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Transcatheter closure of perimembranous ventricular septal defect is an alternative to surgery in selected small to medium defects with a reasonable distance between the defect and the aortic or tricuspid valves. Surgical closure is generally preferred for large defects with an inlet to outlet extension, aortic malalignment, or deficient aortic rim. We report two successful cases of percutaneous closure of large perimembranous ventricular septal defects with inlet to outlet extension using an Amplatzer Vascular Plug-II via a retrograde approach. Transcatheter closure of large perimembranous ventricular septal defect with inlet to outlet extension is feasible and facilitated by the characteristics of the Amplatzer Vascular Plug-II device and the specific implantation technique.

A patent ductus arteriosus in patients with transposition of the great arteries is usually beneficial to allow shunting between pulmonary and systemic circulations. However, if the duct is too large, it can cause haemodynamic instability, pulmonary oedema and compromised organ perfusion. We present a neonate in whom a 5 mm short and tubular ductus arteriosus was causing significant cardiac failure with necrotising enterocolitis and liver impairment, leaving him too unstable for the arterial switch operation. At day 14 of life (3.1 kg), the baby underwent successful transcatheter closure using an Amplatzer vascular plug (Abbott, Chicago, Il, USA) delivered through a 5Fr catheter from the aortic side. The procedure was uncomplicated and successful as the neonate was extubated 2 days later. He subsequently underwent successful arterial switch surgery.

Background: Multi-system inflammatory syndrome in children and Kawasaki disease have overlapping clinical features but comparative echocardiographic studies are lacking. Methods: We reviewed echocardiography findings of all multi-system inflammatory syndrome cases between 1st April and 31st July, 2020 and typical Kawasaki disease patients with coronary arteries abnormalities consecutively followed between 1st October, 2016 and June 30th, 2019. Results: We included 40 multi-system inflammatory syndrome children (25 males, 62.5%) and 45 Kawasaki disease patients (31 males, 68.9%) at a mean age of 6.4 years old and 8 years old, respectively. Four out of 40 multi-system inflammatory syndrome children had coronary arteries abnormalities. Left ventricle ejection fraction was normal in both groups. Global longitudinal strain was normal although Kawasaki disease group had significantly lower values (–20.0 versus –21.7%; p = 0.02). Basal segments were the most affected in Kawasaki disease patients with significant differences in the basal anterior, anterolateral, and anteroseptal strain: –18.2 versus –23.0% (p = 0.002), –16.7 versus –22.0% (p < 0.001), –16.7 versus –19.5% (p = 0.034), respectively. The basal anterolateral and anteroseptal segments in Kawasaki disease patients were the only ones with an absolute reduction of longitudinal strain (–16.7% both) consistent with the greater left main coronary involvement in this cohort. Conclusions: Our findings are consistent with the transient cardiac involvement in multi-system inflammatory syndrome, as opposed to the subtle and chronic myocardial involvement in Kawasaki disease children with coronary arteries abnormalities. We speculate that the mechanism of cardiac impairment in the few multi-system inflammatory syndrome children with reduced global longitudinal strain is not related to coronary arteries abnormalities.

The Atrial Flow Regulator (AFR) is a new self-expandable percutaneous-delivered fenestrated device providing an interatrial shunt. Its use in pediatric population has been reported in failing Fontan, pulmonary hypertension, or in patients with cardiomyopathy and venoarterial extracorporeal membrane oxygenator (ECMO) support. Its use in small children under 10 kg has not been reported.We report the initial single center experience of the AFR implantation in children below 10 kg.Four children underwent AFR implantation. Patients' age and weight ranged between 9 and 22 months and 5-8.7 kgs. Indications were to unload the left atrium during ECMO support for end-stage cardiomyopathy and to enlarge a restrictive interatrial shunt in two other patients with complex congenital heart diseases. Devices implanted were AFR-6 and AFR-8. Delivery sheaths used via venous femoral access were undersized and ranged from 9 to 11 Fr. Devices were successfully implanted and provided unrestrictive interatrial shunt in all cases. One child developed a nonocclusive thrombus in the inferior vena cava which resolved within 1 month. Clinical improvement and femoral vein patency were observed in all cases.AFR implantation is feasible in selected children under 10 kg. The AFR can be safely deployed through sheaths that are 1-2 Fr smaller than the recommended manufacturer size. More studies are needed to confirm safety and efficacy of the device in selected pediatric patients.

