubo cardiomyopathy. Patient was discharged after 1 week in good clinical conditions and fully recovered cardiac function. The development of takotsubo cardiomyopathy in transplanted heart suggests that re-innervation occurs, thus representing a target for catecholamine-induced cardiac dysfunction.

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

Invasive peak-to-peak pressure gradients are the current clinical reference standard for assessing aortic coarctation. To obtain them, patients need to undergo arterial heart catheterization. Unless an intervention is performed, the procedure remains purely diagnostic, while the concomitant risks remain.To validate MRI-based pressure mapping against pressure drop derived from heart catheterization and to define minimal clinical requirements.Prospective clinical validation study.Twenty-seven coarctation patients with an indicated heart catheterization were enrolled at two clinical centers.1.5T including 4D velocity-encoded MRI and 3D anatomical imaging of the aorta.Pressure drop across the stenosis was calculated by pressure mapping based on the pressure Poisson equation. Calculated pressure drops were compared with catheter measured data. Spatial and temporal resolution were analyzed using in silico phantom-based data as well as in vivo measurements.Pressure drop was compared to peak-to-peak measurements. A two-sample paired mean equivalence test was used.In patients without imaging artifacts and a required spatial resolution ≥5 voxel/diameter, significant equivalence of pressure mapping compared to heart catheterization was found (17.5 ± 6.49 vs. 16.6 ± 6.53 mmHg, P 0.001).Pressure mapping provides equivalent accuracy to pressure drop obtained from heart catheterization in patients 1) without previous stenting and 2) with sufficient spatial image resolution (at least 5 voxels/diameter). In these patients the method can reliably be performed prior to the actual procedure, and thus allows safe noninvasive treatment planning based on MRI.2 Technical Efficacy: Stage 3 J. Magn. Reson. Imaging 2019;49:81-89.

OBJECTIVES This study was designed to evaluate the difference in the prevalence of long-term arterial hypertension among patients with corrected aortic coarctation according to the existence of associated cardiac congenital lesions. METHODS We identified 235 patients who had undergone surgery for aortic coarctation and classified them into 2 groups: patients with isolated coarctation of the aorta (CoA) and patients with aortic coarctation associated with complex congenital heart disease. Data were retrospectively analysed. RESULTS There were 148 subjects with isolated CoA and 87 with complex CoA (CoA-c). Patients were defined as hypertensive if they required antihypertensive treatment and/or when blood pressure was above 95th percentile. Patients with isolated aortic coarctation were significantly younger than patients with CoA-c (P

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.

Identifying similar patients might greatly facilitate the treatment of a given patient, enabling to observe the response and outcome to a particular treatment. Case-based retrieval services dealing with natural language processing are of major importance to deal with the significant amount of unstructured clinical data. In this paper, we present the development and evaluation of a case-based retrieval (CBR) service tested on a collection of Italian pediatric cardiology cases. Cases are indexed and a search engine is proposed. Search functionalities, such as interactive MeSH normalization and relevance feedback, are proposed. While the qualitative evaluation aims to provide feedback and recommendations, the quantitative evaluation enables to estimate the precision of the system. In more than half of the cases and for up to two thirds of them, the system is able to suggest a similar episode of care at first rank. With an improvement of the feedback relevance strategy, we can expect an improvement of the precision. The CBR can be expanded to multilingual EHR and other fields.

International audience; Personalised 3D modelling of the heart is of increasing interest in order to better characterise pathologies and predict evolution. The personalisation consists in estimating the parameter values of an electromechanical model in order to reproduce the observed cardiac motion. However, the number of parameters in these models can be high and their estimation may not be unique. This variability can be an obstacle to further analyse the estimated parameters and for their clinical interpretation. In this paper we present a method to perform consistent estimations of electromechanical parameters with prior probabilities on the estimated values, which we apply on a large database of 84 different heartbeats. We show that the use of priors reduces considerably the variance in the estimated parameters, enabling better conditioning of the parameters for further analysis of the cardiac function. This is demonstrated by the application to longitudinal data of paediatric cardiomyopathies, where the estimated parameters provide additional information on the pathology and its evolution.

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.

Diabetes remains a predictor of incident heart failure (HF), independent of intercurrent myocardial infarction (MI) and concomitant risk factors. Initial cardiovascular (CV) characteristics, associated with incident heart failure (HF) might explain the association of diabetes with incident HF. METHODS AND RESULTS: Participants to the 2nd Strong Heart Study exam, without prevalent HF or coronary heart disease, or glomerular filtration rate

The presence and type of viral genomes have been suggested as the main etiology for inflammatory dilated cardiomyopathy. Information on the clinical implication of this finding in a large population of children is lacking. We evaluated the prevalence, type, and clinical impact of specific viral genomes in endomyocardial biopsies (EMB) collected between 2001 and 2013 among 63 children admitted to our hospital for acute heart failure (median age 2.8 years). Viral genome was searched by polymerase chain reaction (PCR). Patients underwent a complete two-dimensional echocardiographic examination at hospital admission and at discharge and were followed-up for 10 years. Twenty-seven adverse events (7 deaths and 20 cardiac transplantations) occurred during the follow-up. Viral genome was amplified in 19/63 biopsies (35%); PVB19 was the most commonly isolated virus. Presence of specific viral genome was associated with a significant recovery in ejection fraction, compared to patients without viral evidence (p < 0.05). In Cox-regression analysis, higher survival rate was related to virus-positive biopsies (p < 0.05). When comparing long-term prognosis among different viral groups, a trend towards better prognosis was observed in the presence of isolated Parvovirus B19 (PVB19) (p = 0.07). In our series, presence of a virus-positive EMB (mainly PVB19) was associated with improvement over time in cardiac function and better long-term prognosis.

