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Background: Long-term data on COVID-19 vaccine safety, immunogenicity, and acceptance in adults with CHD are lacking. Methods: This is a prospective study including adults with CHD patients undergoing COVID-19 vaccination from January 2021 to June 2022. Data on adverse events, antispike IgG titre, previous or subsequent COVID-19 infection, booster doses, and patients’ attitude towards vaccination were collected. Results: Four hundred and ninety CHD patients (36 ± 13 years, 53% male, 94% with moderate/complex defects) were prospectively included: 433 (88%) received a Pfizer–BioNTech mRNA vaccine, 31 (6%) Moderna mRNA vaccine, 23 (5%) AstraZeneca–Oxford ChAdOx1 nCov-19 vaccine, and 3 (0.6%) Janssen Vaccine; 310 (63%) received a booster dose. Median follow-up after vaccination was 1.53 [1.41–1.58] years. No major adverse event was reported. Eighty-two fully vaccinated patients contracted COVID-19 during follow-up after a median of 5.4 [4.3–6.5] months from the last dose. One patient with Ebstein’s disease died from severe COVID-19. Symptoms’ duration in patients who tested positive after vaccination was significantly shorter than in the group tested positive before vaccination (5.5 [3–8] versus 9 [2.2–15] days, p = 0.04). Median antispike IgG titre measured in 280 individuals (57%) at a median of 1.4 [0.7–3.3] months from the last dose was 2381 [901–8307] BAU/ml. Sixty patients (12%) also showed positive antinucleocapsid antibodies, demonstrating previous SARS-COV2 exposure. Twenty-nine percent appeared to have concerns regarding vaccine safety and 42% reported fearing potential effects of the vaccine on their cardiac disease before discussing with their CHD cardiologist. Conclusion: COVID-19 vaccines appear safe in the mid-term follow-up in adults with CHD with satisfactory immunogenicity and reduction of symptoms’ duration in case of infection.

To analyze left ventricular (LV) mechanics through advanced echocardiography, including speckle tracking analysis and myocardial work (MW) in a cohort of adults with repaired aortic coarctation (CoA).Data on standard echocardiography, LV speckle-tracking and MW analysis were collected in CoA patients 18 years with no significant recoartation or valvular disease and normal LV ejection fraction at the time of the exam. MW indices were calculated using the blood pressure measured in the right arm. A group of healthy subjects with comparable sex, age and body surface area was included for comparison.Eighty-nine CoA patients and 70 healthy subjects were included. Patients had higher systolic blood pressure (p 0.0001), LV mass index (p 0.0001), left atrial volume index (p = 0.005) and E/E' ratio (p = 0.001). Despite similar LV ejection fraction, speckle tracking analysis revealed lower global longitudinal strain (GLS: - 18.3[17-19] vs - 20.7[19-22]%, p 0.0001) and increased peak systolic dispersion (PSD: 45[40-54] vs 37.5[32-43] ms, p 0.0001) in CoA patients. Global work index (GWI) and global constructive work were similar to healthy controls (p = 0.6 and 0.5, respectively), whereas CoA patients showed significant increased wasted work (GWW: 125[90-185] vs 89.5[64-127]mmHg%, p 0.0001) and a mild but significant reduction in global work efficiency (GWE: 93%[92-95] vs 95%[94-97], p 0.0001). Moreover, when stratifying for GLS values, MW analysis showed increased GWW and PSD with impaired GWE in 54(61%) patients with normal GLS compared to healthy individuals. Spearman's linear method illustrated an inverse relation between GWE and PSD (r: - 0.53, p 0.0001), while GCW was associated with peak (r: 0.2, p = 0.01) and mean gradient across the descending aorta (r: 0.3, p = 0.004) and with systolic blood pressure (r: 0.48, p 0.0001). PSD was the sole univariate predictor of GWE on linear regression analysis (β: - 0.1 [- 0.16 to - 0.07], p 0.0001), whereas female sex, SBP and gradients across the descending aorta were independently associated with higher GCW values. When CoA patients were divided based on the history of redo CoA repair and arterial hypertension, no significant differences in MW indices were found.MW is a novel echocardiographic tool, which provides additional information on LV performance in CoA patients over GLS allowing a more comprehensive understanding of LV dysfunction mechanisms in a setting of increased afterload.

