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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 (

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.

Introduction: Following the SARS-CoV-2 pandemic peak, children suffering from a multiorgan inflammatory disease that often leads to shock have been reported. This condition shares features with Kawasaki disease, but its etiopathogenesis is unknown. Hypothesis: We aimed to describe presentation and hospital course for this pediatric inflammatory multisystemic syndrome associated with COVID-19 (PIMS-TS). Methods: Data were collected from a retrospective review of children from 33 participating European, Asian and American sites. Results: We included 183 patients (109 males, 59·6%) with PIMS-TS, at a mean age of 7·0 (±4·7) years. Fifty-six (30·6%) had black ethnicity and obesity was present in 48 (26·2%) cases. Overall, 114/183 (62·3%) had biological evidence of current or recent SARS-CoV-2 infection. At admission, all presented with fever, 117/183 (63·9%) with gastrointestinal symptoms and 79/183 (43·2%) with shock, that was associated with more frequent black ethnicity, higher inflammatory markers and more cardiac involvement. Twenty-seven patients (14·7%) fulfilled criteria for Kawasaki disease. They were younger with no shock, fewer gastrointestinal, cardio-respiratory and neurological symptoms. Among the remaining PIMS-TS patients, 77 (49·3%) had mainly fever and inflammation with less cardiac involvement. For the entire cohort of 183 patients, the mean duration of admission was 8·6 (±5·6) days. Inotropic support, mechanical ventilation and ECMO were indicated in 72 (39·3%), 43 (23·5%) and 4 (2·2%) patients, respectively. Three patients (1·6%) died. A shorter duration of symptoms before admission was a risk factor for worse outcome and for ECMO/death, with 63% increased risk per day reduction (95%CI 0·39-0·93, p=0·03) and with 51·4% increased risk per day reduction (95%CI 0·36-0·9, p=0·03), respectively. Conclusions: We describe the first largest international series of children with PIMS-TS. Life-threatening shock is a common presentation. A shorter duration of symptoms prior to admission characterizes the fulminant form of the disease with potentially worse outcome.

Introduction: Children with paediatric inflammatory multisystem syndrome temporally associated with SARS CoV2 infection (PIMS-TS) present with evidence of multiorgan dysfunction. Cardiac features include partial or typical Kawasaki disease symptoms or ventricular impairment. The aim of this study was to assess short term sequelae of cardiac involvement in patients with PIMS-TS. Methods: 17 consecutive paediatric patients with PIMS-TS were referred for CMR at our institution. The referring criteria were: Echocardiographic finding of LV dysfunction at presentation (53%), dilated coronary arteries (27%) and high cardiac markers without LV dysfunction (20%). 16 patients completed the scan and were included into the analysis. Results: Age of the patients was 17 months-12 years (mean 6.4 years), 12 (80%) were male. Six (40%) patients required general anaesthesia. The time between the onset of the symptoms and the scan was 12-72 days. All patients had SARS CoV2 PCR negative at the time of scan, 2 patients were previously positive and 12 (80%) had positive IgG. CMR indexed volumes (ml/m2) were in normal range: LVEDV 65±11, LVESV 24±7, RVEDV 67±12, RVESV 27±7. LV mass index were normal 45±8 gr/m2. No overt areas of myocardial oedema or acute inflammation on STIR (short tau inversion recovery) images were found. Mildly reduced LV ejection fraction was found in 1 patient with subtle linear late gadolinium enhancement (LGE) in the midventricular midmyocardial interventricular septum with negative STIR images. Another patient was found to have LGE of the proximal parts of mitral valve papillary muscles, but no abnormalities in the LV ejection fraction or mitral valve function were found. Two patients had a small pericardial effusion. 3 patients had dilated neck and/or innominate veins, 2 of them with phlebectatic appearance. One patient had enlarged liver, another liver and spleen. Conclusion: in conclusion no significant myocardial sequelae have been found in most of the patients presenting with PIMS-TS short-term after onset of symptoms. This might indicate rapid recovery of the cardiac inflammation in most of the patients. More follow up is needed to clarify if there will be any potential sequelae from the acute episode as well as the outcome of phlebectasia.

Background: PIMS-TS is heterogeneous in presentation, with significant proportion showing cardiac involvement. Data on cardiac outcomes is sparse and no consensus yet exists on follow-up management. Aim: To describe clinical and cardiac features during and soon after the acute phase of PIMS-TS patients. Methods: Prospective analysis and comparison of biochemical, electrocardiographic (ECG) and echocardiographic (TTE) data obtained during admission and on follow-up within 2 weeks of discharge. Cardiac MRI (CMR) was done mainly in those with myocardial hit (high Troponin, ECG changes or left ventricular dysfunction on TTE if available) during admission. Results: 35 patients met criteria for PIMS-TS, (23 males, 66%) with a mean age 5 years. 49% were of Black, Asian or Minority Ethnicity (BAME). 75% of tested were IgG positive. Mean interval from symptom onset to admission was 5 days (range 1-12 days). Features included fever (100%), conjuctivitis (66%) oral mucositis/pharyngitis (60%), gastrointestinal symptoms (54%), rash (51%), erythema/oedema of extremities (31%) and cervical lymphadenopathy (31%). Presentation grouped into 3 types - 7/35 (20%) met AHA criteria for Kawasaki Disease (KD), 5 (14%) presented with shock and 23 (66%) had atypical presentations. 1 patient was treated for KD 6 years ago, no other co-morbidities noted in the cohort. Six (17%) needed PICU support (mean 1.8 days, range 1-4). 80% had normal ECGs during admission but abnormal repolarisation (11%), ischaemic changes (3%), arrhythmia (9%) were seen. 26% (9/35) had left ventricular (LV) dysfunction, of whom 7 had high serum Troponin (mean 183ng/mL SD 250) and Brain Natriuretic Peptide (BNP, mean 627ng/L SD 287). Coronary abnormalities were noted in 12 (34%). All blood markers (Troponin, BNP, Platelet count, C-reactive protein, D-dimer, Ferritin) returned to normal levels by discharge/follow-up. Follow-up global longitudinal strain (GLS) was normal in 94% (33/35). 5 (14%) had mild coronary ectasia. Of 16 CMR’s done Conclusions: Most biochemical and cardiac derangement during the acute phase normalised within weeks. Further study is on-going.

