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Objectives: The surgical technique for right partial anomalous pulmonary venous return (PAPVR) depends on the location of the anomalous pulmonary veins (PVs). With this in mind, we sought to evaluate the impact of 3D heart segmentation and reconstruction on preoperative surgical planning. Methods: A retrospective study was conducted on all patients who underwent PAPVR repair at our institution between January 2018 and October 2021; three-dimensional segmentations and reconstructions of all the heart anatomies were performed. A score (the PAPVR score) was established and calculated using two anatomical parameters (the distance between the most cranial anomalous PV and the superior rim of the sinus venosus defect/the sum of the latter and the distance between the PV and the azygos vein) to predict the type of correction. Results: A total of 30 patients were included in the study. The PAPVR score was found to be a good predictor of the type of surgery performed. A value > 0.68 was significantly associated with a Warden procedure (p < 0.001) versus single/double patch repair. Conclusions: Three-dimensional heart segmentations and reconstructions improve the quality of surgical planning in the case of PAPVR and allow for the introduction of a score that may facilitate surgical decisions on the type of repair required.

Background: Although most children may experience mild to moderate symptoms and do not require hospitalization, there are little data on cardiac involvement in COVID-19. However, cardiac involvement is accurately demonstrated in children with MISC. The objective of this study was to evaluate cardiac mechanics in previously healthy children who recovered from severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection in a long-term follow-up by means of two-dimensional speckle-tracking echocardiography (STE). Methods: We analyzed a cohort of 157 paediatric patients, mean age 7.7 ± 4.5 years (age range 0.3–18 years), who had a laboratory-confirmed diagnosis of SARS-CoV-2 infection and were asymptomatic or mildly symptomatic for COVID-19. Patients underwent a standard transthoracic echocardiogram and STE at an average time of 148 ± 68 days after diagnosis and were divided in three follow-up groups (240 days). Patients were compared with 107 (41 females—38%) age- and BSA-comparable healthy controls (CTRL). Results: Left ventricular (LV) global longitudinal strain (post-COVID-19: −20.5 ± 2.9%; CTRL: −21.8 ± 1.7%; p < 0.001) was significantly reduced in cases compared with CTRLs. No significant differences were seen among the three follow-up groups (p = NS). Moreover, regional longitudinal strain was significantly reduced in LV apical-wall segments of children with disease onset during the second wave of the COVID-19 pandemic compared to the first wave (second wave: −20.2 ± 2.6%; first wave: −21.2 ± 3.4%; p = 0.048). Finally, peak left atrial systolic strain was within the normal range in the post-COVID-19 group with no significant differences compared to CTRLs. Conclusions: Our study demonstrated for the first time the persistence of LV myocardial deformation abnormalities in previously healthy children with an asymptomatic or mildly symptomatic (WHO stages 0 or 1) COVID-19 course after an average follow-up of 148 ± 68 days. A more significant involvement was found in children affected during the second wave. These findings imply that subclinical LV dysfunction may also be a typical characteristic of COVID-19 infection in children and are concerning given the predictive value of LV longitudinal strain in the general population.

Background Clinical manifestations of children's coronavirus disease-2019 (COVID-19) were initially considered less severe compared with adult patients. However, there is now increasing evidence of a “long-tail” of COVID-19 related symptoms lasting for several months after recovery from the acute infection. Long COVID-19-related symptoms and mechanisms are poorly characterized and understood, with several phenotypes reported, often driven by long-term tissue damage (such as lung, heart and brain) and pathological inflammation due to viral persistence and/or immune deregulation. Purpose The objective of this study was to evaluate atrio-ventricular mechanics, by means of two-dimensional speckle-tracking echocardiography, in previously healthy children recovered from asymptomatic or mildly symptomatic severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection in a long-term follow-up. Methods We analysed a cohort of 157 paediatric patients, mean age 7 ± 4 years, who had a confirmed diagnosis of SARS-CoV-2 infection and were asymptomatic or mildly symptomatic for COVID-19. Patients underwent standard transthoracic echocardiogram and speckle tracking echocardiographic study 148 ± 68 days after diagnosis. One hundred seven age, sex, and body surface area comparable healthy subjects were used as control group. Results Left ventricular ejection fraction was within normal limits in postCOVID-19 cases and CTRL with no significant differences between the two groups (postCOVID-19: 65.6 ± 4% vs CTRL: 65.0 ± 5%, p = 0.182).Left ventricular (LV) global longitudinal strain (postCOVID-19: -20.5 ± 2.9%; CTRL: -21.8 ± 1.7%; p < 0.001) was significantly reduced in cases compared with CTRLs. An amount of 11 (7%) postCOVID-19 cases showed impaired GLS values < -17% and 95 subjects (60%) presented with a strain lower than -16% in more than 2 segments. These subjects did not show any difference regarding symptoms or serological findings. Moreover, GLS was significantly reduced in children with disease's onset during the second wave of COVID-19 pandemic, compared with those during the first wave (second wave: -20.2 ± 2.6%; first wave: -21.2 ± 3.4%; p = 0.048). Finally, peak left atrial systolic strain was within the normal range in the postCOVID-19 group with no significant differences compared to CTRL (postCOVID-19: 49.1 ± 12%; CTRL: 49.5 ± 18%). Conclusions SARS-CoV-2 infection may affect left ventricular deformation in children despite an asymptomatic or only mildly symptomatic acute illness. Our data show an amount of 60% of children, recovering from asymptomatic or mildly symptomatic COVID-19, with still mild subclinical systolic cardiac impairment in the mid- and long-term follow-up after the infection. This subtle impairment was seen to be worse in children recovering from the second wave of COVID-19 compared to the first one. A follow-up is needed to verify the reversibility of these alterations and their impact on long-term outcomes.

