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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.

ObjectivesTo evaluate the incidence of pericardial effusion (PE) after surgical atrial septal defect (ASD) closure and to investigate the presence of predictive risk factors for its development.MethodsWe collected data from 203 patients followed at Bambino Gesù Children’s Hospital of Rome who underwent cardiac surgery for ASD repair between January 2015 and September 2019.ResultsA total of 200/203 patients with different types of ASD were included. Patients were divided into two groups: Group 1) 38 (19%) who developed PE and Group 2) 162 (81%) without PE. No differences were noted between the two groups with regard to gender or age at the surgery. Fever in the 48 h after surgery was significantly more frequent in group 1 than in group 2 (23.7 vs. 2.5%; p < 0.0001). ECG at discharge showed significant ST-segment elevation in children who developed PE, 24.3 vs. 2.0% in those who did not (p < 0.0001). Group 1 patients were divided into two subgroups on the basis of the severity of PE, namely, 31 (81.6%) with mild and 7 (18.4%) with moderate/severe PE. Patients with moderate/severe PE had a significantly higher BMI value (median 19.1 Kg/m2) (range 15.9–23.4, p = 0.004).ConclusionThe presence of fever and ST-segment elevation after surgery predicts subsequent development of PE suggesting a closer follow-up for these categories of patients. A higher BMI appears to be associated with a higher risk of moderate/severe PE.

Jacobsen syndrome (JS) is a rare syndrome caused by a deletion of chromosome 11q. We report a patient with JS and hypoplastic left heart syndrome (HLHS) who required cardiac transplantation. She had many of the recognized morphological features in addition to immunological (lymphopenia) and hematological (thrombocytopenia) issues. The patient underwent a Norwood procedure with a modified Blalock–Taussig shunt (MBTS) and subsequently a Glenn procedure at six months of age. She developed desaturation, with severe tricuspid regurgitation and right ventricular dysfunction, and underwent heart transplantation at 7 months of age. After the transplant, she was hospitalized several times for severe infections. The diagnosis of Jacobsen syndrome came 2 months after transplant. Now, 5 years post-transplant, she is in relatively good health—her heart is functioning normally, her hospitalization rate is getting lower, and her immunological profile is stable.

Background The aim of this study is to identify echocardiographic predictors of transient left ventricle dysfunction after pulmonary valve balloon dilatation (PVBD), in neonates with pulmonary valve stenosis (PVS) and atresia with intact septum (PAIVS) at birth. Methods The study includes patients admitted at the Bambino Gesù Children Hospital from January 2012 to January 2017. Clinical, echocardiographic and cardiac catheterization data before and after PVBD were retrospectively analyzed. Results Twenty-nine infants were included in the study (21 male and eight female). The median age was 5.8 ± 7.1 days. Eight patients developed transient LV dysfunction (three PAIVS and five PVS) and comparing data before and after the procedure, there was no difference in right ventricle geometrical and functional parameters except for evidence of at least moderate pulmonary valve regurgitation after PVBD. Conclusion Moderate to severe degree pulmonary valve regurgitation was significant associated to LV dysfunction (p < 0.05) in PVS and PAIVS patients.

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.

Conventional echocardiography is an essential tool for the diagnosis, bedside management and follow-up evaluations of children with multisystem inflammatory syndrome associated with COVID-19. However, a more comprehensive echocardiographic exam, including myocardial deformation parameters, may allow early identification of subtle changes in ventricular function, provide risk stratification and, identify sub-clinical cardiac dysfunction at follow-up. Thus, myocardial deformation analysis should be routinely integrated to conventional echocardiography assessment in these patients.

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.

Summary Purpose Our aim is to compare new non-invasive imaging modalities in the evaluation of vascular ring (VR) and pulmonary artery sling (PAS) and to understand the role of bronchoscopy in comparison with them in assessing tracheobronchial tree. Methods We have retrospectively analyzed the data from 41 patients with a VR or a PAS diagnosed at Bambino Gesu Children's Hospital of Rome, between 2008 and 2012. Age, gender, presenting symptoms, clinical history, comorbidities, imaging modalities used for diagnosis (cardiac magnetic resonance [CMR], computed tomography [CT], tracheobronchoscopy [TB]) and surgical treatment were recorded. Results The vascular anatomy was completely defined in all patients, whether evaluated by CMR or CT, with a diagnostic accuracy of 100% based on surgical observation. All CT exams were performed without sedation with a mean dose-length product (DLP32) of 29 ± 9 and an effective dose of 1.56 ± 0.6 mSv, range 0.5–2.5 mSv. CMR required general anesthesia in all patients but involved no exposure to ionizing radiation. CT performed better than CMR in assessing tracheal stenosis when compared to TB. It detected complete tracheal cartilage rings in 2/3 patients with PAS, besides tracheomalacia and/or bronchomalacia in 54% of patients. Conclusions Both cross-sectional imaging modalities (CT and CMR) can reliably and accurately diagnose these congenital vascular anomalies. While CT involves exposure to ionizing radiation, it avoids the risks related to anesthesia needed for CMR, and provides a more accurate assessment of tracheobronchial anatomy. TB remains a fundamental tool in tracheomalacia diagnosis in VR symptomatic patients and PAS. Pediatr Pulmonol. 2015; 50:781–788. © 2014 Wiley Periodicals, Inc.

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.

Our aim is to compare new non-invasive imaging modalities in the evaluation of vascular ring (VR) and pulmonary artery sling (PAS) and to understand the role of bronchoscopy in comparison with them in assessing tracheobronchial tree.We have retrospectively analyzed the data from 41 patients with a VR or a PAS diagnosed at Bambino Gesù Children's Hospital of Rome, between 2008 and 2012. Age, gender, presenting symptoms, clinical history, comorbidities, imaging modalities used for diagnosis (cardiac magnetic resonance [CMR], computed tomography [CT], tracheobronchoscopy [TB]) and surgical treatment were recorded.The vascular anatomy was completely defined in all patients, whether evaluated by CMR or CT, with a diagnostic accuracy of 100% based on surgical observation. All CT exams were performed without sedation with a mean dose-length product (DLP32 ) of 29 ± 9 and an effective dose of 1.56 ± 0.6 mSv, range 0.5-2.5 mSv. CMR required general anesthesia in all patients but involved no exposure to ionizing radiation. CT performed better than CMR in assessing tracheal stenosis when compared to TB. It detected complete tracheal cartilage rings in 2/3 patients with PAS, besides tracheomalacia and/or bronchomalacia in 54% of patients.Both cross-sectional imaging modalities (CT and CMR) can reliably and accurately diagnose these congenital vascular anomalies. While CT involves exposure to ionizing radiation, it avoids the risks related to anesthesia needed for CMR, and provides a more accurate assessment of tracheobronchial anatomy. TB remains a fundamental tool in tracheomalacia diagnosis in VR symptomatic patients and PAS.