Background During the severe acute respiratory syndrome Coronavirus 2 pandemic, there has been an increase in hyperinflammatory presentation in previously healthy children with a variety of cardiac manifestations. Our objective is to describe the cardiac manifestations found in an international cohort of 55 pediatric cases with multi‐system inflammatory syndrome during the severe acute respiratory syndrome Coronavirus 2 pandemic. Methods and Results We reviewed data on previously healthy pediatric patients (≤18 years) with structurally normal hearts who presented at hospitals in the United States, United Kingdom, Spain, and Pakistan with multi‐system inflammatory syndrome in children and had consultation with a pediatric cardiologist. Data collected included demographics, clinical presentation, laboratory values, electrocardiographic abnormalities, echocardiographic findings, and initial therapies. A total of 55 patients presented with multi‐system inflammatory syndrome in children. Thirty‐five patients (64%) had evidence of decreased left ventricular function, 17 (31%) had valvulitis, 12 (22%) with pericardial effusion, and 11 (20%) with coronary abnormalities. Twenty‐seven (49%) required intensive care unit admission and 24 (44%) had evidence of shock. Eleven patients (20%) fulfilled complete Kawasaki disease criteria and had lower NT‐proBNP (N‐terminal pro‐B‐type natriuretic peptide), D‐dimer, and ferritin levels compared with those who did not fulfill criteria. Electrophysiologic abnormalities occurred in 6 patients and included complete atrioventricular block, transient atrioventricular block, and ventricular tachycardia. Conclusions To our knowledge, we describe the first international cohort of pediatric patients with multi‐system inflammatory syndrome in children during the severe acute respiratory syndrome Coronavirus 2 pandemic with a range of cardiac manifestations. This article brings awareness and alertness to the global medical community to recognize these children during the pandemic and understand the need for early cardiology evaluation and follow‐up.

Background Severe pulmonary regurgitation (PR) and progressive right ventricular (RV) dilation and disfunction are common in patients with repaired Tetralogy of Fallot (r-TOF) and should be carefully monitored during the follow up of these patients. In this contest, Echocardiography and Cardiac Magnetic Resonance (CMR) have a complementary diagnostic role. Purpose To correlate Echo and CMR parameters in children ( Methods Paediatric patients with r- TOF with at least moderate PR at the echo evaluation who underwent a CMR study within six months were included by using hospital databases. 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), PR index, ratio of diastolic and systolic time-velocity integrals (DSTVI) of the main pulmonary artery. By speckle tracking we measured also RV global longitudinal strain (RVGLS) and right atrial strain (RAS). All the patients underwent CMR to assess PR and right ventricular volumes and ejection fraction (EF). Of these, 36 patients underwent cardiopulmonary exercise test (CPET). Results Fourty-six children (aged 13.7±3.0 years) were included. Echo derived RV areas correlated significantly with CMR RV volumes (r=0.72, p21.9 cm2/m2 had a good sensitivity (83.3%) and specificity (73.5%) to identify a RV end-diastolic volume (RVEDV) ≥150 ml/m2. No correlation was found among TAPSE, FAC, RVGLS and RVEF calculated by CMR nor between PHT, PR index and DSTVI and PR-RF. Only A' wave velocity showed a significant but modest correlation with CMR RF (r=0.57, p Conclusion In children, flow reversal in pulmonary branches identifies hemodynamically significant PR at CMR. RV area by echocardiogram is a valid first-line parameter to screen RV dilation. Our study suggests that, also for the RV, there is longitudinal systolic dysfunction in presence of preserved RV EF. RAS is the best predictor of peak Vo2 and should be added in the follow up of these patients. Funding Acknowledgement Type of funding source: None

