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Objectives Melody TPV is the first percutaneous valvular bio–prosthesis approved for trans–catheter pulmonary valve implantation (TPVI). We describe our ten–years experience about Melody TPV implantation in patients with congenital heart disease (CHD). Methods This is an observational retrospective single–center study. All patients undergone Melody TPV implantation were included. The early outcomes analyzed were: procedural failure, death of patient, life–threating adverse events. The long–term outcomes analyzed during follow–up were: infective endocarditis, trans–catheter redo–procedure, and surgical redo–procedure. Results From 2012 to 2022, 39 patients were evaluated in our catheterization laboratory for TPVI with Melody TPV. Mean age and weight were 21,31±11,58 (range 8–66) years and 57,5±16,24 (range 23–96) kilograms. The patients were affected by the following CHD: tetralogy of Fallot (22 pts, 56,4%), pulmonary atresia with ventricular septal defect (VSD) (4 pts, 10,3%), aortic stenosis s/p Ross surgery (4 pts, 10,3%), transposition of the great arteries with VSD and PS (3 pts, 7,7%), pulmonary stenosis (PS) (2 pts, 5,1%), truncus arteriosus (2 pts, 5,1%), double outlet right ventricle (1 pt, 2,5%), Ebstein’s anomaly (1 pt, 2,5%) In 4 cases (10,3%), the procedure failed because of coronary compression during balloon RVOT interrogation. One patient (2,5%) died due to post–procedural septic shock. In the other 34 patients (87,2%), the Melody TPV was implanted effectively. No life–threating adverse events were recorded. In 3 cases, the Melody TPV was implanted off–label: in tricuspid position (one case) and in “small conduits” ( Conclusion Trans–catheter Melody TPV implantation is effective to deal RVOT dysfunction. At a mid–term follow–up, the most of the implanted Melody TPV worked properly and only a few patients required surgical valve replacement.

Background Sacubitril/valsartan has been proved to reduce mortality in heart failure and reduced ejection fraction (EF) and is currently recommended as first-line therapy. However, effects in patients with a systemic right ventricle (sRV) have not been systematically investigated yet. Purpose We aimed to assess safety and efficacy of sacubitril/valsartan in patients with a sRV Methods From September 2020 to April 2021, all patients with congenitally corrected transposition of the great arteries (TGA) or TGA after Senning/Mustard repair attending our tertiary centre were prospectively enrolled. Inclusion criteria were: age ≥18 years, 3-months of optimal medical therapy including ACEi/ARB and sRV EF≤40%. Patients with univentricular physiology, systolic blood pressure (SBP) 5.5 mEq/L were excluded. SBP and blood samples were obtained at 1-month of treatment. Other clinical and echocardiographic variables were reassessed at 6 and 12-month follow-up and the medication was progressively up-titrated to the highest tolerated dose. Results Fifty-one patients (38±11 years, 60% male, 34% ccTGA) were included. Up to March 2022, 48 (92%) patients were reviewed after 6 months of therapy and 35 (68%) completed the first year of follow-up. Baseline patients' characteristics are summarized in Table 1. At 1 month, treatment did not impact on the serum potassium values (4.5±0.3 vs 4.4±0.3 mEq/L, p=0.9) and GFR (112±33 vs 112±31 ml/min, p=0.3), while SBP dropped significantly (119±13 vs 108±18 mmHg, p=0.003). Two patients ceased the treatment due to symptomatic hypotension during the first month. One patient developed a nephrotic syndrome at 4 months of follow-up, which was likely unrelated to the treatment. No other major adverse events were reported. One patient was lost to follow-up after 3 months. Despite no significant change in the NYHA class (p=0.9), the 6-minute walking distance increased significantly at 6-month (Table 2). Nt pro BNP values were significantly decreased at 6-month, and returned to baseline at 12-month. Improved sRV systolic function was demonstrated at 6 and 12-month by significant increase in fractional area change, RV global longitudinal strain and sRV EF measured with 3D echocardiography. Conclusions Our mid-term results showed that sacubitril/valsartan is well tolerated in patients with a sRV and leads to signficant improvement of sRV systolic function, supporting its use in this complex population. Funding Acknowledgement Type of funding sources: None.