Background Although the Fontan operation has contributed greatly to the improvement of survival in patients with a single ventricle, cardiac function and exercise capacity may deteriorate over time. However, there have been no quantitative analyses of the effects of Fontan circulation on myocardial work (MW). Recently, a novel non-invasive method for calculating MW has been introduced on the basis of speckle tracking analysis with the estimation of left ventricular (LV) pressure from brachial artery cuff pressure. Purpose Aim of this study was to evaluate the diagnostic performance of the non-invasive myocardial work indices in predicting subclinical myocardial work impairment in Fontan patients. Methods A total of 69 patients were included and compared with healthy age- and sex- matched controls (CTRL). Ventricular systolic function and global longitudinal strain (GLS) were assessed. Cardiopulmonary exercise test was performed. Global myocardial work index (MWI) was calculated as the area of the LV pressure strain loops. From MWI, global constructive work (MCW), wasted work (MWW) and work efficiency (MWE) were estimated. Results The two groups were comparable for blood pressure, weight and height. Mean age of Fontan patients was 21.0±9.2 years. MWI (1162 ± 364 mmHg% vs 1777 ± 240 mmHg%, p < 0.001), MCW (1554 ± 450 mmHg% vs 2102 ± 221 mmHg%, p = 0.001) and MWE (90 ± 6% vs 96 ± 2% p = 0.001) were significantly reduced in Fontan patients compared with healthy CTRL. Moreover, GLS (-13,9 ± 3,1% vs -21,2 ± 1.5%, p < 0.001) and the ejection fraction (EF) (58,9 ± 4.5% vs 63.3 ± 3.9%, p < 0.002) were significantly lower in Fontan patients. Fontan patients with normal EF showed, however, significantly reduced values of MWI compared with CTRL (p Fontan patients with functional right ventricle showed significantly reduced MWE compared with patients with functional left ventricle (p=0.030). In univariate analysis, peak VO2 was significantly associated with age, SatO2, MWI. In multivariate regression, lower peak VO2 was associated with older age (p = 0.003) and lower MWI (p = 0.026). Conclusions Fontan physiology is associated with disadvantageous ventricular work. In Fontan patients, estimation of MWI may be a more sensitive indicator of myocardial work impairment compared with EF and is able to predict exercise capacity.

Advanced echocardiographic and CMR features of patients with repaired Tetralogy of Fallot in the long-term follow-up. Anna Gozzi1, Jolanda Sabatino1, Elena Cuppini1, Irene Cattapan1, Alessia Basso1, Jennifer Fumanelli1, Angela Di Candia1, Martina Avesani1, Domenico Sirico1, Biagio Castaldi1, Elena Reffo1, Alessia Cerutti1, Roberta Biffanti1, Giovanni Di Salvo1 1Department for Women's and Children's Health, University Hospital of Padova, Pediatric and Congenital Cardiology Unit, Via Nicolo` Giustiniani, 2, 35128 Padova, Italy Background Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease and the population of ToF repair survivors is growing rapidly. Children and, more frequently young adults, with repaired ToF develop late complications. Sudden cardiac death and life-threatening ventricular arrhythmia remain a concern in patients with repaired tetralogy of Fallot. The aim of this study was to describe and analyse advanced echocardiographic and CMR features of patients with repaired ToF in the long-term follow-up and to find non-invasive predictors of adverse prognosis. Methods This is a retrospective cohort study. Consecutive 200 patients with repaired ToF who did not undergo pulmonary valve replacement were included. Mean age of all patients was 19 ± 8. The electrocardiographic (ECG), cardiopulmonary exercise testing (CPET), echocardiographic and cardiac magnetic resonance (CMR) data were reviewed retrospectively. Right myocardial work index (RWI) was calculated as the area of the right ventricular pressure strain loops. From RWI, Right Constructive Work (RCW), Wasted Work (RWW) and Work Efficiency (RWE) were estimated. Patients were screened according the age at surgery (12 months) and the time since the repair (< 25 years and ≥ 25 years). A composite end-point of major adverse cardiovascular events (sudden cardiac death, sustained and non-sustained ventricular tachycardia, resuscitated sudden cardiac death, or syncope) was used. Results CPET values were not significantly different in patients with more than 25 years since the corrective surgery. Left ventricular ejection fraction measured either by CMR or echocardiography were slightly but significantly reduced in patients with more than 25 years since the corrective surgery. Patients with older age at surgery (>12 months) have significantly reduced V02max at CPET, larger RA and LA areas and larger RV basal diameter. During a median follow-up of 2.9 years, 1 patient died suddenly, 5 had documented ventricular tachycardia, and other 4 had syncope. On univariate analysis, echocardiographic left atrial area/BSA (P=0.044) and right ventricular longitudinal strain (P=0.010) were significantly related to the combined end point. Patients with reported VT at Holter ECG presented significantly reduced right ventricular function in terms of CMR-EF, echocardiographic FAC and RWI. Finally, TOF patients with borderline/impaired predicted VO2 max at CPET presented with significantly reduced RV freewall longitudinal strain and right ventricular myocardial work index. Conclusion Long-term survival and clinical condition after surgical correction of ToF in infancy is generally good and the late functional status in ToF - operated patients could be excellent up to 25 years after the repair. However, patients with older age at surgery have relative reduced functional status compared with those undergoing early repair. The echocardiographic and CMR evaluation of RV function are usually related to worse prognosis, recurrency of VT and reduced functional status in patients with repaired TOF. Right ventricular myocardial work index is feasible in patients with repaired TOF; disadvantageous right ventricular work may be a more sensitive indicator of right ventricular impairment compared with standard echocardiographic parameters, and is able to predict exercise capacity.