Background Norwood procedure represents the first of three surgical steps toward Hypoplastic left heart syndrome (HLHS) complete palliation. Two are the main surgical techniques allowing the reconstruction of Norwood circulation: the right ventricle-pulmonary artery shunt (RVPAS) and the modified Blalock-Taussing shunt (mBTS). However, still little is known about the impact on right ventricle (RV) function of the required ventriculotomy for the RVPAS. Purpose The aim of the study was to investigate the changes in RV function in HLHS patients after RVPAS vs mBTS. Methods The cohort included 27 consecutive HLHS patients (10 in the modified Blalock-Taussig shunt group and 17 in the RVPAS group) who successfully underwent Norwood procedure in a single tertiary paediatric cardiology centre. Longitudinal strain (LS) and strain rate (LSR), tricuspid annulus peak systolic excursion (TAPSE) and fractional area change (FAC) were evaluated in all patients before Norwood and in three different breakpoints in the steady state after Norwood procedure (30 days after Norwood, 90 days after Norwood, 140 days after Norwood). Results Ventricular loading conditions (diuretic treatment, blood pressure and tricuspid regurgitation) were similar in both groups. No significant differences were found at different time points between RVPAS and mBTS group in terms of LS, LSR, TAPSE and FAC. However, when we compared RV function before and after Norwood procedure, 90 days after the procedure, patients who did not undergo RV ventriculotomy (mBTS group) showed significant improvement in LS compared to pre-surgical assessment (mBTS: +27.35±43.47% vs RVPAS: −8,20±25.25%, p=0,03). This finding was consistent but no longer statistically significant at 140 days after Norwood (mBTS: +13.81±21.99% vs RVPAS: −4.90±27.97%, p=0,12). Conclusion After Norwood procedure mBTS patients showed a significant increase in LS when compared with patients who underwent RVPAS. This finding was consistent but no longer significant at 140 days after Norwood probably because the number of patients was too small to reach a significant level. These data support the use of LS in HLHS patient's evaluation and may be of value to find a patient-tailored timing for the second surgical stage. TAPSE, FAC, LS and LSR trends Funding Acknowledgement Type of funding source: None

Coronavirus disease 2019 (COVID-19) has affected patients across all age groups, with a wide range of illness severity from asymptomatic carriers to severe multi-organ dysfunction and death. Although early reports have shown that younger age groups experience less severe disease than older adults, our understanding of this phenomenon is in continuous evolution. Recently, a severe multisystem inflammatory syndrome in children (MIS-C), with active or recent COVID-19 infection, has been increasingly reported. Children with MIS-C may demonstrate signs and symptoms of Kawasaki disease, but also have some distinct differences. These children have more frequent and severe gastrointestinal symptoms and are more likely to present with a shock-like presentation. Moreover, they often present with cardiovascular involvement including myocardial dysfunction, valvulitis, and coronary artery dilation or aneurysms. Here, we present a review of the literature and summary of our current understanding of cardiovascular involvement in children with COVID-19 or MIS-C and identifying the role of a pediatric cardiologist in caring for these patients.

Persistent patent ductus arteriosus (PDA) is very common in preterm infants, especially in extremely preterm infants. Despite significant advances in management of these vulnerable infants, there has been no consensus on management of PDA—when should we treat, who should we treat, how should we treat and in fact there is no agreement on how we should define a hemodynamically significant PDA. Medical management with non-steroidal anti-inflammatory drugs (NSAIDs) remains the first line of therapy with moderate success rate in closing the PDA. Paracetamol has been reported to be a safe and equally effective medical therapy for closure of PDA. However, additional studies on its long-term safety and efficacy in extremely low birth weight infants are needed before paracetamol can be recommended as standard treatment for a PDA in preterm infants. Surgical ligation of PDA is not without an increased risk of mortality and co-morbidities. Recently, there has been a significant interest in percutaneous transcatheter closure of PDA in preterm infants, including extremely low birth weight infants. Transcatheter PDA closure in preterm ELBW infants is technically feasible with high PDA occlusion success rates and acceptable complication rates as compared to surgical ligation. Many centers have reported promising early- and mid-term follow-up results. However, they need to be further tested in the prospective well-designed studies and randomized controlled trials comparing the results and outcomes of this technique with current treatment strategies including medical treatment before they can be used as the new standard of care for PDA closure in extremely low birth weight infants.

Background Balloon dilation and stenting of the atrial septum are techniques used to unload left heart cavities in acute or end-stage heart failure in children. However, they carry significant risks such as tamponade or device embolization. Case Presentation We report the first case of a child with an end-stage mitochondrial cardiomyopathy on a venoarterial extracorporeal membrane oxygenator as a bridge to heart transplant where an atrial flow regulator device has been implanted. Conclusions This case illustrates the feasibility and safety of atrial flow regulator implantation in this setting. This procedure allowed to wean inotropic support while awaiting heart transplantation.

Summary Background Cardiac catheterization is the gold-standard modality for investigation of cardiovascular morphology before bidirectional cavopulmonary connection, but requires general anaesthesia and is associated with procedural risk. Aims To assess the diagnostic accuracy and safety of computed tomography in diagnosing great vessel stenosis/hypoplasia compared with cardiac catheterization and surgical findings. Methods Twenty-seven patients (10 after Norwood stage I) underwent computed tomography before surgery between January 2010 and June 2016; 16 of these patients also underwent cardiac catheterization. Proximal and distal pulmonary artery, aortic isthmus and descending aorta measurements, radiation dose and complications were compared via Bland-Altman analyses and correlation coefficients. Results The accuracy of computed tomography in detecting stenosis/hypoplasia of either pulmonary artery was 96.1% compared with surgical findings. For absolute vessel measurements and Z-scores, there was high correlation between computed tomography and angiography at catheterization (r = 0.98 for both) and a low mean bias (0.71 mm and 0.48; respectively). The magnitude of intertechnique differences observed for individual patients was low (95% of the values ranged between −0.9 and 2.3 mm and between −0.7 and 1.7, respectively). Four patients (25%) experienced minor complications from cardiac catheterization, whereas there were no complications from computed tomography. Patients tended to receive a higher radiation dose with cardiac catheterization than with computed tomography, even after exclusion of interventional catheterization procedures (median 2.5 mSv [interquartile range 1.3 to 3.4 mSv] versus median 1.3 mSv [interquartile range 0.9 to 2.6 mSv], respectively; P = 0.13). All computed tomography scans were performed without sedation. Conclusions Computed tomography may replace cardiac catheterization in identification of great vessel stenosis/hypoplasia before bidirectional cavopulmonary connection when no intervention before surgery is required. Computed tomography carries lower morbidity, can be performed without sedation and may be associated with less radiation.