Multisystem inflammatory syndrome in children (MIS-C) is a rare, but severe complication of coronavirus disease 2019 (COVID-19). It develops approximately four weeks after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and involves hyperinflammation with multisystem injury, commonly progressing to shock. The exact pathomechanism of MIS-C is not known, but immunological dysregulation leading to cytokine storm plays a central role. In response to the emergence of MIS-C, the European Academy of Allergy and Clinical Immunology (EAACI) established a task force (TF) within the Immunology Section in May 2021. With the use of an online Delphi process, TF formulated clinical statements regarding immunological background of MIS-C, diagnosis, treatment, follow-up, and the role of COVID-19 vaccinations. MIS-C case definition is broad, and diagnosis is made based on clinical presentation. The immunological mechanism leading to MIS-C is unclear and depends on activating multiple pathways leading to hyperinflammation. Current management of MIS-C relies on supportive care in combination with immunosuppressive and/or immunomodulatory agents. The most frequently used agents are systemic steroids and intravenous immunoglobulin. Despite good overall short-term outcome, MIS-C patients should be followed-up at regular intervals after discharge, focusing on cardiac disease, organ damage, and inflammatory activity. COVID-19 vaccination is a safe and effective measure to prevent MIS-C. In anticipation of further research, we propose a convenient and clinically practical algorithm for managing MIS-C developed by the Immunology Section of the EAACI.

Prosthetic paravalvular leaks (PVLs) are associated with congestive heart failure and hemolysis. Surgical PVL closure carries high risks. Transcatheter implantation of occluding devices in PVL is a lower risk but challenging procedure. Of the available devices, only two have been specifically approved in Europe for transcatheter PVL closure (tPVLc): the Occlutech® Paravalvular Leak Device (PLD) and Amplatzer™ ParaValvular Plug 3 (AVP 3). Here, we review the various tools and devices used for tPVLc, based on three observational registries including 748 tPVLc procedures performed in 2005–2021 at 33 centres in 11 countries. In this case, 12 registry investigators with over 20 tPVLc procedures each described their practical tips and tricks regarding imaging, approaches, delivery systems, and devices. They considered three-dimensional echocardiography to be the cornerstone of PVL assessment and procedure guidance. Anterograde trans-septal mitral valve and retrograde aortic approaches were used in most centres, although some investigators preferred the transapical approach. Hydrophilic-coated low-profile sheaths were used most often for device deployment. The AVP 3 and PLD devices were chosen for 89.0% of procedures. Further advances in design and materials are awaited. These complex procedures require considerable expertise, and experience accumulated over a decade has no doubt contributed to improve practices.

Transcatheter closure of patent ductus arteriosus (PDA) in premature infants is a feasible, safe, and an effective alternative to surgical ligation and may be performed with an implant success rate of 97%. Major procedural complications related to transcatheter PDA closure in extremely low birth weight (ELBW) infants are relatively infrequent (

Rare cases of cardiac inflammation following vaccination for severe acute respiratory coronavirus 2 (SARS-CoV-2) have been reported.To study paediatric patients with clinical findings of acute inflammation post coronavirus disease 2019 (COVID-19) Pfizer/BioNTech vaccination using cardiovascular magnetic resonance imaging (MRI) in acute and subacute phases.We enrolled adolescents younger than 18 years who presented at one of two institutions between July 2021 and August 2022 with clinical and laboratory findings of acute myocarditis shortly following COVID-19 Pfizer/BioNTech vaccination. They all underwent cardiovascular MRI using the institutional myocarditis protocol.Five adolescents (four boys) underwent eight scans between 3 days and 109 days (mean 49 days) after the onset of symptoms following COVID-19 vaccination. Myocardial oedema appeared on short tau inversion recovery (STIR) T2-weighted images in three adolescents at presentation (3-12 days after symptom onset). In these children, the myocardial oedema/acute inflammation had resolved at follow-up cardiovascular MRI (53-68 days after first MRI). However, in all three adolescents, a persistent area of late gadolinium enhancement was evident at follow-up, suggesting post-myocarditic fibrosis. One adolescent scanned only once, 66 days after being symptomatic, had no acute inflammation but persistent fibrotic changes. This last adolescent, who underwent the first scan 109 days after symptom onset, had findings compatible with an episode of previous myocarditis, with mild ongoing regional myocardial oedema/inflammation.This study on post-vaccine myocarditis demonstrates residual lesions with persistent areas of late gadolinium enhancement/myocardial fibrosis with ongoing myocardial oedema after resolution of the initial myocardial oedema a few weeks after Pfizer/BioNTech vaccination. There is an urgent need to recognise and fully investigate the outcome of post-vaccination myocarditis.