OBJECTIVES The role of an additional ventricle in patients with a functional single ventricle undergoing the Fontan operation has been debated due to conflicting data. Our goal was to report our experience with Fontan circulation for complex congenital heart disease, with a focus on the influence that an additional ventricular chamber may have on early and long-term clinical outcomes. METHODS We performed a retrospective clinical study including all patients undergoing the Fontan procedure between 1978 and 2019. Clinical data were retrieved from our institutional database. A ‘biventricular’ Fontan (BVF) was defined as that performed in a patient with single ventricle anomaly where an additional diminutive ventricular cavity was present at echocardiographic evaluation. RESULTS A total of 210 consecutive patients with functional single ventricle were included. Among these, 46 had BVF (21.9%). Early complications occurred in 42 patients (20.0%; 11 in BVF vs 31 in univentricular Fontan; P = 0.53) There were 18 early deaths (8.6%) with no difference between the groups. At a median follow-up of 12.7 years (interquartile range 5.4–20.7), there were no significant differences in late mortality, whereas cardiac rhythm disturbances resulted more frequently in univentricular Fontan (P = 0.018). Statistical analysis showed an equal distribution of BVF across time (P = 0.620), and there were no significant differences in terms of early and late survival (P = 0.53 and P = 0.72, respectively) or morbidity (P = 0.45 and P = 0.80, respectively). CONCLUSIONS A secondary ventricle in Fontan circulation is not significantly related to any clinical disadvantage in terms of survival or onset of complications. However, the immediate postoperative course may be influenced negatively by the presence of an additional secondary ventricle.

Aims To investigate pulmonary valve (PV) and right ventricular function by echocardiography in paediatric patients with repaired Tetralogy of Fallot (ToF), comparing PV preservation surgical strategies to standard transannular patch (TAP) repair. Methods and results All patients undergoing transatrial-transpulmonary repair for ToF at our institution between January 2007 and May 2020 were reviewed retrospectively. Patients were divided into 2 groups, according to the different techniques used (PV preservation strategy vs TAP repair). All patients underwent standard echo-Doppler study including RV areas, fractional area change (FAC) and tricuspid annular plane systolic excursion (TAPSE); Pulmonary regurgitation (PR) was assessed by Color Doppler, continuous-wave (CW) Doppler, pressure half time (PHT) and PR index. By speckle tracking we measured also, in a subgroup of patients, right atrial strain (RAS), RV and left ventricle (LV) global longitudinal strain (RVGLS, LVGLS) and their time to peak (TTP) values. Eighty-two patients underwent a PV preservation strategy while 34 underwent a standard TAP repair. Five-year actuarial freedom from moderate/severe PV regurgitation was significantly higher in the PV preservation group compared to the TAP (61.3% [95% CI: 48-73%] vs 25.9% [95% CI: 12-43%], respectively; p = 0.02). After adjusting for age, gender, BSA, and type of PV, the use of a TAP was still significantly associated with an increased risk for PV regurgitation at Follow-up (HR: 1.85, 95% CI: 1.09, 3.15; p = 0.02). At a mean follow-up of 6.9 ± 0.3 years, patients undergoing PV preservation showed an increased right ventricular fractional area change (46.9 ± 0.8% vs 42.5 ± 1.7%, P Conclusion Surgical repair of ToF with PV preservation provides excellent outcomes in terms of PV competence and right ventricular function and should be advocated whenever possible.

Aims Pulmonary valve stenosis accounts for 6–9% of all congenital heart diseases. The main effect of this obstructive lesion is a rise in right ventricular pressure; this overload leads to multiple changes in shape, dimensions, and volume of the ventricle. The diagnosis is based on transthoracic echocardiography and invasive heart catheterization. Usually the stenosis is classified into mild, moderate, and severe based on pressure gradient between right ventricle and pulmonary artery and on the ratio between right ventricle and left ventricle systolic pressure. Percutaneous balloon valvuloplasty is the treatment of choice in severe pulmonary valve stenosis in patients of all ages; alternatively surgical valvotomy is an option in selected cases. The aim of this study is to evaluate the mechanical changes of the right ventricle in patients undergoing balloon pulmonary valvuloplasty using transthoracic and speckle-tracking echocardiography (STE). Furthermore, we sought to investigate the correlation between haemodynamic and echocardiographic parameters to better evaluate the degree of pulmonary valve stenosis before and after treatment. Methods and results Forty-three pediatric patients (19 males), mean age 3.2 ± 4.9 years with severe pulmonary valve stenosis and indication for percutaneous balloon valvuloplasty were recruited at the University Hospital of Padua. All patients underwent standard transthoracic echocardiography (TTE), STE with analysis of right ventricle global longitudinal strain (RVGLS) one day before and one day after the procedure. For each patient were collected invasive parameters during the interventional procedure before and after balloon valvuloplasty. After the procedure, there was an immediate statistically significant reduction of both peak-to-peak transpulmonary gradient (Dp post) and ratio between the systolic pressure of right and left ventricle (RV/LV ratio) with a drop of 29.3 ± 14.67 mmHg and 0.43 ± 0.03, respectively. Post-procedural echocardiography showed peak and mean transvalvar pressure gradient drop (50 ± 32.23 and 31 ± 17.97, respectively). The degree of pulmonary valve regurgitation was mild in 8% of patients before the procedure, following the intervention it reached 29% with a statistically significant increase (P = 0.007). However, the incidence of pulmonary valve moderate and severe regurgitation remained stable after the procedure. The analysis of right ventricular function and mechanics showed a significant improvement of Fractional Area Change (FAC) immediately after the procedure (40.11% vs. 44.42%, P = 0,01). On the other hand, right ventricular longitudinal systolic function parameters, TAPSE (P = 0.60) and longitudinal strain (P = 0.31), did not improve significantly after intervention. Finally, pre-procedural invasive RV/LV ratio showed good correlation to echocardiographic transvalvular peak and mean pressure gradient (R = 0.375, P = 0.019 and R = 0.40, P = 0.012, respectively), as well as with FAC (R = 0.31, P = 0.05), TAPSE (R = 0.62, P Conclusions Percutaneous balloon pulmonary valvuloplasty represents an efficient and safe procedure to relieve severe pulmonary valve stenosis. Interestingly, the analysis of right ventricular mechanics on echocardiography demonstrated an immediate global systolic function improvement following afterload reduction. Conversely, longitudinal systolic function did not show improvement immediately after intervention, possibly due to the necessity of longer time to recover. Finally, invasive preprocedural RV/LV ratio demonstrated better correlation with echocardiographic evaluation of stenosis degree and right ventricular function compared to invasive peak-to-peak pressure gradient. Therefore, RV/LV ratio should be preferred for the assessment of pulmonary valve stenosis.