International audience; Aims: In univentricular hearts, selective lung vasodilators such as phosphodiesterase type 5 (PDE5) inhibitors would decrease pulmonary resistance and improve exercise tolerance. However, the level of evidence for the use of PDE5 inhibitors in patients with a single ventricle (SV) remains limited. We present the SV-INHIBITION study rationale, design, and methods.Methods and results: The SV-INHIBITION trial is a nationwide multicentre, randomized, double blind, placebo-controlled, Phase III study, aiming to evaluate the efficacy of sildenafil on the ventilatory efficiency during exercise, in teenagers and adult patients (>15 years old) with an SV. Patients with a mean pulmonary arterial pressure >15 mmHg and a trans-pulmonary gradient >5 mmHg, measured by cardiac catheterization, will be eligible. The primary outcome is the variation of the VE/VCO2 slope, measured by a cardiopulmonary exercise test, between baseline and 6 months of treatment. A total of 50 patients are required to observe a decrease of 5 ± 5 points in the VE/VCO2 slope, with a power of 90% and an alpha risk of 5%. The secondary outcomes are clinical outcomes, oxygen saturation, 6 min walk test, SV function, NT-proBNP, peak VO2 , stroke volume, mean pulmonary arterial pressure, trans-pulmonary gradient, SF36 quality of life score, safety, and acceptability.Conclusions: The SV-INHIBITION study aims to answer the question whether PDE5 inhibitors should be prescribed in patients with an SV. This trial has been built focusing on the three levels of research defined by the World Health Organization: disability (exercise tolerance), deficit (SV function), and handicap (quality of life).Trial registration: ClinicalTrials.gov NCT03997097.

Background Pulmonary regurgitation (PR) and right ventricular (RV) dilatation and disfunction are common in patients with repaired Tetralogy of Fallot (r-TOF). Aims To compare Echo data with the gold standard CMR in a paediatric population of r-TOF with significant PR, to assess the reliability of standard and advanced echo parameters. In addition, to evaluate their correlation with peak oxygen consumption (VO2). Methods and results All patients underwent standard echo-Doppler study, speckle tracking analysis, and CMR to assess PR and RV size and function. Thirty-six patients underwent also cardiopulmonary exercise test. Fourty-six patients (aged 13.7 ± 3.0) were included. Echo derived RV areas correlated with CMR RV volumes (p 21.9 cm2/m2 had a good sensitivity (83.3%) and specificity (73.5%) to identify a RV end-diastolic volume ≥ 150 ml/m2. RVEF was preserved in all patients, while TAPSE was reduced in 78.2% and RVGLS in 60.8%. Flow-reversal in pulmonary branches showed a sensitivity of 95.8% and a specificity of 59.1% to identify CMR pulmonary regurgitant fraction (RF) ≥ 35%. None of the CMR parameters correlated with peak VO2. Among the Echo data only right atrial strain (RAS) correlated with peak VO2. Conclusion In children, flow-reversal in pulmonary branches identifies hemodynamically significant RF with a good sensitivity but poor specificity. RV area by echocardiogram is a valid first-line parameter to screen RV dilation. RV longitudinal systolic dysfunction coexists with a still preserved EF. RAS correlates strongly with peak VO2 and should be added in their follow up.

Measures of left ventricular (LV) systolic and diastolic function are known predictors of mortality after repair of tetralogy of Fallot. We aimed to characterise LV reverse remodelling achievable with ramipril therapy.A blinded post-hoc analysis of baseline and 6-month follow-up echocardiograms from the APPROPRIATE (ISRCTN: 97515585) randomised double-blinded placebo-controlled trial of ramipril therapy was performed in 64 patients: 32 in ramipril and 32 in placebo group. Tissue Doppler systolic and diastolic myocardial velocities, mitral inflow velocities and time intervals were measured. Left atrial area and left atrial emptying fraction were calculated. There was significant increase in long axis shortening mean (standard deviation); MAPSE [1.9 (4.2) mm vs -0.2 (3.7) mm; p = 0.030], peak lateral systolic velocity; S' lateral [1.0 (2.0) cm/s vs -0.3 (2.2) cm/s; p = 0.025], peak lateral early diastolic velocity; E' lateral [0.57 (2.4) cm/s vs -3.3 (3.9) cm/s; p 0.001], transmitral to lateral mitral annular early diastolic velocity ratio; E/E' lateral [-0.7 (1.9) vs 1.5 (1.9); p 0.001] over the study period in the ramipril compared to the placebo group. Significantly higher measurements were observed in the ramipril arm of the subgroup of patients with right ventricular restrictive physiology in terms of peak late diastolic velocity; A [5.9 (13.5) cm/s vs -5.8 (12.5) cm/s; p = 0.041] and early to late diastolic transmitral velocity ratio; E/A [-0.18 (0.42) vs 0.23 (0.48); p = 0.037].Six months' ramipril treatment appears to limit progression of both diastolic and systolic LV function in adults late after tetralogy of Fallot repair. With increased appreciation that even subtle LV disease predicts tetralogy of Fallot outcomes, further clinical trials of drug therapies are justified.