Background: Congenitally corrected transposition of the great arteries (ccTGA) is a rare disease of unknown cause. We aimed to better understand familial recurrence patterns. Methods: An international, multicentre, retrospective cohort study was conducted in 29 tertiary hospitals in 6 countries between 1990 and 2018, entailing investigation of 1043 unrelated ccTGA probands. Results: Laterality defects and atrioventricular block at diagnosis were observed in 29.9% and 9.3%, respectively. ccTGA was associated with primary ciliary dyskinesia in 11 patients. Parental consanguinity was noted in 3.4% cases. A congenital heart defect was diagnosed in 81 relatives from 69 families, 58% of them being first-degree relatives, including 28 siblings. The most prevalent defects in relatives were dextro-transposition of the great arteries (28.4%), laterality defects (13.6%), and ccTGA (11.1%); 36 new familial clusters were described, including 8 pedigrees with concordant familial aggregation of ccTGA, 19 pedigrees with familial co-segregation of ccTGA and dextro-transposition of the great arteries, and 9 familial co-segregation of ccTGA and laterality defects. In one family co-segregation of ccTGA, dextro-transposition of the great arteries and heterotaxy syndrome in 3 distinct relatives was found. In another family, twins both displayed ccTGA and primary ciliary dyskinesia. Conclusions: ccTGA is not always a sporadic congenital heart defect. Familial clusters as well as evidence of an association between ccTGA, dextro-transposition of the great arteries, laterality defects and in some cases primary ciliary dyskinesia, strongly suggest a common pathogenetic pathway involving laterality genes in the pathophysiology of ccTGA.

Transcatheter closure of a patent ductus arteriosus in children is widely performed to reduce symptoms and decrease the risk of endocarditis. Most arterial ducts are closed successfully with dedicated devices. However, in a tubular or "type C" patent ductus arteriosus with congestive heart failure, the occlusion is more challenging with these devices, with a higher risk of complications, such as aortic or left pulmonary stenosis and device embolization.To report our experience with muscular ventricular septal defect devices for patent ductus arteriosus occlusion in small children.Retrospective observational series of patients weighing10kg, with a tubular patent ductus arteriosus (typeC) and congestive heart failure, who underwent transcatheter closure with a muscular ventricular septal defect device between 2017 and 2019.Eight patients were included. The mean age and weight at closure were 6.3 months (range 1-18 months) and 5.3kg (range 2.4-8.2kg), respectively. All patent ductus arteriosus were occluded successfully using Occlutech® (N=3) or Amplatzer® (N=5) muscular ventricular septal defect devices. In four cases, the muscular ventricular septal defect device was used after failure to close the patent ductus arteriosus with a dedicated patent ductus arteriosus device. Two patients had mild left pulmonary artery stenosis, with a maximum velocity on continuous Doppler of 3m/s and 2.7m/s, respectively. After a mean follow-up of 28 months (range 14-41 months), all patients were asymptomatic with excellent results. The mild pulmonary stenosis improved, with a maximum velocity of 2.3m/s in both patients.Closure of tubular patent ductus arteriosus in small children with congestive heart failure using a muscular ventricular septal defect device is safe in this preliminary experience. Further studies with more patients are warranted.

