Mid-term follow-up after COVID-19 vaccination in adults with CHD: a prospective study.

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.

Advanced echocardiographic assessment in adults with repaired aortic coarctation: myocardial work analysis provides novel insights on left ventricular mechanics.

AIM: 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). METHODS: 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. RESULTS: 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 (ß: - 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. CONCLUSIONS: 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.

Safety and Efficacy of Sacubitril/Valsartan in Patients With a Failing Systemic Right Ventricle: A Prospective Single-Center Study.

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<0.001), and right ventricle global longitudinal strain (-13.9 [-15.1, -11.8] versus -15.3 [-17.2, -13.4]%; P<0.001) and free-wall global longitudinal strain (-14.3 [-17.3, -12.3] versus -17.2 [-19.3, -15.8]%; P<0.001) raise, whereas tricuspid regurgitation severity improved only in transposition of the great arteries patients (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<0.001), and significantly increased systemic right ventricle ejection fraction (35.6±8.1 versus 41.5±7.5%; P<0.001). Clinical improvement was suggested by New York Heart Association class change (P<0.001), increased 6-minute walking distance (425 [333, 480] versus 500 [443, 560] m; P<0.001) as well as improved quality of life at 1-year follow-up. Beneficial effects were observed irrespective of the underlying anatomy and were more pronounced in those on target dose. 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.

Wearable cardioverter-defibrillator (life-vest): A feasible bridging treatment in adult congenital heart disease.

BACKGROUND: Wearable cardioverter-defibrillators (WCDs) are currently used in patients at temporarily heightened risk for sudden cardiac death (SCD) who are temporarily unable to receive an implantable cardioverter-defibrillator (ICD). WCD can safely record and terminate life-threatening arrhythmias through a non-invasive electrode-based system. The current clinical indications for WCD use are varied and keep evolving as experience with this technology increases. METHODS: We reviewed and explored the data behind indications for WCD use and discuss its usefulness in congenital heart disease (CHD) patients. RESULTS: We considered 8 consecutive patients (mean age 35.25 years, range 18-51 years, average duration of WCD use 4 months, range 3-6 months) with complex CHD, in which a WCD was used between June 2018 and January 2022. No sustained ventricular arrhythmias requiring shocks were recorded in the observation period. No inappropriate shocks were recorded. All the patients showed a good compliance and a very high mean wear time per day (21.2 ± 1 h a day). Four patients implanted a permanent device (3 CRT-D, 1 ICD), three underwent cardiac surgery at the end of the WCD period and one is still on the waiting list for the operation. CONCLUSIONS: Larger trial could confirm the possible conceivable benefit from an extended use of the WCD in certain populations with complex CHD as in our case series, especially in patients with life-treating ventricular arrhythmias waiting for surgery for residual cardiac defects or in the early phases following the surgical/hemodynamic interventions, patients with tachycardiomyopathy expected to improve after the arrhythmias are removed and patients awaiting implantation of an ICD at high risk due to active infection.

Prognostic Relevance of Thyroid Disorders in Adults With Congenital Heart Disease.

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 ACHD >18 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, p <0.001) and New York Heart Association class (27% vs 13% in class III to IV, p <0.0001) 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, p <0.001). TD were independently associated with higher risk of death (hazard ratio [HR] 4.1, p = 0.009), arrhythmic events (HR 3.8, p <0.0001), and hospitalization for HF (HR 8.02, p <0.0001). 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, p <0.0001). 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.

Chylothorax Due to Superior Vena Cava Obstruction in a Patient With Complex Congenital Heart Disease.

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.).

A frightening giant QRS complex in a 3-months-old infant.

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.

COVID-19 vaccination in adults with congenital heart disease: Real-world data from an Italian tertiary centre.

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 the incidence of minor events was not different between ACHD patients and the control group. One patient was diagnosed with acute pericarditis. There were two deaths unrelated to the vaccine 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 associated with lower antibody response, independently from previous viral exposure (p<0.0001). Fourteen percent refused COVID-19 vaccination at our institution. However, 50% of vaccinated patients declared to have been influenced by the discussion with the ACHD cardiologist and 66% of those vaccinated in situ reported that undergoing COVID-19 vaccination at the ACHD centre made them feel safer. Conclusion: COVID-19 vaccines appear safe in ACHD with satisfactory immunogenicity. However, the most vulnerable patients showed lower antibody response. ACHD team may play a key role in vaccine acceptance.

Pulmonary valve endocarditis in adults with congenital heart disease: the role of echocardiography in a case series.

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.

Caring for adults with CHD in the era of coronavirus disease 2019 pandemic: early experience in an Italian tertiary centre.

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.

[Hypnosis in transesophageal echocardiography. The experience in a Pediatric Cardiology and Congenital Heart Disease Unit]. / L'ipnosi nell'ecocardiografia transesofagea. L'esperienza di un Centro di Cardiologia Pediatrica e delle Cardiopatie Congenite.

BACKGROUND: The purpose of this study was to use hypnosis in patients with congenital heart disease undergoing transesophageal echocardiography (TEE). METHODS: 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. RESULTS: 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 (p<0.001) with a greater decrease in patients of the hypnosis group (p<0.001). 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 (p<0.001), 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 (p<0.05). No differences were found in the other answers between the two groups. CONCLUSIONS: Hypnosis in TEE is useful to improve the emotional experience of patients with congenital heart disease.

Anomalous aortic origin of coronary arteries: Early results on clinical management from an international multicenter study.

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 < 0.001). Coronary unroofing was the most frequent procedure (56.4%). Operative mortality was 1.3% (2 patients with preoperative severe heart failure). At a median follow up of 18 months (range 0.1-23 years), 89.9% of survivors are in NYHA ≤ II, while only 3 elderly surgical patients died late. Return to sport activity was significantly higher in Surgical patients (48.1% vs 18.2%, p < 0.001). CONCLUSIONS: Surgery for AAOCA is safe and with low morbidity. When compared to Medical patients, who remain on exercise restriction and medical therapy, surgical patients have a benefit in terms of symptoms and return to normal life. Since the long term-risk of sudden cardiac death is still unknown, we currently recommend accurate long term surveillance in all patients with AAOCA.

The outcome of adults born with pulmonary atresia: High morbidity and mortality irrespective of repair.

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 ±â€¯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.