Aims Autoimmune congenital heart block (CHB) is a severe manifestation of neonatal lupus syndrome. There is lack of consensus regarding treatment of pregnant women with anti-SSA/SSB autoantibodies. To evaluate the effectiveness of the combined protocol therapy (oral steroid, plasmapheresis, and IVIG) for the CHB treatment. Methods and results All cases of CHB from 2000 to 2020 were retrospectively enrolled. All the patients underwent foetal echocardiography for the evaluation of CHB, ventricular rate and main related foetal complications: cardiomegaly, pericardial and pleural effusion, foetal hydrops, dilated cardiomyopathy, and heart failure. Moreover, postnatal major adverse cardiovascular, such as death and pacemaker implantation, were recorded. For statistical analysis, the population was divided into two cohorts: a protocol group receiving in utero combined therapy and a control group receiving other therapies or not treated. Among 252 pregnancies with anti-SSA/SSB antibodies, 36 developed CHB. At birth, complete CHB treated with protocol therapies showed a significantly higher ventricular rate (P = 0.042), a significant reduction in intrauterine or postnatal mortality (P = 0.018), and a lower rate of pacemaker implantation (P = 0.049). Conclusions The combined treatment protocol has proven effective in improving foetal and neonatal short- and long-term survival.

Aims To describe the current application of exercise stress echocardiography (ESE) in our Paediatric Cardiology Departments. Methods and results Baseline and under stress symptoms, vital parameters, ECG, and echocardiograms from patients who underwent ESE in our centres, were retrospectively analysed and compared, as well as clinical management plans formulated based on ESEs results. Forty-five patients from Centre 1 (median age 16 years), including 87% of patients with congenital heart diseases (CHDs), and 20 patients from Centre 2 (median age 11 years), mainly tested to rule out myocardial ischemia, were included. Among patients from Centre 1, 28 had previously been treated surgically, 6 percutaneously, and 11 were under follow-up. Indications for ESE/patients’ native diagnosis are illustrated in the picture. Centre 1: Exercise was maximal in 17 patients, with 2 of them having symptoms at the peak of exercise. It was stopped beforehand in 28 patients because of dyspnoea (3) and muscle fatigue (25). No arrhythmia was detected. ESE was considered as positive in 14 patients; after that, 3 patients underwent percutaneous interventions, 2 underwent cardiac surgery, 3 received indication for cardiac catheterization, 4 for advanced cardiac imaging, and 2 for exercise restriction and medical therapy. Centre 2: 15 patients (75%) completed the exercise, and none of them developed symptoms. In the remaining 25%, exercise was stopped because of muscle exhaustion. No arrhythmia was detected, and all the ESEs were negative. Comparing the cohorts, no differences in terms of ejection fraction were noticed at rest and under stress. Patients in cohort 1 were older (P = 0.002), they achieved lower average maximal heart rate (P = 0.0001), performed less lasting exercise (P = 0.05), and ESE was maximal less frequently (P = 0.005). Lastly, they had significant changes in clinical decisions (P = 0.004). Conclusions In paediatric cardiology, particularly in children with CHDs, ESE is a promising technique and could influence significantly clinical management plans.

SCA from the right sinus is the rarest coronary anomaly. We describe 2 cases: 1 with SCA type-1RI; 2 with SCA type-2RII-A. Appropriate and successful treatment (CABG in case-1; PTCA in case-2) was chosen relying on accurate morphological description provided by MDCT, in order to recognize all the possible mechanisms of myocardial ischemia.

Funding Acknowledgements Type of funding sources: None. Background Despite sudden cardiac death (SCD) is a rare phenomenon in pediatric age, the use of the implantable defibrillator (ICD) is progressively increasing in consideration of its life-saving role both in primary and in secondary prevention. Our work aims at evaluating our pediatric population from the point of view of distribution according to heart disease, indication and implantation technique. We also want to verify the protective role of the device in terms of SCD prevention, acute and chronic complications and to evaluate the role played by the device as a bridge to transplantation. METHODS The retrospective study includes patients under the age of 18 underwent ICD implantation, following the guidelines and recommendations currently in force, in the time period between March 2000 and July 2020. Each device has been programmed in a personalized way based on age and cardiac pathology. The subjects were divided into three groups depending on the cardiac pathology. All patients were then followed-up by evaluating the appropriate shocks, the acute and chronic complications, especially the inappropriate shocks. RESULTS The study group includes 55 subjects (41 males and 12 females) with a mean age of 14 ± 4 years and a mean follow-up time of 6.2 years. Thirty subjects had cardiomyopathy (CM), 15 electrical disease (ED) and 10 congenital heart disease (CHD) respectively. We used three different implantation technique (endocavitary, subcutaneus and hybrid technique) depending on the size of the patient and the cardiac anatomy. There is no gender difference as regards the indication to the implant in primary/secondary prevention (67% in secondary prevention vs 33% in primary prevention). CHD and CM are mostly implanted in primary prevention. The rate of appropriate shocks in secondary prevention is higher than that in the primary one regardless of the disease. Thirty-eight percent of patients received appropriate shock, of which 66.7% were patients who had received defibrillator as a bridge to heart transplant. Two patients (3.6%) received inappropriate shocks on supraventricular tachycardia. There were no acute post-implantation complications. None dysfunctions or ruptures of the shock catheter or episodes of T wave oversensing took place. Peri-procedural and post-transplant mortality is zero, except for a deceased subject awaiting transplantation due to congestive heart failure. CONCLUSIONS The study confirmed the protective role of the device, in terms of prevention of sudden cardiac death, and its relevance as bridge to heart transplantation in pediatric age. An implant technique adequate to the size, age and heart disease of the patient associated with an adequate programming of the device significantly reduced inappropriate shocks in our patients.