BACKGROUND: Sacubitril/valsartan was demonstrated to reduce hospitalization rate and mortality in patients with heart failure with reduced ejection fraction. Data on the effects of sacubitril/valsartan in patients with a systemic right ventricle are still lacking. METHODS: Patients with transposition of the great arteries following Senning/Mustard procedure or congenitally corrected transposition of the great arteries with impaired systemic right ventricle systolic function were prospectively included. Primary end points included sacubitril/valsartan safety and efficacy. Primary efficacy end points were NT-proBNP (N-terminal pro-B-type natriuretic peptide) and systolic function improvement. Secondary end points included New York Heart Association class, 6-minute walking distance, and quality of life change. RESULTS: Fifty patients (38±12 years, 60% male, 35% congenitally corrected transposition of the great arteries) were included and followed for 1 year. No major adverse events occurred. Two (4%) patients ceased treatment due to hypotension and 1 (2%) developed a nephrotic syndrome. The target dose was reached in 20 (42%) patients. NT-proBNP values decreased significantly immediately after treatment initiation, while returned to baseline at 1 year. Echocardiography showed progressive fractional area change increase (29.2±5.8 versus 34.9±5.1%; P P P P =0.006). Moreover, 3-dimensional echocardiography demonstrated right ventricle volumes reduction (end-diastolic volume: 181±63 versus 156±50 mL; P =0.002; end-systolic volume: 117±48 versus 89±33 mL; P P P P CONCLUSIONS: Our data showed that sacubitril/valsartan is well tolerated and is associated with systemic right ventricle remodeling and improved systolic function as well as improved clinical status, supporting its use in this complex population.

Obstruction of the superior vena cava represents an under-recognized cause of chylothorax in the adult population. Our case report describes the successful conservative management of chylothorax due to bilateral superior vena cava obstruction in an adult patient with complex congenital heart disease. (Level of Difficulty: Beginner.), Central Illustration

Background Pulmonary valve (PV) endocarditis is a frequent complication during follow-up in patients with repaired right ventricular outflow tract (RVOT) obstruction and poses relevant diagnostic and treatment challenges. We aimed to describe in details the possible different clinical presentations of this rare condition and to highlight the role of both transthoracic and transoesophageal echocardiography which, in experienced hands, may provide comprehensive useful information for the clinicians. Case summary We below describe the clinical presentation and the echo findings of three cases of pulmonary valve endocarditis complicating disease course after different repair modalities of congenital right ventricular outflow tract obstruction. Discussion The present case series outlines the diagnostic challenges of this increasingly frequent complication during follow-up of patients with congenital RVOT dysfunction after both surgical and percutaneous repair. Despite the diffusion of multimodality imaging, echocardiography with PV-dedicated views play a pivotal role in diagnosing such condition and guiding clinical management. Furthermore, this case series highlight that the suspicion of infective endocarditis should be raised whenever a sudden increase in transvalvular gradient is found during follow-up.

Background:Adults with CHD are known to greatly benefit from a prompt access to continuous expert care. On the other hand, coronavirus disease 2019 pandemic has determined a dramatic worldwide reconfiguration of the healthcare systems, with rapid redeployment of resources towards this emergency. Italy was the first Western country affected by a large-scale spread of coronavirus disease 2019. The aim of our study is to analyse the impact of the coronavirus disease 2019 outbreak on in-hospital care of patients with CHD in an Italian tertiary centre.Methods and results:We retrospectively reviewed data on CHD hospital admissions in our centre since 1 March, 2020, when the adoption of a strict infection containment policy throughout the country resulted in limited access of patients to routine hospital care and resources reallocation to the care of infected patients. Comparison with data from the previous year was performed in order to identify any relevant differences attributable to the outbreak. Despite cancellation of all elective procedures, the overall number of urgent hospital admission remained stable throughout the period of study. Patients admitted during the pandemic had greater disease complexity (p = 0.001) with longer length of in-hospital stay (p = 0.01). No adverse events or positive swabs were reported among CHD patients who were admitted to hospital or medical personnel caring for these patients.Conclusion:Data from our early experience suggest that coronavirus disease 2019 pandemic did not impact significantly on the provision of urgent care to adult patients with CHD.