Objectives This study sought to assess procedural characteristics, early clinical outcome, and long-term complications after transcatheter closure of atrial septal defect (ASD) in children. Background Transcatheter closure has become the preferred strategy in most cases of isolated secundum ASD. However, reported experience in the pediatric population is limited. Methods A 1998 to 2016 retrospective multicenter study was performed in 9 French tertiary institutions. All children who had an attempt of percutaneous ASD closure with an Amplatzer Septal Occluder were included. Results In 1,326 children (39% males; median age, 9 years [0.7 to 18]; weight, 29 kg [3.6 to 92]), transcatheter ASD closure was performed. Median ASD size was 15 mm (3 to 41); 254 (19.1%) patients had a large ASD (≥20 mm/m2). Procedural success rate was 95.3% (95% confidence interval: 93.9% to 96.3%). No death was observed but periprocedural complications occurred in 24 patients (1.8%). After a median follow-up of 3.5 years (range 6 months to 18 years; 173 patients [13%] followed >10 years), delayed major complications were minimal (n = 12; 1.04%) including no death and/or cardiac erosion. Periprocedural and delayed complications rates were significantly higher in children ≤15 kg (5.2% vs. 1.5%; p = 0.007 and 3.1% vs. 0.7%; p Conclusions Transcatheter ASD closure using Amplatzer Septal Occluder is safe in children with a minimal rate of periprocedural complications and a favorable long-term outcome, especially with no death or cardiac erosion despite a substantial proportion of large defects. Children ≤15 kg and those with large ASDs had a greater risk of complications.

ObjectivesInfants with symptomatic partial and transitional atrioventricular septal defect undergoing early surgical repair are thought to be at greater risk. However, the outcome and risk profile of this cohort of patients are poorly defined. The aim of this study was to investigate the outcome of symptomatic infants undergoing early repair and to identify risk factors which may predict mortality and reoperation.MethodsThis multicentre study recruited 51 patients (24 female) in three tertiary centres between 2000 and 2015. The inclusion criteria were as follows: (1) partial and transitional atrioventricular septal defect, (2) heart failure unresponsive to treatment, (3) biventricular repair during the first year of life.ResultsMedian age at definitive surgery was 179 (range 0–357) days. Sixteen patients (31%) had unfavourable anatomy of the left atrioventricular valve: dysplastic (n=7), double orifice (n=3), severely deficient valve leaflets (n=1), hypoplastic left atrioventricular orifice and/or mural leaflet (n=3), short/poorly defined chords (n=2). There were three inhospital deaths (5.9%) after primary repair. Eleven patients (22%) were reoperated at a median interval of 40 days (4 days to 5.1 years) for severe left atrioventricular valve regurgitation and/or stenosis. One patient required mechanical replacement of the left atrioventricular valve. After median follow-up of 3.8 years (0.1–11.4 years), all patients were in New York Heart Association (NYHA) class I. In multivariable analysis, unfavourable anatomy of the left atrioventricular valve was the only risk factor associated with left atrioventricular valve reoperation.ConclusionsAlthough surgical repair is successful in the majority of the cases, patients with partial and transitional atrioventricular septal defect undergoing surgical repair during infancy experience significant morbidity and mortality. The reoperation rate is high with unfavourable left atrioventricular valve anatomy.

Background: 2D-transesophageal echocardiography (TEE) is routinely performed to guide percutaneous ASD closure in children. We aimed to assess whether two-dimensional (2D)-transthoracic echocardiography (TTE) is a safe alternative for assessment and guidance of atrial septal defect (ASD) closure in unselected children. Methods: We performed a retrospective single-center study including 389 consecutive children aged less than 15-year-old who underwent percutaneous ASD closure under 2D-TEE (1998–2005, n=133) or 2D-TTE (2005–2014, n=256). A balloon calibration was performed in all cases for the Amplatz Septal Occluder choice. Results: ASDs were larger and rims deficiencies were more frequent in the TTE-guided group. The procedure was successful in 376 patients [96.7%; 95% confidence interval (CI), 94.4–98.2%]. The success rate tended to be higher in the TTE- versus TEE-guided group (98.0% versus 94.0%, P=0.069). Device migration occurred in 4 patients (1.0%; 95% CI: 0.3–1.6%), all after TEE-guided procedure (P=0.013). Early major adverse events were observed in 5 patients (1.3%; 95% CI: 0.4–3.0%), all in the TEE group (P=0.004). Fluroroscopic time and irradiation dose were not different among the 2 groups (P=0.450 and P=0.130 respectively). After a median follow-up of 7 years (range, 1–16 years), no adverse events was reported. One (0.3%, 95% CI: 0–1.4%) 12-year-old patient developed atrial fibrillation 5 years after the procedure. Pregnancies were uneventful in 72 cases. Conclusions: When a balloon sizing is performed, 2D-TTE imaging is as efficient as 2D-TEE to guide percutaneous ASD closure in children. The procedure can safely be done in spontaneously breathing children under TTE guidance alone in experienced centers.