Access to complex stenotic pulmonary arteries can be challenging due to their anatomy or secondary to prior multiple surgeries and interventions. Two techniques have been previously described to address this issue: the telescopic catheter-in-long sheath parallel to a stiff guidewire technique and the use of a microcatheter in a telescopic scope. We integrated and modified these techniques creating a super telescopic system with a SuperCross® microcatheter-in-catheter-in-long sheath, parallel to a contralateral stiff guidewire to access a previously repaired and stented left pulmonary artery. The stiff wire support and the 90° flexiblity of the Supercross® microcatheter assembled coaxial to the diagnostic catheter and the long sheath contributed to the successful ballooning and stenting-in-stent of the pulmonary artery.

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.

AimsThis international multi-center study aimed to demonstrate the feasibility and reliability of non-invasive myocardial work (MW) parameters in the pediatric population, and to provide normal reference ranges for this useful echocardiographic tool in this specific subset of patients.Methods and ResultsIn this retrospective multi-center study involving three pediatric laboratories, 150 healthy children and adolescents (mean age of 10.6 ± 4.5, 91 males) were enrolled. A complete echocardiographic examination has been performed, including global longitudinal strain (GLS) assessment. The following parameters of non-invasive MW have been obtained through a dedicated software: global work index (GWI), global constructive work (GCW), global work waste (GWW), and global work efficiency (GWE), using left ventricular (LV) strain loops and non-invasive brachial artery cuff pressure values. The lowest expected values were for GWI 1,723 mmHg% in males and 1,682 mmHg% in females, for GCW 2,089 and 2,106 mmHg%, for GWE 95.9 and 95.5% whereas the highest expected value for GWW was 78 mmHg% in men and 90 mmHg% in women. The univariable and multivariable analysis showed significant associations between either GWI or GCW with SBP (β coefficient = 0.446, p < 0.001; β coefficient = 0.456, p < 0.001, respectively) and LV GLS (β coefficient = −0.268, p = 0.001; β coefficient = −0.233, p = 0.003, respectively). Inter- and intra-observer variability showed good reproducibility of non-invasive MW parameters.ConclusionNon-invasive MW parameters were feasible and reliable in the pediatric population. This study provided normal reference ranges of these useful echocardiographic indices.

The main indication for closing a secundum atrial septal defect (ASD) is a significant left-to-right shunt, with acceptable pulmonary vascular resistances.1 2 Transcatheter closure has become an accepted alternative to surgical repair for most types of ostium secundum ASD. The technique is commonly referred to for patients with a defect under 38 mm in diameter without deficient rims and allows safe and effective ASD closure in most unselected cases. The main limit for percutaneous closure is the size of the defect as well as the existence of appropriate rims.1–3 In many patients referred for transcatheter closure, the question is often ‘How large is too large?’3 In Dolgner et al ’s4 study, the above-mentioned statements are challenged after they report 10% of stroke in a selected study population of 346 patients with a secundum ASD and no known atrial arrhythmias. The authors provide a risk stratification based on the three following independent risk factors for this population: a body mass index >25, smoking and a prominent Eustachian valve. They conclude that ASD closure could be considered in this patient population, even without a significant left-to-right shunt.4 This study is remarkable because this is the …

Background: Transcatheter closure for atrial septal defect (ASD) with inferior-posterior rim deficiency has been scarcely reported with proper identification of the indications and limits. We aimed to assess the safety and feasibility of transcatheter closure of ASDs with deficient rims, paying particular attention to cases with inferior-posterior rim deficiency. Methods: From January 2008 to January 2013, 241 patients underwent transcatheter ASD closure, including 50 cases (20.7%) with deficient rims, other than the anterior-superior one. Eighteen patients (12 females) presented inferior-posterior rim deficiency. Their median age was 8 (1.4–85) years and their median weight was 24 [9–97] kg. Transcatheter closure was performed in all cases under transesophageal echocardiography (TEE) guidance in children and intracardiac echocardiography (ICE) guidance in adults. Results: Out of 18 patients with inferior-posterior rim deficiency, only 8 underwent successful immediate transcatheter closure. Four cases failed to be closed. Major complications occurred in 6 patients, including 4 device embolizations, 1 pericardial effusion and 1 complete atrioventricular block that resolved after surgical removal of the device. During a median follow up of 54±13 months, a residual right-to-left shunt was documented in 2 more cases, requiring surgery in one case because of cyanosis. Transcatheter closure was successfully performed in the rest of the 223 patients, including in the 32 cases with deficient rims other than inferior-posterior. Conclusions: Transcatheter closure of ASDs with inferior-posterior rim deficiency cannot be recommended.