International audience; Background and aim of the study: We sought to evaluate the appropriateness of cardiac anatomy renderings by a new virtual reality (VR) technology, entitled DIVA, directly applicable to raw magnetic resonance imaging (MRI) data without intermediate segmentation steps in comparison to standard three-dimensional (3D) rendering techniques (3D PDF and 3D printing). Differences in post-processing times were also evaluated.Methods: We reconstructed 3D (STL, 3D-PDF, and 3D printed ones) and VR models of three patients with different types of complex congenital heart disease (CHD). We then asked a senior pediatric heart surgeon to compare and grade the results obtained.Results: All anatomical structures were well visualized in both VR and 3D PDF/printed models. Ventricular-arterial connections and their relationship with the great vessels were better visualized with the VR model (Case 2); aortic arch anatomy and details were also better visualized by the VR model (Case 3). The median post-processing time to get VR models using DIVA was 5 min in comparison to 8 h (range 8-12 h including printing time) for 3D models (PDF/printed).Conclusions: VR directly applied to non-segmented 3D-MRI data set is a promising technique for 3D advanced modeling in CHD. It is systematically more consistent and faster when compared to standard 3D-modeling techniques.

Multisystem Inflammatory Syndrome in Children (MIS-C) is a known severe condition affecting children previously exposed to SARS-CoV-2. The aim of our study was to describe the early cardiac abnormalities in patients with MIS-C, evaluated by speckle tracking echocardiography (STE) and cardiac MRI (CMR). Clinical, laboratory and microbiological data were measured for all patients. All children underwent standard transthoracic echocardiography, STE with analysis of left ventricle global longitudinal strain (GLS). Seventeen (75%) of the children were evaluated with CMR. Twenty-three patients (13M, 10F) were recruited, mean age was 8.1 ± 4 years. Cardiovascular symptoms were present in 10 (43.5%). Nine children (39.1%) shared Kawasaki Disease-like symptoms. Four patients (17.4%) needed ICU admission. In-hospital survival was 100%. TnI was elevated in 15 (65.2%) and BNP in 20 (86.9%) patients. The median time to STE evaluation was 8 days and to CMR was 18 days after fever onset. Mean LVEF was 59 ± 10%. Coronary dilation was observed in six (26.1%) patients. STE showed a reduced mean LVGLS (−17 ± 4.3%). LGE with a non-ischemic pattern was evident in six out of seventeen patients (35.2%). The elevation of myocardial necrosis markers, the reduction of LVGLS and the presence of LGE on CMR in about a quarter of MIS-C patients supports the hypothesis of a post-viral immune-mediated myocarditis-like pathogenesis.

Funding Acknowledgements Type of funding sources: None. INTRODUCTION Many centers have recently adopted pulmonary valve (PV) preservation procedures to prevent the detrimental long-term effects of chronic pulmonary regurgitation after tetralogy of Fallot (ToF) repair. PURPOSE The aim of our study was to investigate pulmonary valve (PV) and right ventricular function by echocardiography in paediatric patients with repaired Tetralogy of Fallot (ToF), comparing PV preservation surgical strategies to standard transannular patch (TAP) repair. METHODS All patients undergoing transatrial-transpulmonary repair for ToF at our institution between January 2007 and May 2020 were reviewed retrospectively. Patients were divided into 2 main groups, according to the different techniques used: patients undergoing a PV preservation strategy and patients undergoing TAP repair. All patients underwent standard echo-Doppler study including RV end-diastolic area (RVEDA), end-systolic area (RVESA), fractional area change (FAC) and TAPSE; PR was assessed by Color Doppler, continuous-wave (CW) Doppler and derived parameters such as pressure half time (PHT) and pulmonary regurgitation (PR) index. By speckle tracking we measured also, in a subgroup of patients, right atrial strain (RAS), RV and left ventricle (LV) global longitudinal strain (RVGLS, LVGLS) and their time to peak (TTP) values. RESULTS Overall, 82 patients underwent a successful PV preservation strategy while 34 underwent a standard TAP repair. At index surgery, BSA (0.31 ± 0.1 m2, P = 0.3), age (4.8 ± 0.3 months, P = 0.5) and preoperative PV Z-score (-3.20 ± 0.1, P = 0.1) did not different between groups. Five-year actuarial freedom from moderate/severe PV regurgitation was significantly higher in the PV preservation group compared to the TAP (61.3% [95% CI: 48-73%] vs 25.9% [95% CI: 12-43%], respectively; p = 0.02). After adjusting for age, gender, BSA, and type of PV, the use of a TAP was still significantly associated with an increased risk for PV regurgitation at follow up (HR: 1.85, 95% CI: 1.09, 3.15; p = 0.02). At a mean follow-up of 6.9 ± 0.3 years, patients undergoing PV preservation showed an increased right ventricular fractional area change (46.9 ± 0.8% vs 42.5 ± 1.7%, P CONCLUSIONS Surgical repair of ToF with PV preservation provides excellent outcomes in terms of PV competence and right ventricular function and should be advocated whenever possible. Abstract Figure. Degree of pulmonary regurgitation