Summary Background Transcatheter perimembranous ventricular septal defect (pmVSD) closure remains challenging and is seldom used in France given the risk of atrioventricular block (AVB). pmVSD closure with the Nit-Occlud Le VSD coil was recently introduced in France as an alternative to occluder devices. Aims To study the safety and feasibility of pmVSD closure with the Nit-Occlud Le VSD coil. Methods All consecutives cases of pmVSD closure with the Nit-Occlud Le VSD coil in 20 tertiary French centres were included between January 2015 and December 2018. Results Among 46 procedures in five centres, indications for pmVSD closure were left ventricle overload (76.1%), exertional dyspnoea (17.4%), history of infective endocarditis (4.3%) and mild pulmonary hypertension (2.2%). The median (interquartile [IQR]) age of the patients was 13.9 (5.7–31.8) years. Aneurismal tissue was identified in 91.3% of patients. VSD median (IQR) size was 8 (7–10) mm on the left ventricle side and 5 (4–6) mm on the right ventricle side. Implantation was successful in 40 patients (87.0%; 95% confidence interval [CI] 73.7–95.1%). Severe complications occurred in six patients (13.0%, 95% CI 4.9–26.3%), mainly severe haemolysis (8.7%, 95% CI 2.4–20.8%). One aortic valve lesion required surgical aortic valvuloplasty. Occurrence of severe complications was significantly related to the presence of haemolysis (P = 0.001), residual shunt (P = 0.007) and multi-exit VSD (P = 0.005). Residual shunt was observed in 40% of cases with the implanted device shortly after closure and 15% after a median follow-up of 27 months. No immediate or delayed device embolization or complete AVB was recorded. Conclusion pmVSD closure with the Nit-Occlud Le VSD Coil is feasible in older children and adults. However, residual shunting (leading to haemolysis) is a dreaded complication that should not be tolerated. pmVSD closure with the Nit-Occlud Le VSD as a therapeutic strategy remains controversial and is limited to selected patients.

Funding Acknowledgements EACVI Training Grant Introduction Kawasaki disease (KD) is an acute vasculitis of unknown etiology. It is associated with high morbidity and mortality due to the development of coronary artery aneurysms and myocardial dysfunction. Purpose The aim of this study was to evaluate sensitivity and specificity of left ventricular (LV) pressure–strain loop (PSL) area, which reflects regional myocardial work and metabolic demand, in predicting subtle myocardial abnormalities in KD patients with coronaries aneurisms. Methods A total of 88 patients (59 male, age 8.95 ± 4.95 years) were included in our study. Among the children admitted in our institution with a diagnosis of KD during the study time frame, 42 patients (KDg) (29 male) with coronary artery dilatation (Z-score >2.5) were selected. These cases were compared with 46 (30 male) age-matched controls (CTRg). Classical echocardiographic parameters of LV systolic function were normal in both groups, while global longitudinal strain (GLS) was decreased in 6 KD patients. Global work index (GWI) was calculated as the area of the LV PSL. From GWI, it was estimated also Global Constructive Work (GCW), Global Wasted Work (GWW) and Global Work Efficiency (GWE). We also made a subgroup analysis between KD patients with normal GLS (> -19 %) and control patients. Results Despite normal LV systolic function by routine echocardiography, compared to controls, KD patients had lower GWI (1448 ± 382 mmHg% in KDg vs 1751 ± 263] mmHg% in CTRg, p = 0.00003), GCW (1904 ± 390 mmHg% in KDg vs 2174 ± 292 mmHg% in CTRg, p= 0.0004) and GWE (94 ± 5 % in KDg vs 96 ± 2 % in CTRg, p= 0.01). There was not significant difference in GWW between the KDg and CTRg. When KD patients with normal GLS were analysed separately, they preserved a significant difference in GWI, GCW and GWE in comparison with controls (GWI: 1490 ± 347 mmHg% in KDg vs 1751 ± 263 mmHg% in CTRg, p = 0.0002; GCW: 1972 ± 321 mmHg% in KDg vs 2174 ± 292 mmHg% in CTRg, p = 0.004; GWE: 95 ± 3 in KDg vs 96 ± 2 % in CTRg, p= 0.04). No association was found between GWI, GCW, GWW, GWE and number or dimension of the involved aneurysmatic coronaries. Conclusions The estimation of myocardial work by PSL is a novel tool for the evaluation of patients with KD. GWI, GCW and GWE were significantly reduced in KD patients with dilated coronaries. In KD patients with normal GLS, estimation of GWI, GCW and GWE may be a sensitive indicator of myocardial dysfunction and an adjuvant criterion to avoid delayed diagnosis of KD. Abstract P316 Figure. Myocardial work in KD and CTR.

Introduction Hypoplastic Left Heart Syndrome (HLHS) has high mortality and morbidity and systemic right ventricle (RV) dysfunction may play a key-role. Study aim is to evaluate the accuracy of speckle-tracking echocardiographic (STE) assessment of RV deformation and 2D standard echo parameters in predicting outcome in HLHS patients. Methods We studied 27 HLHS patients (17 male) who successfully completed Norwood palliation. All the patients underwent in-hospital interstage stay. Serial echocardiographic assessment was performed: baseline, one-month after Norwood, three-months after Norwood, one-week before bidirectional cavopulmonary anastomosis (BCPA) and two-months after BCPA. From the apical view we measured: tricuspid annulus peak systolic excursion (TAPSE), fractional area change (FAC), longitudinal strain (LS) and strain rate (LSR). Results After a mean follow-up of 1.18 (± 1.16) years, 8 out of 27 of the included patients met the composite endpoint of death/heart transplant (HT). At pre-Norwood assessment, there was no difference in echo measurements between survivors and patients with events. In death/HT group TAPSE and LS declined already one-month after Norwood procedure: TAPSE ≤5 mm had good sensitivity (85.71%) and moderate specificity (63.16%) for death/HT (AUC = 0.767); a decrease of LS > 8.7% vs baseline showed 100% sensitivity and 84.21% specificity for death/HT (AUC = 0.910). At multivariate analysis, one-month-after-Norwood LS drop >8.7% was the best predictor of outcome (P = 0.01). Conclusions RV dysfunction in HLHS carries prognostic value. Our findings encourage serial measurements of RV function to identify the subgroup of HLHS patients at higher risk. In our experience, ∆ LS showed the best predictive value.

