Exercise-induced type 1 Brugada pattern in a child.

The exercise stress testing may unmask the type 1 Brugada pattern on the surface electrocardiogram in a portion of patients with Brugada syndrome. The occurrence of the type 1 Brugada pattern during an exercise test in pediatric patients is not common. Consequently, the diagnostic yield of the exercise test in this population is still to be explored. We present a case of exercise-induced type 1 Brugada pattern in a 12-year-old child with episodes of palpitations and discuss the available evidence on the role of the exercise stress test in the diagnosis and risk stratification of patients with Brugada syndrome.

Contact allergy to subcutaneous implantable cardioverter defibrillator in a child with Brugada syndrome.

Allergic reactions to components of cardiac implantable electronic devices are rare and often go undiagnosed, which can lead to a misdiagnosis of device infection. Contact allergy to subcutaneous implantable cardioverter defibrillator (S-ICD) is extremely rare. In this report, we present a case of cobalt-related contact allergy in a pediatric patient with Brugada syndrome who underwent S-ICD implantation.

Single-Chamber and Dual-Chamber Pacemaker Devices in Adults with Moderate and Complex Congenital Heart Disease: A Single Tertiary Referral Center Experience.

The number of device implantation procedures has increased in adult patients with congenital heart disease (ACHD). Despite significant improvements in materials and implantation techniques, these patients are exposed to higher risk of device related complications than general population. Herein, we describe our single tertiary referral center experience on transvenous pacemaker (PM) implantation and follow-up in adult patients with moderate and complex congenital heart disease (CHD) as limited data are available on long-term outcome. We considered all adults with moderate and complex CHD aged more than 16 years who underwent transvenous single-chamber and dual-chamber PM implant for sinus node dysfunction or atrioventricular block between January 2013 to December 2022 at our Unit. Seventy-one ACHD patients were included in the study (mean age 38.6 ± 15.2 years, 64% with moderate CHD, 36% with complex CHD). Among 32 patients implanted with a dual chamber PM (DDD PM), 4 devices were reprogrammed in VDD mode, 3 in VVI and 2 in AAI mode during follow-up because of lead dysfunction or permanent atrial arrhythmia. In addition, 26 patients had a single chamber PM (AAI or VVI PM) and 13 patients had single-lead pacing system with a free-floating atrial electrode pair (VDD PM). Just one of 13 single-lead VDD PM was reprogrammed in VVI mode due to a low atrial sensing. In DDD PM group, 10 re-interventions were needed due to lead dysfunction (8 cases) and lead-related infective endocarditis (2 cases). Only 3 patients in the single-lead PM group developed lead dysfunction with 2 re-interventions needed, but no infective endocarditis was reported. The rate of long-term complications is high in moderate and complex ACHD with transvenous PM devices, and it is mainly lead-related. In our experience, the less leads implanted, the less complications will occur. Considering the heterogeneity of the ACHD population, transvenous single-chamber or dual-chamber PM device implantation should always be tailored on the single patient, balancing risks and benefits in this complex population.

Triple Antiarrhythmic Therapy in Newborns with Refractory Atrioventricular Reentrant Tachycardia.

Atrioventricular reentrant tachycardia (AVRT) is the most common form of supraventricular tachycardia in newborns. AVRT is sometimes refractory to conventional antiarrhythmic therapy. We describe our experience about the use of the triple combination of flecainide + propranolol + amiodarone as third-line regimen for refractory and recurrent AVRT in newborns. We considered a series of 14 patients who had failed both first-line and second-line therapy and were treated using the combination of flecainide + propranolol + amiodarone. Transoesophageal electrophysiologic study (TES) was performed to test the effectiveness of medical therapy during hospitalization and to try to reduce the amount of therapy, after amiodarone wash-out, before 1 year of age. TES was repeated at 1 year of age to test the spontaneous resolution of the arrhythmia after treatment discontinuation. Rhythm control was achieved in all 14 patients. At a mean age of 9.3 ± 2 months, AVRT was not inducible by TES in 11/12 amiodarone-free patients. At a mean age of 14.1 ± 3 months, AVRT was still inducible in 7/12 patients after interrupting the entire antiarrhythmic therapy (58.3%). Triple combination was effective as third-line option to suppress AVRT refractory to single and double antiarrhythmic therapy, with no significant adverse events. Our experience suggests that triple therapy could be maintained for a short-term treatment, discontinuing amiodarone before 1 year of age to avoid long-term side effects. Newborns who needed triple therapy appear to have a lower chance of accessory pathway disappearance at 1 year of age. TES could be useful for risk stratification of recurrences at the time of drug discontinuation in infants considered to be at higher risk of recurrent AVRT.