Aims Compared with adult patients, clinical manifestations of children’s coronavirus disease-2019 (COVID-19) are generally perceived as less severe. The objective of this study was to evaluate cardiac involvement in previously healthy children with asymptomatic or mildly symptomatic severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection. Methods and results We analysed a cohort of 53 paediatric patients (29 males, 55%), mean age 7.5 ± 4.7 years, who had a confirmed diagnosis of SARS-CoV-2 infection and were asymptomatic or only mildly symptomatic for COVID-19. Patients underwent standard transthoracic echocardiogram and speckle tracking echocardiographic study at least 3 months after diagnosis. Thirty-two age, sex, and body surface area comparable healthy subjects were used as control group. Left ventricular ejection fraction was within normal limits but significantly lower in the cases group compared to controls (62.4 ± 4.1% vs. 65.2 ± 5.5%; P = 0.012). Tricuspid annular plane systolic excursion (20.1 ± 3 mm vs. 19.8 ± 3.4 mm; P = 0.822) and left ventricular (LV) global longitudinal strain (−21.9 ± 2.4% vs. −22.6 ± 2.5%; P = 0.208) were comparable between the two groups. Regional LV strain analysis showed a significant reduction of the LV mid-wall segments strain among cases compared to controls. Furthermore, in the cases group, there were 14 subjects (26%) with a regional peak systolic strain below −16% (−2.5 Z score in our healthy cohort) in at least two segments. These subjects did not show any difference regarding symptoms or serological findings. Conclusion SARS-CoV-2 infection may affect left ventricular deformation in 26% of children despite an asymptomatic or only mildly symptomatic acute illness. A follow-up is needed to verify the reversibility of these alterations and their impact on long-term outcomes.

The role of immunosuppressive therapy (IT) in paediatric autoimmune/immune‐mediated myocarditis remains poorly defined. To explore its role, we present a series of three consecutive paediatric patients with biopsy‐proven, virus negative, autoimmune/immune‐mediated myocarditis, with distinct clinical and pathological features, who have been successfully treated with IT, a 14‐year‐old boy with Loeffler's fibroblastic parietal endomyocarditis, a 6‐year‐old child with celiac disease with chronic active lymphocytic myocarditis, and a 13‐year‐old boy with long‐standing heart failure and active lymphocytic myocarditis. Patients started IT and entered follow‐up between July 2017 and September 2019; the first patient completed IT. IT was associated with a substantial and sustained recovery of cardiac function in our patients, regardless of their heterogeneous clinical and pathological features. Combination IT was well tolerated and enabled tapering and weaning off steroids.

Background: Pulmonary artery banding (PAB) is reported as an innovative strategy for children with end-stage heart failure (ESHF) to bridge to transplantation or recovery. We report our early experience with PAB to evaluate outcomes, indications, and limitations.Materials and Methods: This is a single-center prospective clinical study, including infants and children admitted for ESHF owing to dilated cardiomyopathy (DCM) with preserved right ventricular function after failure of maximal conventional therapy. All patients underwent perioperative anticongestive medical therapy with ACE inhibitor, beta blocker, and spironolactone. Post-operatively, all patients underwent echocardiographic follow-up to assess myocardial recovery.Results: We selected five patients (four males) who underwent PAB at a median age of 8.6 months (range 3.9–42.2 months), with preoperative ejection fraction (EF)

We report a case of inflammatory myofibroblastic tumor affecting the pulmonary artery in a 15-year-old male, presenting with a clinical scenario of recurrent pulmonary embolisms. During diagnostic workup for persistent fever, a mass in main pulmonary artery was detected at echocardiography and confirmed at angio-CT scan. The patient underwent a first successful surgical resection and discharged home with no echocardiographic evidence of residual lesions, but, after 5 months, he was admitted for hemoptysis and an angio CT-scan showed a mass in right pulmonary artery with multiple distal perfusion defects, suspicious for both thrombotic and secondary lesions. To prevent further embolisms, the patient was scheduled for a second surgical procedure, which allowed a complete removal of the tumor from major branches of right pulmonary arteries. Our experience highlights that, despite of its intermediate malignancy, inflammatory myofibroblastic tumor may behave as an extremely dangerous condition, requiring multiple surgeries an integrated and multidisciplinary approach.

Background Mitral valve resistance (MVR) is a hemodynamic consequence of mitral stenosis but it has no clear threshold and it has a shortage of data to be reliable. Purpose is to investigate match and mismatch between opening area and resistance especially in patients with moderate and mild mitral stenosis. Materials This cross section case control study comprised 88 patients with moderate and mild rheumatic mitral stenosis. Transthorathic echocardiographic study estimated: mitral valve area (MVA) both by planimerty (2D) and pressure half time (PHT), mitral valve score (MVS), mean transmitral pressure gradient (MPG), diastolic filling time(DFT), left ventricular out flow tract diameter (LVOTd) and velocity time integral (LVOT vti) , the MVR was calculated as: MPG/aortic flow ratio [(LVOTd) (LVOTvti)/ DFT] in dynes.sec.cm5, NYHA function class of all patients was estimated. We classified our patients into 2 groups, group 1 (51 patients) with matched MVR and group 2 (37 patients) with unmatched MVR (unexpected high MVR in relation to valve area). Methods Patients with moderate mitral stenosis have MVR less than 105 dynes.sec/cm5, while patient with mild mitral stenosis have MVR less than 76 dynes.sec/cm5 this is in the matched group, but there are patients with unmatched higher MVR. Group 2 compared to group 1; had higher NYHA function class (1.4±0.6 vs. 1.2±0.4, P < 0.05), MVS (8.1±1.8 vs 7±0.9, P < 0.05), MPG (11,3±3.7 vs.7.8±2.5 mmHg, P < 0.01) and higher MVR (122.37±29.87 vs. 67.18±20.12 dynes.sec/cm5 , P < 0.01), MVR showed positive correlation with MVS (r=0.5, P < 0.05), Step wise logistic regression analysis showed that MVS is the only independent predictor of the MVR severity in the mismatched (unexpected high) group , so the higher the MVS the higher the expected MVR whatever the MVA is ; (B±SE=6.997±2.826, t=2.476, 95% CI 1.241±12.752 with an odds ratio=0.412, P < 0.05). Conclusion It would make much more sense to investigate match and mismatch between opening area and resistance in rheumatic mitral stenosis, the only independent predictor of mismatch is the mitral valve score.