Many interventionists are infatuated by the recent resurgence of the coilwire design with the Super Arrow-Flex® sheath (Teleflex, Inc., NC, United States of America). This exclusive sheath is a highly flexible, durable, conduit intended for use in diagnostic and interventional procedures with several advantages and maximum effectiveness in challenging cases. We report failure to easily advance memory shape occluders through Super Arrow-Flex® sheaths larger than the recommended implant French size. We detail the technical reasons behind this non-previously reported drawback and describe benchside tests as possible solutions.

Objective: Investigating accuracy of cardiac tomography (CT) derived post-processing3D reconstruction (CT-PPR) and 3D printing to predict percutaneous pulmonary valve implantation (PPVI) feasibility. Background: PPVI feasibility remains challenging in large native regurgitant right ventricle outflow tract (RVOT). Methods: Fifteen patients with large native RVOT were investigated. CT-PPR consisted in RVOT long-axis curvilinear reconstruction (LACR) to measure the landing zone (LZ), and 3D volume rendering for morphological evaluation. A STL was generated to create 3D printed model (flexible resin). Balloon sizing was subsequently performed to measure LZ diameter (3D-MBD), compared to invasive balloon diameter (IBD) during catheterization, considered as the Gold Standard. Two operators predicted the feasibility of PPVI using CT-PPR and 3D printed models independently and blinded to outcome. Results: On 3D printed models, RVOT shape was tubular in 5 patients, divergent in 5 patients, concave in 4 patients and convergent in one. Agreement with CT-PPR RVOT shape morphology assessment was observed in 93% of cases (Kappa coefficient 0.91, p

Percutaneous closure of secundum atrial septal defect (sASD) in children with interrupted inferior vena cava is challenging, especially in case of deficient aortic rim. Trans-jugular access is generally preferred in this scenario. Patients with situs inversus and sASD also carry technical difficulties for transcatheter closure because of the orientation of the atrial septum. We report a successful case of percutaneous closure of a sASD with deficient aortic rim using an occlutech figulla flex II ASD device through the left internal jugular vein in a child with situs inversus, dextrocardia, and interrupted IVC. This case was facilitated by the absence of left-sided hub of the Occlutech device to provide stable opening of the device into the left atrium, whereas the ball-connection of the delivery system allowed an angle of almost 180 degrees between the device and the atrial septum.

Background Short-term sequelae of Multisystem Inflammatory Syndrome in Children (MIS-C), recently published by our institution, showed rapid improvement of the cardiac abnormalities within a few weeks after the onset of the disease. However, subtle residual abnormalities, affecting mainly the myocardial interstitium, were shown in some of the patients. The current study aimed to assess myocardial deformation with CMR shortly after MIS-C. Methods Sixty children were included into the study; 30 following MIS-C (onset-to-scan mean 27 days, SD 11) and 30 controls. Strain values were compared between patients and controls and additionally to published paediatric normal CMR values. U-Mann Whitney test was used for comparison of the myocardial deformation between patients and controls. Results Median age of the patients was 9.0 years (range 0.99–14.4) and controls 9.8 years (range 4.7–14.9). All conventional CMR parameters in patients were in normal range. Strain values were significantly lower in patients than in controls. When compared to published centile graphs, radial and circumferential global strain was within 2.5th and 97.5th centile in all patients. Eleven patients had global longitudinal strain between 2.5th centile and 50th centile, 1 patient was below 2.5th centile and all the others above 50th centile. Only 3 controls had global longitudinal strain between 2.5th centile and 50th centile, all other had higher strain. Conclusions This study demonstrates that myocardial deformation indices measured by CMR are within normal range in the vast majority of the patients within a few weeks after the onset of MIS-C. However, when compared to healthy controls, all strain parameters were lower in patients.