Implantable Loop Recorder with Long Sensing Vector: Safety, Acceptability, and Sensing Performance in Pediatric Patients.

Implantable loop recorders (ILRs) are effective tools for detecting arrhythmias by long-term continuous heart rhythm monitoring. Benefits have been demonstrated even in pediatric patients. ILR with a long sensing vector has recently been designed to improve signal quality in terms of P wave visibility and R wave amplitude. However, there are no data on its use in pediatric patients. We considered a series of pediatric patients implanted with a long sensing vector ILR. Sensing performance, including R wave amplitude and P wave visibility, device-related complications, and diagnostic yield were collected. During follow-up, each patient guided by his/her parents/guardians was also asked to complete a brief questionnaire to assess patient acceptability of the device. Twenty-five consecutive pediatric patients (mean age 11.3 ± 3.5 years, 72% male) were enrolled. The insertion success rate was 100% on the first attempt with no complications. The median amplitude of the R wave was 1.15 mV (interquartile range, 1.01-1.42) with no significant differences between patients aged ≤ or > 10 years (p = 0.726) and between female and male (p = 0.483). P wave was classified as 'always visible' in 24/25 patients (96%). ILR was generally well accepted and tolerated by all involved patients. During a median follow-up of 297 days (117-317), we achieved in 5 patients a correlation between symptoms and rhythm disorders (20%) and ruled out significant arrhythmias in 6 symptomatic children (24%). Long sensing vector ILR showed to be well accepted, with good signal quality and an excellent safety profile even in pediatric patients.

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.

Subcutaneous finger cardioverter-defibrillator in low weight paediatric patients: a case series.

Placement of traditional transvenous implantable cardioverter defibrillator (ICD) system in low-weight children is often difficult because of their vessel size, the elevated risk of lead malfunction and failure, children's growth and various anatomic constraints, creating the need for alternative solutions. Subcutaneous array leads combined with an abdominally placed ICD device can minimize the surgical approach. In this case series, we analyse the data behind indications for subcutaneous finger cardioverter defibrillator (SFCD) and discuss the preliminary clinical experience in low-weight children. We considered 4 consecutive children (mean age 3.9 years, range 3-5.5 years, mean body weight 17.6 Kg, range 14-23 Kg) who underwent SFCD implant from April 2016 to August 2020. All patients showed a good compliance to the device system with no complications (infections or skin erosions). No patients experienced in the observation period (mean time 44.5±21.5 months) sustained ventricular arrhythmias requiring shocks. No inappropriate shocks released by the device occurred. No significant changes were observed in LET (lowest energy tested) performed around 24 months of follow-up. All patients showed a good compliance and stable atrio-ventricular sensing and pacing thresholds. In smaller children in whom a transvenous approach is not feasible or not possible for anatomic reasons, the SFCD appears to be a safe method to prevent SCD with little surgical trauma and preservation of an intact vascular system, providing an adequate bridge to transvenous ICD or subcutaneous ICD implant late in the life.

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.

Retrieval of a leadless transcatheter pacemaker from the right pulmonary artery: A case report.

This case report describes a procedure of retrieval of a leadless transcatheter pacemaker from the right pulmonary artery 3 days after device implantation. An 80-year-old man affected by ischemic cardiomyopathy and sinus node dysfunction was implanted with a leadless transcatheter pacemaker in the low septum of the right ventricle. Three days after the procedure, the leadless pacemaker migrated into a side branch of the right pulmonary artery. The device was successfully removed using two snares hooked to a tine via the proximal retrieval feature.

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.

The role of 3D-high definition mapping systems in treating postoperative drug-resistant intra-atrial reentrant tachycardia.

We report the case of a patient with recurrent episodes of postoperative intra-atrial reentrant tachycardia occurring after surgical patch closure of a sinus venosus atrial septal defect (SVASD). Arrhythmic episodes, as usually reported in scar-related arrhythmias, were resistant to antiarrhythmic therapy and worsened patient's quality of life. Therefore, we decided to perform an intracardiac electrophysiology study using a 3D-high definition mapping system to identify and treat the precise arrhythmic substrate with radiofrequency ablation. There is a growing evidence that this technique represents a valid therapeutic option in postoperative arrhythmias refractory to medical treatment in patients with repaired congenital heart disease.

