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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 According to current guidelines, the diagnosis of pulmonary hypertension (PH) relies on echocardiographic probability followed by right heart catheterization. How echocardiography predicts PH recently re-defined by a mean pulmonary artery pressure (mPAP) >20 mmHg instead of ≥25 mmHg and pulmonary vascular disease defined by a pulmonary vascular resistance (PVR) >3 or >2 Wood units has not been established. Methods A total of 278 patients referred for PH underwent a comprehensive echocardiography followed by a right heart catheterization. Fifteen patients (5.4%) were excluded because of insufficient quality echocardiography. Results With PH defined by a mPAP >20 mmHg, 23 patients had no PH, 146 had pre-capillary and 94 post-capillary PH. At univariate analysis, maximum velocity of tricuspid regurgitation (TRV) ≥2.9 and ≤3.4 m/s, left ventricle (LV) eccentricity index >1.1, right ventricle (RV) outflow tract (OT) notching or acceleration time 1 and PA diameter predicted PH, whereas inferior vena cava diameter and right atrial area did not. At multivariable analysis, only TRV ≥2.9 m/s independently predicted PH. Additional independent prediction of PVR >3 Wood units was offered by LV eccentricity index >1.1 and RVOT acceleration time 20 mmHg, mPAP ≥25 mmHg, mPAP >20 mmHg + PVR >2 Wood units and mPAP ≥25 mmHg + PVR >3 Wood units are illustrated in Figure 1A–D. Conclusions Echocardiography as recommended in current guidelines can be used to assess the probability of re-defined PH in a referral center. However, the added value of indirect signs is modest and sufficient quality echocardiographic signals may not be recovered in some patients. 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.

Introduction The maintenance of military surgeons’ operative skills is challenging. Different and specific training strategies have been implemented in this context; however, little has been evaluated with regard to their effectiveness. Cancer surgery is a part of military surgeons’ activities in their home hospitals. This study aimed to assess the role of oncological surgery in the improvement of military surgeons’ operative skills. Methods Between January and June 2019, the surgical activities of the departments of visceral, ear, nose, and throat, urological, and thoracic surgery were retrospectively reviewed and assessed in terms of the operative time (OT). All surgeons working at the Sainte Anne Military Teaching Hospital were sent a survey to rate on a 5-point scale the current surgical practices on their usefulness in improving surgical skills required for treating war injuries during deployment (primary endpoint) and to compare on a 10-point visual analog scale the influence of cancer surgery and specific training on surgical fluency (secondary endpoint). Results Over the study period, 2,571 hours of OT was analyzed. Oncological surgery represented 52.5% of the surgical activity and almost 1,350 hours of cumulative OT. Considering the primary endpoint, the mean rating allocated to cancer surgery was 4.53 ± 0.84, which was not statistically different than that allocated to trauma surgery (4.42 ± 1.02, P = 0.98) but higher than other surgery (2.47 ± 1.00, P Conclusion This study demonstrates the usefulness of cancer surgery in improving the operative skills of military surgeons.

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 Transthoracic echocardiogram is the most important non-invasive screening tool for pulmonary hypertension (PH), but right heart catheterization (RHC) remains mandatory to establish the diagnosis. According to the current guidelines, the echocardiographic probability of PH is based on the combination of direct and indirect signs. In the 6th World Symposium on Pulmonary Hypertension, PH has been defined as mean pulmonary artery pressure (mPAP) >20 mmHg, and pulmonary vascular resistance (PVR) ≥3 WU. Aim To validate the echocardiographic direct and indirect signs of PH suggested by the guidelines in view of the new definition of PH in patients who underwent echocardiography and RHC. Methods All consecutive patients referred between January 2018 and December 2019 to undergo RHC for suspected PH were prospectively enrolled in the study. Echocardiography was performed within one hour from indicated RHC. Results Overall, 263 patients were enrolled: 33 (13%) had normal pulmonary pressures, and 230 (87%) had PH. Among the 230 patients with PH, 136 (59%) had pre-capillary and 94 (41%) had post-capillary PH. All underwent echocardiography within one hour from RHC. At univariable logistic regression test, tricuspid regurgitant jet (TRJ) >2.9 m/sec [OR 8.32 (3.15–26.37, p1.1 [OR 5.57 (2.44–14.41, p25 mm [OR 4.74 (2.01–13.09, p=0.001)], right-to-left ventricle basal diameter >1 [OR 4.54 (1.83–13.74, p=0.003)], and early diastolic pulmonary regurgitation velocity >2.2 m/sec [OR 3.14 (1.07–13.46, p=0.067)], predicted PH, whereas dilated inferior vena cava and right atrial area did not. At multivariable analysis, only TRJ >2.9 m/sec [OR 7.95 (2.78–27.07, p1.1 [OR 4.89 (1.75–15.35, p=0.004)] showed a positive correlation to PH. The best area under the curve (AUC, 0.86) for predicting PH was obtained with TVR >2.9 m/sec + 2 indirect signs (sensitivity 0.77, specificity 0.94). Non-invasive estimate of mPAP showed a good correlation to invasive measurement (regression coefficient β 16.8±0.7, p Conclusions Echocardiographic direct and indirect signs of PH as suggested by the current guidelines should be used to assess the probability of PH being present. Echocardiographic measurements have high accuracy (low bias) and moderate precision (acceptable limits of agreement) The best area under the curve for predicting PH according to the new definition (mPAP >20 mmHg and PVR ≥3 WU) can be obtained with TVR >2.9 m/sec + 2 indirect signs. Funding Acknowledgement Type of funding sources: None. Figure 1

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

Background The International Agency for Research on Cancer evaluated formaldehyde (F) as carcinogenic for human in association with Nasopharyngeal Cancer (NPC). Occupational exposure to F occurs in many industrial sectors also in those non-traditional. For example in Tuscany F was detected in a bakery where a NPC case had ever worked. Methods In this study a) A case control approach (OCCAM) was used for monitoring occupational risks based on current information sources. Three Italian Regional Operating Centres, collected NPC cases from cancer registries and/or hospital discharge records. Controls were randomly sampled from the regional health service population data. Occupational histories were available through record linkage with the social security pension database (INPS). Study results were reported by industrial sectors, area and gender. b) A specific study on F exposure was conducted in bakeries and pastry industry carrying out measurements to determine the concentration of F in specific tasks and positions. Results 717 cases linked with INPS database. Increased ORs for several industrial sectors such as iron and steel, wood and plastic were observed. In two regions also health and veterinary services and hairdressers were at increased risk, but based on few cases of exposed workers. In the non traditional sector of bakeries and pastry industries, where workplaces were monitored, high levels of F in personal air samplings were found, in particular in processes that involve a strong leavening. Conclusions Many productive sectors, in which F exposure could occurred, were observed to be at higher risk. The measurements performed in the non traditional sector monitored, confirmed the F exposure in some phases of the work process. With this study we contributed to increased knowledge on the risk of NPC within the monitoring system of occupational risks, and to deepen exposure to F in a non traditional productive sector such as bakeries and pastry industries. Key messages The epidemiological method used (OCCAM) in this study provides further information on the role of occupational exposure in the development of nasopharyngeal cancer. The measurements performed in the bakeries and pastry industries provide new additional information on the exposure to formaldehyde in some work process phases of a non-traditional productive sector.