Kawasaki disease (KD) can be associated with high morbidity and mortality due to coronary artery aneurysms formation and myocardial dysfunction. Aim of this study was to evaluate the diagnostic performance of non-invasive myocardial work in predicting subtle myocardial abnormalities in Kawasaki disease (KD) children with coronary dilatation (CADL). A total of 100 patients (age 8.7 ± 5 years) were included: 45 children with KD and CADL (KD/CADL) (Z-score > 2.5), 45 age-matched controls (CTRL) and, finally, an additional group of 10 children with KD in absence of coronary dilatation (KD group). Left ventricular (LV) systolic function and global longitudinal strain (GLS) were assessed. 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. Despite normal LV systolic function by routine echocardiography, KD/CADL patients had lower MWI (1433.2 ± 375.8 mmHg% vs 1752.2 ± 265.7 mmHg%, p . MWI, MCW and MWE were significantly reduced in KD children despite normal LVEF and normal GLS. These abnormalities seems independent from CADL. Thus, in KD with normal LVEF and normal GLS, estimation of MWI may be a more sensitive indicator of myocardial dysfunction.

Introduction Congenitally corrected transposition of the great arteries (ccTGA) is a rare disease of unknown aetiology. We aimed to better understand familial recurrence pattern. Methods An international, multicentre, retrospective cohort study was conducted in 29 tertiary hospitals in 6 countries between 1990 and 2018, leading to investigate 1043 unrelated ccTGA probands. Results Atrioventricular block at diagnosis and laterality defects were observed in 35.4% and 29.9%, respectively. ccTGA associated with primary ciliary dyskinesia in 10 patients. Parental consanguinity was noted in 3.4% cases. A congenital heart defect was diagnosed in 81 relatives from 69 families, 58% of them being first-degree relatives, including 28 siblings. The most prevalent defects in relatives were dextro-transposition of the great arteries (d-TGA: 28.4%), laterality defects (13.6%) and ccTGA (11.1%); 36 new familial clusters were described, including 8 pedigrees with concordant familial aggregation of ccTGA, 19 pedigrees with familial co-segregation of ccTGA and d-TGA and 9 familial co-segregation of ccTGA and laterality defects. In one family there was co-segregation of ccTGA, d-TGA and heterotaxy syndrome in 3 distinct relatives. Conclusion ccTGA is not always a sporadic congenital heart defect. Familial clusters as well as evidence of an association between ccTGA, d-TGA, laterality defects and in some cases primary ciliary dyskinesia strongly suggest a common pathogenetic pathway involving laterality genes in the pathophysiology of ccTGA.

Funding Acknowledgements Type of funding sources: None. Introduction The paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) and Kawasaki disease (KD) have overlapping features. This study aimed to describe the strain segmental analysis among both entities. Methods Retrospective review of strain segmental analysis within 4 weeks of presentation of symptoms among children diagnosed with PIMS-TS between April and June 2020 and a historic cohort of typical KD from the Royal Brompton Hospital, London. Results We included 33 PIMS-TS patients (23 males, 69.7%) at a mean age of 8 ± 4,9 years old and 45 KD patients (31 males, 68,9%) at a mean age of 5,8 ± 4,5 years old. PIMS-TS patients were older at presentation (p = 0.038). Left ventricle ejection fraction (LVEF) was normal in both groups (63,3% vs 63,5%; p= 0,89), 4/33 PIMS-TS children (12,1%) had coronary arteries abnormalities (CAA), whereas 100% of KD cohort had CAA. Both groups had a normal global longitudinal strain (GLS),but in PIMS-TS it was significantly reduced compared to the KD group (-20% vs -22%; p = 0,008). Basal segments were the most affected in PIMS-TS with significant difference in the basal anterior and anterolateral strain compared to KD (respectively -18,2% vs -23,4%; p < 0,001 and - 16,7% vs -22,7%; p < 0,001). PIMS-TS had a greater anterior, anterolateral and posterior segments involvement with a significant reduction in the anterolateral mid-wall longitudinal strain (-18,3% vs -22%; p = 0,002). Apical segments were less involved, with significant difference only in the septal and inferior apical strain (respectively p = 0.001 and p = 0,032). Conclusions These preliminary data showed that after 4 weeks from the onset of symptoms, all PIMS-TS patients had a normal LVEF but they had a significant reduction in GLS and different segmental involvement compared to KD cohort. We hypothesize that these findings may be related to direct myocardial damage in PIMS-TS rather than caused by coronaries perfusion abnormalities. Abstract Figure. Bull"s eye