Background Wolf-Parkinson-White Syndrome (WPW) has been associated with reduced local myocardium deformation during systole. According to guidelines, when the left ventricle (LV) dysfunction is present catheter ablation of the accessory pathway may be required, even in asymptomatic patients. Purpose The study aimed to assess the diagnostic value of non-invasive myocardial work in predicting subtle myocardial abnormalities in children with WPW. Methods Eighty pediatric patients (age 7.91±3.2 years) were included in the study: 20 cases with manifest WPW and 60 age- and sex-matched controls (CTR). Global myocardial work index (MWI) was measured as the area of the LV pressure-strain loops. From MWI, global Constructive Work (MCW), Wasted Work (MWW) and Work Efficiency (MWE) were estimated. In addition, standard echocardiographic parameters of LV function were evaluated. Results Patients characteristics are summarized in the table. Despite normal LV EF and GLS, children with WPW had worse MWI (WPW 1292.90±307.67 mmHg% vs CTR 1658.73±241.74 mmHg%, p=0.0000005), MCW (WPW 1844.15±267.96 mmHg% vs CTR 2104.90±237.85 mmHg%, p=0.00009), MWW (WPW 101.5 [85–148.25] mmHg% vs CTR 72 [54.75–109.25] mmHg%, p=0.004) and GWE (WPW 94 [91.75–95] mmHg% vs 96 [95–97] mmHg%, p=0.00012) (table). In the WPW group, MWE (r=−0.6, p=0.009) and MWW (r=0.6, p=0.01) was found to correlate with a prolonged QRS (figure). A multiple regression model was generated to identify the relation between abnormal myocardial work indices (worst interquartile range as dependent variable) and several independent variables. QRS was the best independent predictor of impaired myocardial work. In particular, a QRS>110 msec showed an 86% sensibility and 85% specificity for abnormal MWE (AUC of 0.85), and a 75% sensibility and 83% specificity for abnormal MWW (AUC of 0.83) (figure). Conclusion In children with WPW, myocardial work indices were found significantly reduced, even in presence of normal LV EF and GLS. Prolonged QRS independently predicted myocardial impairment. In pediatric patients with WPW, myocardial work may represent a sensitive measure of LV dysfunction and help in the decision-making. Funding Acknowledgement Type of funding sources: None.