Aims Wolff–Parkinson–White syndrome (WPW) is a clinical condition characterized by pre-excitation on electrocardiogram (ECG) and symptoms of arrhythmias. It has been described that premature ventricular activation induces septal wall motion abnormalities and ventricular dyssynchrony, causing detrimental effects on cardiac performance. The new speckle-tracking-based technique, blood speckle imaging (BSI), has been previously used to evaluate blood flow characteristics in normal and dysfunctional hearts in patients with normal atrio-ventricular (AV) conduction. We aimed to study the features of left ventricle blood vortices in patients with WPW and short AV conduction by using BSI. Methods and results Nineteen paediatric patients (age 7 ± 2.9 years) were included in the study: 13 patients with manifest WPW (WPW group) and 6 age and sex -matched controls with normal AV conduction (CTR group). A complete 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 characteristics were analysed during the filling phase of the left ventricle. We focused on the anterior vortex generated by the mitral valve, which persisted longer during the cardiac cycle and is assumed to contribute to optimizing cardiac function. For each child in the WPW group, Arruda criteria were used to esteem accessory pathway localization from a 12-lead surface ECG. All patients in the WPW group manifested the accessory pathway in the septal region. All patients in the CTR group presented one major anterior vortex along the septal wall during the filling phase (Figure A), while in the WPW group, 10 patients out of 13 (P = 0.009) lacked this main anterior vortex, showing instead fragmented vortices (Figure B). Interestingly, the main differences in blood vortices pattern were showed in the last part of filling phase, when blood flow could be affected by premature activation of the septal wall. There were no differences in terms of left ventricle function (WPWg 59.8 ± 4.02% vs. CTRg 59.0 ± 2.5%, P = 0.6) and global longitudinal strain (WPWg −18.6 ± 1.0% vs. CTRg −19.6 ± 3.1%, P = 0.5) between the two groups. 136 Figure A and B Conclusions In our preliminary study, patients with WPW showed a fragmented pattern of diastolic blood vortices that adapted to septal dyssynchrony. We speculate this fragmentation may contribute to impair the performance of the left ventricle.

Aims Paediatric patients with a diagnosis of Wolff–Parkinson–White (WPW) Syndrome may develop a reduction of local myocardial deformation because of accessory pathway-related electrical dyssynchrony, which may lead to an impairment of left ventricular systolic function. The presence of ventricular dysfunction may be an indication for these patients to undergo radiofrequency catheter ablation (RFCA), even if asymptomatic. However, myocardial abnormalities are sometimes subtle and cannot be detected by standard echocardiographic evaluation. The purpose of this study was to assess the diagnostic value of non-invasive myocardial work in predicting subtle myocardial abnormalities in paediatric patients with WPW Syndrome. Methods and results Forty-four paediatric patients (age 8.2 ± 4.3 years) were included in this study: 12 cases with manifest WPW Syndrome and 32 age-, sex-, and arterial pressure-matched controls (CTR). Left ventricular ejection fraction (LVEF) 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. Despite normal LV systolic function by standard echocardiographic parameters, paediatric patients with WPW Syndrome 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 LVEF between patients with WPW Syndrome and controls. Conclusions WPW Syndrome was found to be associated with a significant reduction of myocardial work indices in the paediatric population. The assessment of MWI may represent a sensitive measure to identify a subtle myocardial dysfunction in an early stage, even when LVEF and GLS are normal. It might be considered a further diagnostic parameter for referring little patients to RFCA.