Funding Acknowledgements Type of funding sources: None. Background Left ventricular non-compaction cardiomyopathy (LVNC) is associated with poor clinical outcome in childhood, with heart failure, arrhythmias, and embolic events as main clinical manifestations. LV twist has recently been demonstrated having good predictive value in diagnosing LVNC in young patients. However, how LV twist may influence long-term clinical outcome of LVNC children and adolescents is still unknown. The aim of this study was to analyse a set of echocardiographic parameters to find imaging predictors of worse clinical outcomes in a long-term follow-up of LVNC children and young patients. Methods Children and adolescents, followed from May 2012 to June 2020, were enrolled in a retrospective study. All patients underwent 2-dimensional speckle tracking echocardiography and cardiovascular magnetic resonance imaging (1.5 Tesla) at our Institution’s first evaluation. Death, heart failure hospitalization, aborted sudden cardiac death, ventricular arrhythmias (sustained and non-sustained ventricular tachycardia), and embolisms (i.e. stroke, peripheral arterial embolism and/or pulmonary thromboembolism) were registered and referred to as major adverse cardiovascular events (MACEs). Results Recruited for the study were 47 children (mean age: 11.1± 5; age range: 0–18 years). Twenty-three patients fulfilled the cardiovascular magnetic resonance imaging diagnostic criteria for LVNC (LVNC group), while the remaining 24 did not and were included in the LV hypertrabeculation group (LVHT). They were followed for 4.9 ± 1.0 years, and MACEs were registered. Thirteen children (56% of LVNC, 28% of total) had at least one MACE. Global longitudinal, circumferential and radial strains (GLS, GCS, GRS), LV twist and LVEF resulted being significantly reduced in children with MACEs at follow-up. A multivariable analysis was performed by combining four parameters: LV ejection fraction, GLS, GCS, LV twist. These independent variables were chosen according to univariable analyses and clinical relevance. The results from the analysis demonstrated that LV twist was the only independent predictor (P = 0.033, coeff. B 0.726) of worse clinical outcomes in young patients with LVNC. Conclusions LV twist is a promising tool to stratify and predict prognosis in LVNC young patients. Our findings show the importance of LV twist assessment to detect the severity of LVNC and to plan for early clinical intervention.

Percutaneous closure of PDA in premature babies can be achieved with a high degree of success as an alternative to surgical ligation. Early and mid-term follow-up confirms promising results. Future efforts should be aimed at optimizing patient selection and timing of the procedure. Further studies are needed to assess the result and outcome of this technique versus current treatment strategies including medical treatment.

Funding Acknowledgements Type of funding sources: None. BACKGROUND Myocardial work (MW) estimation by pressure-strain loops (PSL) allows a non-invasive evaluation of myocardial performance, as recently demonstrated in adult patients. Aim of this study is to provide the reference values for global myocardial work index (MWI), constructive work (MCW), wasted work (MWW), and work efficiency (MWE) in a group of healthy children. METHODS Assessment of MW was performed using a commercially available software package (Echopac, GE). MW was measured from PSLs areas, derived from non-invasive LVP curves combined with strain acquired speckle tracking echocardiography (STE). After calculating GLS, values of brachial blood pressure were inserted and the time of valvular events by echocardiography were indicated, then the software was able to measure non-invasive PSLs. RESULTS Two-dimensional (2D) standard and speckle-tracking echocardiography were performed in 90 healthy children (mean age 9.9 ±4.9 [1-17] years, females: 57%) together with the assessment of MW by means of PSLs. Mean ± standard deviation, 5° and 95° percentile values for global MWI, MCW, MWW, and MWE in the whole population were 1769 ± 254 mm Hg, (1354-2193); 2201 ± 290 mm Hg, (1657-2658); 78 ± 47 (29-163) mm Hg%; 96 ± 1.8 (92-99)%, respectively. CONCLUSIONS The assessment of MW is feasible in healthy children. This study provides useful 2-dimensional echocardiographic reference ranges for novel indices of non-invasive MW.