Background Adults with congenital heart disease (ACHD) are a vulnerable population. Routine vaccination is the only strategy to prevent a life-threatening infection. However, concerns on the cardiac safety and efficacy of COVID-19 vaccines have been raised. Aim To assess safety and efficacy of available COVID-19 vaccines in ACHD patients. Methods Data on COVID-19 infection and vaccines including booster doses and any suspected or confirmed adverse events were prospectively collected for all ACHD patients attending our tertiary centre from the beginning of the vaccination campaign (March 2021). A group of 75 healthy volunteers, matched per age and sex, was included for comparison. Antispike IgG titre was routinely obtained at the ACHD clinic. Patients' attitude towards COVID-19 was explored with a questionnaire. Results As of February 2022, 498 ACHD patients (36.7±16 years, 54% male,69% with moderate-complex defects, 48% with advanced physiological stage) were enrolled. Four hundred and sixty-one (92%) were fully vaccinated: the type of vaccine was Pfizer-BioNTech for 399 (86%) patients, Moderna for 20 (4%) and AstraZeneca for 26 (6%), 9 received a mixed vaccine regimen (2%). Forty-two (9%) had a history of previous COVID-19 infection and therefore received only one dose. Two-hundred and sixty-nine (58%) patients received a booster dose. Adverse events were mainly mild and transient. One patient complaining of chest pain following administration of mRNA-based vaccination was diagnosed with acute pericarditis, which made full remission after appropriate therapy. Two patients reported a non-specific increment of inflammatory markers. No other severe adverse events were reported. Thirty-seven (7%) refused COVID-19 vaccination being scared of potential cardiac/extra-cardiac adverse events. Among those not-vaccinated, 9 (24%) had a history of previous mild COVID-19 infection. IgG titre was measured in 243 patients at 1915 [835–5934] BAU/ml, which was significantly higher compared to controls (1196 [827–2048] BAU/ml, p=0.002). Three ACHD patients contracted COVID-19 infection after the first dose, while 65 (14%) fully vaccinated patients tested positive for COVID-19, all with mild to moderate symptoms. COVID-19 symptoms duration was significantly longer in case of infection before vaccination (10 [2.7–15] vs 3 [1.2–7], p=0.03). One Fontan patient was tested positive for COVID-19 twice, before and after COVID-19 vaccination, requiring hospitalization in both cases. Four hundred and seven patients completed the questionnaire: 128 (31%) declared to be scared of potential cardiac effects of the vaccine and that the discussion with the ACHD cardiologist was crucial to decide to undergo COVID-19 vaccination. Conclusions Our data provide real-world evidence on COVID-19 vaccines safety and efficacy in ACHD patients. Patients' education from the ACHD team may play a key role in vaccine acceptance in this vulnerable population. Funding Acknowledgement Type of funding sources: None.

Background Multifenestrate aneurysms of interatrial septum (ISA) are a challenge in paediatric age, not only for the complex anathomy, but also for small body surface and small dimensions of cardiac chambers of the patients, that may limit the use of large and multiple devices. Aim of the study: to evaluate the efficacy of percutaneous closure of multifenestrate aneurysms during mid–term and long–term follow–up; to evaluate if the morphological characteristics of interatrial septum may influence the success of the procedure. Materials and Methods We retrospectively analyzed 63 patients (mean–age 9,12±3,12 years) undergone to cardiac catheterism from 2000 to 2021, for percutaneous closure of interatrial aneurysm in our division of Cardiology, subdivided into 3 groups on the basis of side and morphological characteristics of the interatrial aneurysm: Group I (2 o more defects DIA>5 mm); Group II (one defect >5 mm and more than one further fenestrations); Group III (multiple fenestrations). Results Percutaneous closure was efficacy in 60 patients (95%), while in 3 patients (5%) surgery closure was necessary. In half of the treated patients (30) we used 2 devices. Complications occurred in 4 patients (6%). Only in 2 patients occurred major complications (Atrio–Ventricular Block that needed PMK implantation and partial displacement of device, treated by removal and percutaneous replanting). Residual shunts, not emodynamically significant appear in the immediate post–operative period in 26% and during the follow–up in 18%. The group I was associated with greater risk of failure of the procedure (P Conclusions Percutaneous closure of multifenestrate aneurysm of interatrial septum in paediatric age are effective and shows low incidence of failure and complications. If is present residual shunt is not significant. The anathomy of interatrial septum influences procedural outcome.