Surgical re-implantation of the left coronary artery (LCA) is the treatment of choice in anomalous origin of left coronary artery from pulmonary artery (ALCAPA). Despite normalization of left ventricular function after surgery, residual coronary lesions or myocardial fibrosis may be found. The aim of this study was to detect regional left ventricular dysfunction predictive of coronary lesions or residual myocardial fibrosis using speckle tracking. We enrolled ten patients treated with surgical re-implantation of LCA for ALCAPA. All patients were asymptomatic, and ejection fraction (EF) was normal. Using S-SR imaging, we studied longitudinal, radial, and circumferential function. A cardiac MRI was performed to assess myocardial fibrosis and the anatomy of the coronaries. In case of suspected coronary stenosis, a coronary angiography was performed. Finally, 20 normal subjects were enrolled as control group. Median age at surgery was 188 days, and mean follow-up was 8.7 ± 4.7 years. Longitudinal and circumferential functions were reduced in LCA territory compared to RCA territory and normal. MRI showed LCA stenosis in three of ten patients, confirmed by coronary angiography: these patients had the lowest longitudinal strain values in LCA territories (-11.7, -14.7 and -14.8%). Radial strain was preserved (Normal 45.6 ± 12.1, ALCAPA 43.5 ± 10.7%, p = ns), while circumferential strain was mildly depressed (-23.5 ± 3.8 vs. -20.3 ± 2.0%, p 0.05). After LCA re-implantation, ALCAPA patients showed a residual sub-endocardial damage in LCA territories. Despite a normal systolic and diastolic function, the prevalence of residual coronary lesions was high. A mean longitudinal strain-15% in LCA territories was able to identify those patients.

Congenital heart defects (CHDs) occur in 1% of live-born infants and frequently are associated with extracardiac malformations. This study aimed to assess the feasibility and accuracy of three-dimensional ultrasonography (3DUS) in fetuses with CHD and to investigate whether 3DUS can add information about the heart and general fetal morphology that shows other congenital malformations or suggests syndromes. For 30 fetuses affected by CHD, 3DUS was performed using a Sonos 7500 ultrasound machine with a cardiac 3D transducer. In 44% of the exams, 3DUS was completely diagnostic for the CHD, providing additional information in 28% of the exams. Furthermore, 3DUS showed 82% of associated malformations, providing the complete diagnosis in 57% of the cases and helping with recognition of syndromes in others. The diagnostic accuracy of 3DUS was superior, with a higher number of acquisitions per exam. Performance was better in fetuses younger than 24 weeks for general morphologic details and in fetuses older than 24 weeks for the heart morphology.

The right ventricle (RV) is the main ventricular chamber in many congenital heart diseases before and after surgical correction, and it is the most important determinant of outcome in postoperative tetralogy of Fallot and other complex malformations. Unfortunately its irregular crescentic shape does not allow the use of the geometric assumption used for the left ventricle. Many methods have been suggested in the literature to overcome this problem, none fully reliable. The introduction of volume-rendered 3-dimensional (3D) reconstruction of echocardiography images provides a tool for the direct measurement of cardiac chambers, not based on geometric assumptions. The aim of this research study was to determine the accuracy of 3D echocardiography (3DE) to measure RV volumes in pediatric patients with secundum atrial septal defects, compared with direct volume measurements performed during the intervention. We performed 3DE study in the operating department, with the patient anesthetized, intubated, and ventilated before the surgical procedure. Sequential 2-dimensional echocardiographic images for subsequent 3D rendering were acquired using an ultrasound machine with a transthoracic 4-MHz rotational or 5-MHz transesophageal omniplane probe; in the last 5 patients a machine was used that was equipped with a 3600-crystal real-time 3D probe. To validate the 3DE measurements, these were compared with the volume of the RV directly measured in the operating department, at the end of the surgical procedure, injecting saline solution through the tricuspid valve, using a graduate syringe. Among 25 pediatric patients enrolled in the study, with an age range of 1 and 14 years (mean 4 years) and a weight range of 8.5 to 57.4 kg (mean 18.6 kg), in 23 a mean of 3 echocardiographic acquisitions were performed and compared with the direct measurement. A close comparison was found between RV volumes measured by 3DE and direct volume measurements (P < .00001). The regression line, shifted toward the y axis, which describes the 3DE volumes, indicated that the echocardiographic measures overestimate the surgical ones. In our study this overestimation had the mean of 9% with values comprised between 3% and 19%. The coefficient of repeatability was 4.79 mL with all the values within this range (2 SD of the mean). We conclude that 3DE provides an accurate measurement of RV volume in pediatric patients with RV volume overload. It is a reliable, noninvasive, and nongeometric method of evaluation of the volume of this chamber, and can be considered a precious tool in the armamentarium of the pediatric cardiologist.