OBJECTIVES: To describe presentation, hospital course, and predictors of bad outcome in multisystem inflammatory syndrome in children (MIS-C). METHODS: Retrospective data review of a case series of children meeting the published definition for MIS-C who were discharged or died between March 1, 2020, and June 15, 2020, from 33 participating European, Asian, and American hospitals. Data were collected through a Web-based survey and included clinical, laboratory, electrocardiographic, and echocardiographic findings and treatment management. RESULTS: We included 183 patients with MIS-C: male sex, 109 (59.6%); mean age 7.0 ± 4.7 years; Black race, 56 (30.6%); obesity, 48 (26.2%). Overall, 114 of 183 (62.3%) had evidence of severe acute respiratory syndrome coronavirus 2 infection. All presented with fever, 117 of 183 (63.9%) with gastrointestinal symptoms, and 79 of 183 (43.2%) with shock, which was associated with Black race, higher inflammation, and imaging abnormalities. Twenty-seven patients (14.7%) fulfilled criteria for Kawasaki disease. These patients were younger and had no shock and fewer gastrointestinal, cardiorespiratory, and neurologic symptoms. The remaining 77 patients (49.3%) had mainly fever and inflammation. Inotropic support, mechanical ventilation, and extracorporeal membrane oxygenation were indicated in 72 (39.3%), 43 (23.5%), and 4 (2.2%) patients, respectively. A shorter duration of symptoms before admission was found to be associated with poor patient outcome and for extracorporeal membrane oxygenation and/or death, with 72.3% (95% confidence interval: 0.56–0.90; P = .006) increased risk per day reduction and 63.3% (95% confidence interval: 0.47–0.82; P < .0001) increased risk per day reduction respectively. CONCLUSIONS: In this case series, children with MIS-C presented with a wide clinical spectrum, including Kawasaki disease–like, life-threatening shock and milder forms with mainly fever and inflammation. A shorter duration of symptoms before admission was associated with a worse outcome.

Introduction: The paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) and Kawasaki disease (KD) have overlapping features. This study aimed to describe the strain segmental analysis among both entities. Methods: Retrospective review of strain segmental analysis within 4 weeks of presentation of symptoms among children diagnosed with PIMS-TS between April and June 2020 and a historic cohort of typical KD from the Royal Brompton Hospital, London. Results: We included 33 PIMS-TS patients (23 males, 69.7%) at a mean age of 8 ± 4,9 years old and 45 KD patients (31 males, 68,9%) at a mean age of 5,8 ± 4,5 years old. PIMS-TS patients were older at presentation (p = 0.038). Left ventricle ejection fraction (LVEF) was normal in both groups (63,3% vs 63,5%; p= 0,89), 4/33 PIMS-TS children (12,1%) had coronary arteries abnormalities (CAA), whereas 100% of KD cohort had CAA. Both groups had a normal global longitudinal strain (GLS), but in PIMS-TS it was significantly reduced compared to the KD group (-20% vs -22%; p=0,008). Basal segments were the most affected in PIMS-TS with significant difference in the basal anterior and anterolateral strain compared to KD (respectively -18,2% vs -23,4%; p < 0,001 and - 16,7% vs -22,7%; p < 0,001). PIMS-TS had a greater anterior, anterolateral and posterior segments involvement with a significant reduction in the anterolateral mid-wall longitudinal strain (-18,3% vs -22%; p=0,002). Apical segments were less involved, with a significant difference only in the septal and inferior apical strain (respectively p=0.001 and p=0,032). Conclusions: These preliminary data showed that after 4 weeks from the onset of symptoms, all PIMS-TS patients had a normal LVEF but they had a significant reduction in GLS and different segmental involvement compared to KD cohort. We hypothesize that these findings may be related to direct myocardial damage in PIMS-TS rather than caused by coronaries perfusion abnormalities.