Background real-world data on COVID-19 vaccine safety, immunogenicity and acceptance in adults with congenital heart disease (ACHD) are lacking. Methods ACHD patients who were offered COVID-19 vaccination from January to June 2021 were included. Data on adverse events, on patients’ attitude towards vaccination and antispike IgG titre were retrospectively collected. A group of healthy individuals with similar age and sex undergoing vaccination was included for comparison. Results 208 patients followed in a single ACHD tertiary centre (33.3 [26–45]years,54%male) received COVID-19 vaccine, 65% vaccinated at our institution: 199 (96%)received Pfizer–BioNTech BNT162b2 vaccine, 4 (2%)Moderna-1273 and 5 (2%)AstraZeneca–ChAdOx1. Median follow-up after vaccination was 79 [57–96]days. No major adverse event was reported and there was no difference in the in adverse events incidence between ACHD patients and the control group. One patient was diagnosed with acute pericarditis. There were two unrelated deaths during follow-up. Three (1.5%)vaccinated patients tested positive for COVID-19. Antispike IgG titre, available in 159 (76%)patients, was 1334 [600–3401]BAU/ml, not significantly different from the control group (p = 0.2). One patient with Fontan failure was seronegative. Advanced physiological stage was related to lower antibody response, independently from previous viral exposure (p, Graphical abstract Image 1

Adults with congenital heart disease (ACHD) are frequently affected by thyroid diseases (TDs). However, the clinical relevance of TD in ACHD remains unknown. We aimed to describe the prevalence of TD in the ACHD population and to ascertain whether TD are associated with worse outcome. Patients with ACHD18 years attending our tertiary center for a day-case between 2014 and 2019 were included. Clinical data between patients' first visit and December 2020 were collected. Primary end point was a combination of death, hospitalization for heart failure (HF), and new-onset of arrhythmic events. Secondary end points were each part of the primary outcome as separate end points. A total of 495 patients with ACHD (32.2 [24.5 to 45.6] years; 54% women) were included. Median follow-up was 9.4 (4.5 to 13.1) years. The prevalence of TD was 30%. TD group showed worse clinical status, as demonstrated by N-terminal pro b-type natriuretic peptide values (243.5 [96.5 to 523] vs 94 [45 to 207] pg/ml, p0.001) and New York Heart Association class (27% vs 13% in class III to IV, p0.0001) with higher incident rate of adverse events at follow-up (4.45 [3.43 to 5.69] % vs 1.29[0.94 to 1.75] % per person-year, p0.001). TD were independently associated with higher risk of death (hazard ratio [HR] 4.1, p = 0.009), arrhythmic events (HR 3.8, p0.0001), and hospitalization for HF (HR 8.02, p0.0001). There was a fourfold increased risk of primary end point in the TD group even after propensity score matching for clinical variables including age, gender, disease complexity, physiological stage, previous palliative surgery, ventricular function, pulmonary arterial hypertension, cyanosis, and presence of systemic right ventricle (HR 4.47, p0.0001). In conclusion, TD are predictive of adverse outcome in the ACHD population. Routine screening of thyroid function during follow-up in this population may be helpful to identify those with higher risk of death, arrhythmias, and HF.

BACKGROUND: Anomalous aortic origin of coronary arteries (AAOCA) is a rare abnormality, whose optimal management is still undefined. We describe early outcomes in patients treated with different management strategies. METHODS: This is a retrospective clinical multicenter study including patients with AAOCA, undergoing or not surgical treatment. Patients with isolated high coronary take off and associated major congenital heart disease were excluded. Preoperative, intraoperative, anatomical and postoperative data were retrieved from a common database. RESULTS: Among 217 patients, 156 underwent Surgical repair (median age 39 years, IQR: 15-53), while 61 were Medical (median age 15 years, IQR: 8-52), in whom AAOCA was incidentally diagnosed during screening or clinical evaluations. Surgical patients were more often symptomatic when compared to medical ones (87.2% vs 44.3%, p