Funding Acknowledgements EACVI Training Grant Introduction Children with Hypoplastic Left Heart Syndrome (HLHS) have a high mortality (up to 95%) and morbidity. Systemic right ventricle (RV) dysfunction plays a key-role in their outcome. Purpose The aim of this study is to evaluate the accuracy of speckle-tracking echocardiographic (STE) assessment of RV deformation and 2D standard echo parameters in predicting death and need for heart transplantation (HT) in HLHS patients. Methods 31 patients with diagnosis of HLHS successfully completed Norwood (n = 29) or comprehensive Norwood stage II (n = 2) at our Institution between 2015 and 2019. Survival at 6 months was 90.32%, survival at 18 months was 85.72%. We studied 29 HLHS patients (17 male). Patients with HLHS variant (n = 2) were excluded. All the studied patients underwent in-hospital interstage stay. Serial echocardiographic assessment was performed in all the included patients (baseline, one month after Norwood, three months after Norwood, one week before bidirectional cavopulmonary anastomosis [BCPA] and two months after BCPA). From the apical view we measured: tricuspid annulus peak systolic excursion (TAPSE), fractional area change (FAC), longitudinal strain (LS) and strain rate (LSR). Results After a mean follow-up of 1.83 ± 1.16 years, 8 out of 29 of the included patients met the composite endpoint of death/HT. At pre-Norwood assessment, there was no statistical difference in echo measurements between survivors and patients who reached the endpoint. In death/HT group TAPSE and LS declined already at one-month after Norwood procedure evaluation. At one-month evaluation, a TAPSE ≤ 5 mm had a good sensitivity for death/HT (85.71%) and a moderate specificity (66.67%), with an area under the curve of 0.789. Always at one-month evaluation, a Δ LS ([{baseline LS – one-month post Norwood LS}/ baseline LS] *100) of 8.7% showed a 100% sensitivity and good specificity (80.95%) for death/HT, with an area under the curve of 0.888. Multivariate analysis showed that one-month-after-Norwood Δ LS was the best predictor of worse outcome (p = 0.02). Conclusions HLHS patients with Δ LS of > 8.7% at one-month after Norwood procedure had a high likelihood of death or HT. These data encourage the routine use of LS to monitor cardiac function in HLHS patients. Abstract 1233 Figure. HLHS LS and trend of TAPSE and LS.

Persistent Patent ductus arteriosus (PDA) is a common finding in extremely low gestational age newborn infants and its prevalence is inversely proportional to the gestational age. The presence of a persistent PDA is associated with increased mortality and several significant morbidities including intraventricular hemorrhage, pulmonary hemorrhage, necrotizing enterocolitis, and chronic lung disease or bronchopulmonary dysplasia. However, treating PDA has not been demonstrated to have beneficial impact on the long term outcomes. Currently there is no consensus on whether to treat the PDA or not, and if treat, when to treat and how to treat. The echocardiography is the investigation of choice to diagnose PDA, estimating the magnitude of shunt volume and assessing its hemodynamic significance, and to exclude/diagnose any associated congenital heart defect before any intervention. Various echocardiographic parameters and staging/scoring systems have been described to help the clincians making the clinical decisions and some of theses scoring systems are quite complex to apply in a busy day to day clinical practice. This concised review paper is focused to help the clinicians in making a clinical decision based upon clincial and echocardiography parameters. Hence, only the parameters which are commonly used and helpful in making the clinical decisions in day to day clincial practice have been described in this paper.

In a recent issue of the Journal, Stankowski et al. (1) report their single centre experience of 7 years of patent ductus arteriosus ligation using video-assisted thoracoscopy (VATS) in paediatric population. They studied 127 patients between 2012 and 18, including 38.6% preterm infants.

Background Pulmonary vascular resistance (PVR) is an essential parameter assessed during cardiac catheterization. It is used to confirm pulmonary vascular disease, to assess response to targeted pulmonary hypertension (PH) therapy and to determine the possibility of surgery, such as closure of intra-cardiac shunt or transplantation. While PVR is believed to mainly reflect the properties of the pulmonary vasculature, it is also related to blood viscosity (BV). Objectives We aimed to assess the relationship between measured (mPVR) and viscosity-corrected PVR (cPVR) and its impact on clinical decision-making. Methods We assessed consecutive PH patients undergoing cardiac catheterization. BV was assessed using the Hutton method. Results We included 465 patients (56.6% female, median age 63y). The difference between mPVR and cPVR was highest in patients with abnormal Hb levels (anemic patients: 5.6 [3.4–8.0] vs 7.8Wood Units (WU) [5.1–11.9], P2.0WU) difference between mPVR and cPVR, and this was more pronounced in those with anemia (52.9%) or raised Hb (77.6%). In patients in the upper quartile for this difference, mPVR and cPVR differed by 4.0WU [3.4–5.2]. Adjustment of PVR required Conclusions We report, herewith, a clinically significant difference between mPVR and cPVR in a third of contemporary patients assessed for PH. This difference is most pronounced in patients with anemia, in whom mPVR significantly underestimates PVR, whereas in most patients with raised Hb, mPVR overestimates it. Our data suggest that routine adjustment for BV is necessary.