Funding Acknowledgements Type of funding sources: None. Background Blood speckle imaging (BSI) is a new speckle-tracking-based technique for the evaluation of blood flow. Ventricular flow vortices have been studied in sinus rhythm in normal and dysfunctional hearts, however, data are lacking in patients with Kent accessory pathway (KAP) and short atrio-ventricular (AV) conduction. Purpose We aimed to evaluate the characteristics of left ventricle blood vortices in children with KAP. Methods Nineteen paediatric patients (age 7 ± 2.9 years) were included in the study: 13 patients with manifest KAP (KAP group) and 6 age and sex -matched controls with normal AV conduction (CTR group). A thorough echocardiographic evaluation with 2D, color doppler and BSI was performed in all the included patients. BSI was recorded in apical 3-chamber view with a 6S-D probe. Vortices characteristic were analysed during filling phase of the left ventricle. We focused on the anterior vortex generated by mitral valve, which persisted longer during the cardiac cycle and is assumed to contribute to optimizing cardiac function. A standard 12-lead ECG was also recorded for each child in KAP group to esteem KAP localization. Results According to Arruda criteria for KAP localization, all patients in KAP group manifested the accessory pathway in the septal region. All patients in CTR group presented one major anterior vortex during filling phase, while in KAP group 10 patients out of 13 (p= 0.009) lacked of this main anterior vortex, showing instead fragmented different vortices. There were no differences in term of left ventricle function (KAPg 59.8 ± 4.02% vs CTRg 59.0 ± 2.5%, p= 0.6) and global longitudinal strain (KAPg -18.6 ± 1.0% vs CTRg -19.6 ± 3.1%, p= 0.5) between the two groups. Conclusion In our preliminary analysis, in patients with septal KAP, blood vortices adapted their diastolic traces to the septal dyssynchrony showing a fragmented pattern. We speculate this fragmentation may contribute to impair the performance of the left ventricle. Abstract Figure. BSI in control vs patient with WPW

Funding Acknowledgements Type of funding sources: None. Background Wolff-Parkinson-White Syndrome (WPW) has been associated with reduced local myocardium deformation during systole. Left ventricle (LV) dysfunction due to electrical dyssynchrony may require catheter ablation of the accessory pathway, even in asymptomatic patients. Purpose The study aimed to assess the diagnostic value of non-invasive myocardial work in predicting subtle myocardial abnormalities in patients with WPW. Methods Forty-four paediatric patients (age 8.2 ± 4.3 years) were included in the study: 12 cases with manifest WPW and 32 age-, sex- and arterial pressure- matched controls (CTR). LV ejection fraction (EF) and global longitudinal strain (GLS) were evaluated. Global myocardial work index (MWI) was measured as the area of the LV pressure-strain loops. From MWI, global Constructive Work (MCW), Wasted Work (MWW) and Work Efficiency (MWE) were estimated. Results Despite normal LV systolic function by standard echocardiographic parameters, patients with WPW had lower MWI (1233.5 ± 281.6 mmHg% in WPW vs 1624.0 ± 305.8 mmHg% in CTR, p = 0.0004), MCW (1833.4 ± 257.9 mmHg% in WPW vs 2069.0 ± 319.9 mmHg% in CTR, p= 0.03), MWW (187.6 ± 117.7 mmHg% in WPW vs 90.9 ± 58.9 mmHg% in CTR, p= 0.0008) and GWE (90.5 ± 4.8% in WPW vs 95.2 ± 2.2% in CTR, p= 0.00006). There were no significant differences in GLS and EF between patients with WPW and controls. Conclusion Myocardial work indices were found significantly reduced in patients with WPW, even in presence of normal LV EF and GLS. In patients with WPW, MWI may represent a sensitive measure of myocardial dysfunction and help in the decision-making for catheter ablation. Abstract Figure. Myocardial work in WPW Abstract Figure. Myocardial work in control patient