We describe a case of 17-year-old Chinese girl referred to our Pediatric Cardiology Unit for asthenia, reduced exercise tolerance, and dyspnea. Past medical history was relevant for multiple chest pain episodes in childhood and several syncopal episodes, for which the patient had been never evaluated. Clinical examination, electrocardiogram, and echocardiography were compatible with Williams–Beuren syndrome; such condition was later confirmed by genetic analysis. Cardiac magnetic resonance imaging showed transmural fibrosis of the apex with impaired left ventricular ejection fraction (29 %), severe stenosis of aortic sinotubular junction with left and right coronary ostia involvement; more importantly, the whole coronary artery tree beyond ostia was affected by multiple stenosis and aneurysmatic tracts. Ascending aorta proved hypoplastic, with post-stenotic dilation and multiple aneurysms. At the end of the diagnostic process, surgical risk was considered too high to proceed with the correction. The presented case is of educational value since it provides good iconographical illustration of diffuse, multiple-site coronary artery tree involvement, a rather rare co-morbidity in Williams–Beuren syndrome. Electronic supplementary material The online version of this article (doi:10.1186/s40064-015-1231-0) contains supplementary material, which is available to authorized users.

We report a case of a 3-month-old child, presenting with signs of myocardial ischemia and hemodynamic instability, requiring extracorporeal membrane oxygenation support. Due to the unclear preoperative instrumental findings, a surgical exploration was performed revealing the presence of coronary ostia stenosis and an intramural course of both coronary arteries. The child underwent a successful plasty of the coronary artery ostia by resecting the excessive obstructing fibrous tissue followed by unroofing of the intramural coronary artery course with restoring of a normal myocardial perfusion and function.

A 1-day-old newborn was referred with prenatal echocardiographic diagnosis of cardiac mass. Born by caesarean section at 38+3 weeks of pregnancy, he presented with cyanosis and bradycardia at birth, requiring immediate intubation and mechanical ventilation. Chest x-ray demonstrated cardiomegaly with a cardiothoracic index of 0.7 (Figure 1). Echocardiographic examination (Figure 2 and Movie I in the online-only Data Supplement) confirmed the diagnosis of a large cardiac mass located at the posterior wall and at the apex of the left ventricle (LV). The child presented with several episodes of paroxysmal supraventricular tachycardia, which were treated with DC shock and various pharmacological agents, such as amiodarone, adenosine, flecainide, digoxin, and metoprolol, and 1 episode of ventricular fibrillation with cardiac arrest requiring resuscitation. MRI showed a single large mass (5.5×5 cm) arising from the left atrioventricular groove and extending to the LV lateral wall and apex, up to the interventricular septum, with no LV inflow or outflow obstruction, and was suggestive …

Background Arterial Tortuosity Syndrome (ATS) is a very rare autosomal recessive connective tissue disorder (CTD) characterized by tortuosity and elongation of the large- and medium-sized arteries and a propensity for aneurysm formation and vascular dissection. During infancy, children frequently present the involvement of the pulmonary arteries (elongation, tortuosity, stenosis) with dyspnea and cyanosis. Other CTD signs of ATS are dysmorphisms, abdominal hernias, joint hypermobility, skeletal abnormalities, and keratoconus. ATS is typically described as a severe disease with high rate of mortality due to major cardiovascular malformations. ATS is caused by mutations in the SLC2A10 gene, which encodes the facilitative glucose transporter 10 (GLUT10). Approximately 100 ATS patients have been described, and 21 causal mutations have been identified in the SLC2A10 gene. Case presentation We describe the clinical findings and molecular characterization of three new ATS families, which provide insight into the clinical phenotype of the disorder; furthermore, we expand the allelic repertoire of SLC2A10 by identifying two novel mutations. We also review the ATS patients characterized by our group and compare their clinical findings with previous data. Conclusions Our data confirm that the cardiovascular prognosis in ATS is less severe than previously reported and that the first years of life are the most critical for possible life-threatening events. Molecular diagnosis is mandatory to distinguish ATS from other CTDs and to define targeted clinical follow-up and timely cardiovascular surgical or interventional treatment, when needed. Electronic supplementary material The online version of this article (doi:10.1186/s12881-014-0122-5) contains supplementary material, which is available to authorized users.

Primary cardiac tumours in infants and children are extremely rare, with an estimated incidence of 0.2% according to echocardiographic studies. Owing to their rarity, there is very little literature available, and most knowledge is based on collections of case reports. Therefore, we reviewed retrospectively our 27 years of clinical experience on the overall management of cardiac tumours among children in order to improve not only our knowledge but also to provide others with information about the incidence, clinical presentation, management, and long-term outcome of this rare disease. Between April, 1982 and April, 2009, 52 children were diagnosed with cardiac tumours at our Institution. Medical records and follow-up echocardiographic evaluations were studied. The diagnosis was prenatal in 35% of the patients. The most frequent tumour types were rhabdomyomas (61.5%), fibromas (15.4%), and myxomas (5.8%). There were no cases of primary malignant tumours. All diagnoses were achieved using two-dimensional echocardiography, and for 12 patients a pathological analysis was carried out. A total of 41 patients (79%) were managed medically, whereas 11 (21%) patients underwent surgical treatment. At a mean follow-up of 7.2 ± 5.4 years, two patients died of complications related to cardiac transplantation; all the remaining patients are in excellent clinical condition. In conclusion, cardiac tumours in paediatric practice are usually clinically and histologically benign. Only a few cases need surgery. Up to one-third of the cardiac masses are detectable prenatally. Rhabdomyoma is the most common histotype, followed by fibroma and myxoma. The long-term prognosis is generally good.