An inappropriately high left ventricular mass (iLVM) may be detected in patients with diabetes mellitus. Several hemodynamic and nonhemodynamic factors stimulating LVM growth may actively operate in these patients. In this study, we assessed prevalence and factors associated with iLVM in patients with diabetes mellitus.We analyzed baseline data from 708 patients (61 ± 7 years, 57% treated for hypertension) with type 2 diabetes mellitus without evidence of cardiac disease enrolled in the left ventricular dysfunction in diabetes study. iLVM was diagnosed by Doppler echocardiography as LVM more than 28% of the expected LVM predicted from height, sex and stroke work.iLVM was detected in 166 patients (23%), irrespective of concomitant hypertension. Patients with iLVM were more frequently women, had higher BMI and prevalence of metabolic syndrome, higher serum triglyceride levels and were treated more frequently with metformin and diuretics. In a multivariate model, female sex [odds ratio (OR) 1.502 (95% confidence interval (CI) 1.010-2.231), P = 0.04], higher serum triglyceride levels [OR 1.007 (95% CI 1.003-1.012), P 0.001] and BMI [OR 1.220 (95% CI 1.116-1.335), P 0.001] emerged independently related to iLVM.iLVM is detectable in about a quarter of patients with type 2 diabetes mellitus without evidence of cardiac disease and is unrelated to blood pressure levels. The association between LVM and some components of metabolic syndrome in these patients may have important practical implications.

OBJECTIVE The aim of this study was to evaluate whether diabetes (DM) and impaired fasting glucose (IFG) were associated with early alterations in left ventricular geometry and function in a large population of adolescents and young adults independently of major confounders. RESEARCH DESIGN AND METHODS We analyzed echocardiographic data of 1,624 14- to 39-year-old participants (mean age 26.6 ± 7.7 years; 57% female) without prevalent cardiovascular disease from the fourth Strong Heart Study examination; 179 (11%) participants had DM and 299 (18%) had IFG. RESULTS Participants with DM and IFG were older and more often obese and hypertensive than participants with normal fasting glucose (NFG) (all P < 0.05). After adjustment for age, sex, systolic blood pressure, and body fat, diabetic and IFG participants had higher left ventricular mass index than those with NFG (41.5 ± 8.7 and 39.6 ± 9.2 vs. 35.6 ± 7.8 g/m2.7) and reduced stress-corrected midwall shortening (98 ± 8.6 and 99 ± 7.5 vs. 101 ± 8.5%; all P < 0.05). The prevalence of left ventricular hypertrophy was higher in DM (20%) and IFG (17%) than in NFG participants (12%; P < 0.05). Compared with the other groups, DM was also associated with higher prevalence of inappropriate left ventricular mass, concentric geometry, and more diastolic abnormalities independently of covariates (all P < 0.05). CONCLUSIONS In a population of adolescents and young adults, DM is independently associated with early unfavorable cardiovascular phenotype characterized by increased left ventricular mass, concentric geometry, and early preclinical systolic and diastolic dysfunction; early cardiovascular alterations are also present in participants with prediabetes.

Individuals with diabetes mellitus (DM) have a higher risk to develop heart failure. Clinical guidelines emphasize the importance of early diagnosis of left ventricular dysfunction (LVD) and preventive interventions in these patients. In this study we assessed the prevalence of LVD, systolic or diastolic, in DM patients without known cardiac disease recruited in the 'left ventricular DYsfunction in DiAbetes (DYDA)' study.We performed clinical, ECG, laboratory, and echocardiographic exams in 960 patients (61 ± 8 years, 59% hypertensive) recruited in the DYDA study from 37 Italian diabetes referral centres. ECG and echo exams were read in central facilities. Systolic LVD was defined as ejection fraction ≤ 50% or midwall shortening (MFS) ≤ 15%. Diastolic LVD was identified when transmitral E/A was out of the range of 0.75-1.5 or deceleration time of mitral E wave ≤ 140 msec.Echocardiographic data were obtained in 751 patients (78.2%). Isolated systolic LVD was detected in 22.0% of patients, isolated diastolic LVD in 21.5%, and combined systolic and diastolic LVD in 12.7%. All patients with systolic LVD had MFS ≤ 15%, while only 9% had an ejection fraction ≤ 50%. Higher LV mass, relative wall thickness, prevalence of concentric geometry, and LV hypertrophy characterized the patients with LVD.LVD is present in more than half of DM patients without clinically detectable cardiac disease and is associated with LV hypertrophy and concentric LV geometry. One-third of patients exhibits systolic LVD detectable at the midwall level.

Despite significant advances in its treatment, acute myocardial infarction (AMI) remains an important cause of heart failure (HF). Contemporary data remain lacking, however, describing long-term trends in the incidence rates, demographic and clinical profile, and outcomes of patients who develop HF as a complication of AMI. Our study sample consisted of 11,061 residents of the Worcester (MA) metropolitan area hospitalized with AMI at all greater Worcester hospitals in 15 annual study periods between 1975 and 2005. Overall, 32.4% (n=3,582) of patients with AMI developed new onset HF during their acute hospitalization. Patients who developed HF were generally older, more likely to have pre-existing cardiovascular disease, and were less likely to receive cardiac medications or undergo revascularization procedures during their hospitalization than patients who did not develop HF (p < 0.001). Incidence rates of HF remained relatively stable between 1975 and 1991 at 26%, but declined thereafter. Declines were also noted in the hospital and 30-day death rates among patients with acute HF (p < 0.001). However, patients who developed new-onset HF remained at significantly higher risk for dying during their hospitalization (21.6%) than patients who did not develop this complication (8.3%) (p