A 3-months-old infant was urgently admitted for drowsiness and lack of appetite started 24 h before. The ECG showed sinus rhythm with a prolonged AV interval (200 ms) and very large QRS complexes (280 ms) due to Flecainide overdosing following incorrectly administration for poor communication between parents resulted in both giving a dose to the infant. Flecainide serum level was 1.2μg/ml, confirming the diagnosis of an accidental drug intoxication. The patient started continue hydration with a close monitoring. Three hours later a significant narrowing of the QRS complex (150 ms) was observed, then over the following 24 h, the QRS almost completely normalized.

The purpose of this study was to use hypnosis in patients with congenital heart disease undergoing transesophageal echocardiography (TEE).From January 2016 to July 2017, 50 adult patients undergoing TEE were randomly assigned to two groups: TEE in hypnosis (n = 23), TEE in sedation (n = 27). Vital parameters (heart rate [HR], blood pressure [BP], oxygen saturation [SO2] before, during and after the procedure) and drug administration were recorded. The State-Trait Anxiety Inventory was performed before and after TEE, the memory and experience of TEE through a structured interview were assessed.All patients in the hypnosis group performed TEE without any sedation. As for anxiety before TEE, no significant differences were observed between groups; after TEE all patients were less anxious than at the beginning (p0.001) with a greater decrease in patients of the hypnosis group (p0.001). Before TEE, there were no significant differences also in HR, BP and SO2. During TEE in both groups a similar increase in HR and BP was found (p0.001), whereas SO2 values remained stable. In the responses to the structured interview, 94% of patients in the sedation group remembered everything vs 36% of the hypnosis group (p0.05). No differences were found in the other answers between the two groups.Hypnosis in TEE is useful to improve the emotional experience of patients with congenital heart disease.

Objectives To describe the characteristics and long-term outcome of a large adult cohort with pulmonary atresia. Background Patients with pulmonary atresia (PA) are a heterogeneous population in terms of anatomy, physiology and surgical history, and their management during adulthood remains challenging. Methods Data on all patients with PA followed in our center between January 2000 and March 2015 were recorded. Patients were classified into the following groups: PA with ventricular septal defect (PA-VSD, 1), PA with intact ventricular septum (PA-IVS, 2) and other miscellaneous PA (PA-other, 3). Results Two-hundred twenty-seven patients with PA were identified, 66.1% female, mean age 25.5 ± 8.7 years. Over a median follow-up of 8.8 years, 49 (21.6%) patients had died: heart failure (n = 21, 42.8%) and sudden cardiac death (n = 8, 16.3%) were the main causes. There was no significant difference in mortality between the 3 Groups (p = 0.12) or between repaired and unrepaired patients in Group 1 (p = 0.16). Systemic ventricular dysfunction and resting oxygen saturations were the strongest predictors of mortality. Additionally, 116 (51%) patients were hospitalized, driven mainly by the need for invasive procedures, heart failure and arrhythmias. Conclusions Adult survivors with pulmonary atresia have a high morbidity and mortality irrespective of underlying cardiac anatomy and previous reparative or palliative surgery. We present herewith predictors of outcome in adult life that may assist with their tertiary adult congenital care.

Pulmonary arterial hypertension (PAH) is a serious complication of congenital heart disease, causing an increase in morbidity and mortality. The progressive and irreversible pulmonary vascular disease is more often the consequence of a significant, uncorrected, left-to-right shunt. The rise in pulmonary vascular resistance may lead to the reversal of the shunt and cyanosis, condition known as Eisenmenger syndrome. The management of this population is challenging and requires specific expertise both for diagnosis and follow-up. The progress in the understanding of the underlying pathophysiology of this condition has promoted recent pharmacological trials. New therapeutic options are now available that could improve the long-term prognosis and the quality of life of these patients, but several controversial points still remain and need to be addressed.