Aims Patent ductus arteriosus (PDA) is common in preterm infants and can contribute to morbidity and mortality. We aimed to compare results and outcome of transcatheter closure using the Amplatzer Piccolo Occluder versus surgical closure in 2 matched groups of preterm infants weighing Methods and results A total of 147 babies from three tertiary centres were retrospectively analysed. Sixty-four babies undergoing catheter closure were compared with 83 matched surgical cases. Patent ductus arteriosus closure was successful in all cases. During neonatal unit course, mortality was 6.3% (n = 4) after catheterization and 12% (n = 10) after surgery (p = 0.24). Median duration of mechanical ventilation was shorter after catheterisation than after surgery (3 vs 5 days, p = 0.035). Before 4 weeks of age the difference between transcatheter and surgical closure for mechanical ventilation was even more pronounced (3 vs 9 days, p = 0.022). Additionally, when catheterisation was performed before 4 weeks, babies were discharged home earlier as compared to those who underwent closure later in life (39+1 vs. 42+1 weeks, p = 0.021). Such difference was not found in the surgical group. Conclusions Transcatheter closure of patent ductus arteriosus is safe, effective and is associated with shorter mechanical ventilation than after surgery. Hospital stay might be shorter when performed earlier in life.

OBJECTIVES The interstage mortality rate after a Norwood stage 1 operation remains 12–20% in current series. In-hospital interstage facilitates escalation of care, possibly improving outcome. METHODS A retrospective study was designed for hypoplastic left heart syndrome (HLHS) and HLHS variants, offering an in-hospital stay after the Norwood operation until the completion of stage 2. Daily and weekly examinations were conducted systematically, including two-dimensional and speckle-tracking echocardiography. Primary end points included aggregate survival until the completion of stage 2 and interstage freedom from escalation of care. Moreover, we calculated the sensitivity and specificity of speckle-tracking echocardiographic myocardial deformation in predicting death/transplant after the Norwood procedure. RESULTS Between 2015 and 2019, 33 neonates with HLHS (24) or HLHS variants (9) underwent Norwood stage 1 (31) or hybrid palliation followed by a comprehensive stage 2 operation (2). Stage 1 Norwood–Sano was preferred in 18 (54.5%) neonates; the classic Norwood with Blalock–Taussig shunt was performed in 13 (39.4%) neonates. The Norwood stage 1 30-day mortality rate was 6.2%. The in-hospital interstage strategy was implemented after Norwood stage 1 with a 3.4% interstage mortality rate. The aggregate Norwood stage 1 and interstage Kaplan–Meier survival rate was 90.6 ± 5.2%. Escalation of care was necessary for 5 (17.2%) patients at 2.5 ± 1.2 months during the interstage for compromising atrial arrhythmias (2), Sano-shunt stenosis (1) and pneumonia requiring a high-frequency oscillator (2); there were no deaths. A bidirectional Glenn (25) or a comprehensive-Norwood stage 2 (2) was completed in 27 patients at 4.7 ± 1.2 months with a 92.6% survival rate. The overall Kaplan–Meier survival rate is 80.9 ± 7.0% at 4.3 years (mean 25.3 ± 15.7 months). An 8.7% Δ longitudinal strain 30 days after Norwood stage 1 had 100% sensitivity and 81% specificity for death/transplant. CONCLUSIONS In-hospital interstage facilitates escalation of care, which seems efficacious in reducing interstage Norwood deaths. A significant reduction of longitudinal strain after Norwood stage 1 is a strong predictor of poor outcome.

Endocarditis is life threatening after percutaneous pulmonary valve implantation. We report a patient with acute heart failure caused by obstructive pulmonary vegetation in a Melody valve. Because of severe right ventricular dysfunction, immediate cardiac surgery was contraindicated. The patient underwent trans-catheter implantation of a covered stent to relieve obstruction. Following an initially good outcome, surgery was performed 2 weeks later. This was complicated by left main coronary artery tear and fatal bleeding. Despite covered stent was an effective bridge, surgery remains extremely challenging in such cases with important surrounding inflammation and fibrosis as well as potential proximity of coronary arteries.

Pulmonary vascular resistance (PVR) is an essential parameter assessed during cardiac catheterization. It is used to assess response to targeted pulmonary hypertension (PH) therapy; determine the possibility of surgery, such as closure of intracardiac shunt or heart transplantation; and according to

Background: Transcatheter techniques are emerging for left atrial (LA) decompression under venoarterial extracorporeal membrane oxygenation (VA-ECMO). We aimed to assess whether balloon atrioseptostomy (BAS) is a safe and efficient strategy. Methods: All patients who underwent percutaneous static BAS under VA-ECMO at four tertiary institutions were retrospectively reviewed. Results: From 2000 to 2014, BAS was performed in 64 patients (32 adults and 32 children). Indications for ECMO support included acute myocarditis (31.3%) and non-myocarditis cardiac disease, mostly end-stage dilated cardiomyopathy (32.8%). BAS was required because of pulmonary oedema/haemorrhage and left ventricular (LV) distension. The mean balloon diameter was 21.8 ± 8.4mm. Adequate LA decompression was achieved in all patients. Mean LA pressure fell from 24.2 ± 6.9 mmHg to 7.8 ± 2.6 mmHg ( p Conclusions: Percutaneous BAS may be a safe and efficient strategy for discharging the LA in both adults and children supported by VA-ECMO.