Funding Acknowledgements Type of funding sources: None. Background Adults with coarctation of the aorta (CoA) may present residual aortic obstruction and develop arterial vasculopathy and subclinical left ventricular (LV) dysfunction. Myocardial work (MW) is a novel non-invasive index of myocardial efficiency calculated from echocardiographic LV pressure-strain loops, which yields incremental information over ejection fraction and global longitudinal strain. (GLS) Purpose Aim of the present study is to analyze LVMW in a cohort of adult patients with operated aortic CoA Methods CoA patients aged > 18 years who underwent transthoracic echocardiography between September 2020 and July 2021 at our tertiary centre were included. Exclusion criteria were significant recoarctation, impaired LVEF, significant valvular disease and suboptimal image quality. A group of healthy individuals with no cardiac abnormalities. GLS and peak strain dispersion(PSD) were measured. MW indices were calculated using the blood pressure measured in the right arm at the time of the exam. Results Sixty patients (26[22-33]years, 66%male) were included. Data on previous medical history, clinical status at last assessment and Coa-related echocardiographic findings are showed in table 1. No significant differences in traditional parameters of LV systolic and diastolic function were found between groups (EF 60[57-64] vs 57[53-61],p = 0.6), however Coa patients had higher LVmass (84[75-97] vs 68[56-75]g). GLS and MW indices in the study population and in the control group are reported in table 2. Coa group showed lower GLS values and higher PSD (p 0.0001). Conclusion MW indices assessment is feasible in Coa patients may provide a more comprehensive understanding of the overall myocardial mechanics and performance. In particular, MW demonstrated impaired LV efficiency, which was strongly related to increased mechanical dispersion in Coa patients. Abstract Figure. Abstract Figure.

Background Adults with congenital heart disease (ACHD) are frequently affected by thyroid diseases (TD). However, the clinical relevance of TD in ACHD remains unknown. Purpose We aimed to describe the prevalence of TD in the ACHD population and to ascertain whether TD are associated with worse outcome. Methods Clinical data on all consecutive patients aged >18 years attending our ACHD unit for a day-case between 2014 and 2019 were retrospectively collected. For statistical analysis, a composite endpoint was created combining the following events at follow-up: hospitalization for heart failure, new-onset tachyarrhythmic or bradyarrhythmic events and death. Results Four hundred ninety-five ACHD patients with a median age of 32.2 [24.5–45.6] years (46% male) were included. There was an overall prevalence of patients with moderate or complex lesions (414=84%). Compared to the group with no history of TD, patients in the TD were older, (41.9 [29.7–53.5] vs 30.2 [24.3–39] years; p Median follow-up was 9.4 [4.5–13.1] years. Patients with TD had a higher unadjusted mortality rate, with a trend towards statistical significance (p=0.07). Sixty-four (42%) patients in the TD group and 43 (12.5%) met our composite endpoint, leading to a 10-year survival free from events of 53.7% Vs 86.5%, respectively (p Conclusion TD is a strong predictor of adverse outcome in the ACHD population after exclusion of potential confounding factors, being related to a fourfold increased risk of events at follow-up. Funding Acknowledgement Type of funding sources: None. Figure 1Table 1

Background Careful, step-wise assessment is required in all patients with an atrial septal defect (ASD) to exclude pre-existing pulmonary vascular disease or left ventricular disease. Fluid challenge test (FCT) and balloon occlusion testing (BOT) may unmask left ventricular disease and challenge the pulmonary circulation, but their complementary role in the evaluation of patients with “operable” ASD is not well established. Aim To evaluate the haemodynamic changes of the pulmonary circulation by FCT and BOT in ASD patients undergoing percutaneous closure according to the current guidelines. Methods Consecutive patients selected for percutaneous ASD closure underwent invasive hemodynamic assessment at baseline and after BOT, FCT and both. Results Fifty patients (mean age 47.3±11.7 years, 72% female) were included. All patients had a pulmonary-to-systemic flow ratio (QP/QS) ≥1.5, pulmonary vascular resistance (PVR) FCT caused a marked increase in pulmonary blood flow of almost 2 liters (p0.1). No difference was observed in the PAWP response to FCT, BOT or both between groups; no patients reached a PAWP ≥18 mmHg following FCT or BOT alone, but 4 (8%) patients did following the addition of FCT to BOT. No acute clinical adverse events were experienced by any patients. Conclusions None of the reported ASD patients presented with FCT criteria of post-capillary PH (that is a PAWP >18 mmHg). Even small rises in PVR may have significant implications on cardiovascular haemodynamics. In fact, patients with PVR Funding Acknowledgement Type of funding sources: None. Figure 1