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.

Echocardiographic assessment of right ventricular contractile reserve in healthy subjects.

BACKGROUND: Exercise-induced increase in pulmonary artery systolic pressure (PASP) as a possible measure of right ventricular (RV) contractile reserve has been shown to predict survival in severe pulmonary hypertension. However, RV contractile reserve can also be measured by changes in stroke volume (SV), tricuspid annular plane systolic excursion (TAPSE), or tricuspid annular systolic velocity (S'). The limits of normal values and the functional significance of these changes in healthy subjects are not well known. METHODS: In this prospective study, 90 healthy subjects (45 male, mean age 39 ± 13 years) underwent exercise stress echocardiography with measurement of TAPSE, S', TAPSE/PASP, SV, and PASP at rest and peak exercise. Maximum and minimum normal values were reported for all indices. RESULTS: Normal values of exercise-induced changes (Δ) were 4 to 10 mm for TAPSE, 6 to 14 cm/s for S', 12 to 57 mm Hg for PASP, 0 to 96 mL for SV, and -1.2 to 0 mm/mm Hg for TAPSE/PASP. At peak exercise, women showed lower ΔTAPSE/PASP, ΔPASP, ΔS', and ΔSV, but higher TAPSE/PASP than men. Aging was associated with decreased ΔTAPSE/PASP, ΔTAPSE, ΔS', ΔPASP, and ΔSV. In addition, ΔS', ΔTAPSE/PASP, ΔPASP, and ΔSV, but not ΔTAPSE, were directly correlated with maximum workload. CONCLUSIONS: Our results provide age- and sex-related limits of normal for RV contractile reserve as assessed by exercise stress echocardiography and demonstrate that RV systolic function indices (PASP, TAPSE, S', and TAPSE/PASP) correlate with maximum exercise capacity.

Irrigated contact force sensing catheter for redo ablation of slow-fast atrioventricular nodal reentrant tachycardia in pediatric and adolescent patients: A case series.

Conventional nonirrigated catheters cannot be able to create adequate lesions for effective slow pathway modulation in certain cases of pediatric atrioventricular nodal reentrant tachycardia ablation. Irrigated contact force sensing catheters may be considered in pediatric and adolescent patients to obtain a more extensive slow pathway modulation for redo ablation, avoiding dangerous radiofrequency applications close to the compact atrioventricular node or complex left-sided procedures.

Combined subcutaneous implantable cardioverter defibrillator and pacemaker devices in complex congenital heart disease: a single-center experienced based study.

BACKGROUND: Subcutaneous implantable cardioverter defibrillators (S-ICD) are widely accepted therapy in congenital heart disease (CHD) patients at risk of life-threatening ventricular arrhythmias or sudden cardiac death (SCD) when pacing is not required. Occasionally, pacemaker (PM)-dependent CHD patients will subsequently develop an indication for a cardioverter defibrillator. The use of S-ICD in complex CHD patients who have had already PM devices implanted implies some specific considerations, as the safety for these patients in unknown and recommendations among physicians may vary widely. METHODS: We review the data and studied the indications for S-ICD in complex CHD with previous PM and discuss its usefulness in clinical practice. RESULTS: From a large cohort of 345 patients enrolled in the S-ICD Monaldi care registry, which encompass all the patients implanted in the Monaldi Hospital of Naples, we considered 11 consecutive complex CHD patients (10M/1F aged 40.4 ±18.4 years) who underwent S-ICD implant after a previous PM implant, from February 2015 to October 2022. Mean follow-up was 25.5 ± 22 months. All the patients showed a good compliance to the device system with no complications (infections or skin erosions). CONCLUSIONS: In complex CHD with already implanted PM devices, S-ICD implant appears to be a safe alternative to PM upgrading to transvenous ICD system, avoiding abandoned leads or life-threatening lead extraction. However, there are important issues with regard to testing and programming that need to be addressed at the time of implantation.

Subcutaneous implantable cardioverter defibrillator in children and adolescents: results from the S-ICD "Monaldi care" registry.

BACKGROUND: Implantable cardioverter defibrillators (ICD) are widely accepted therapy in children and adolescents who are survivors of cardiac arrest or for high-risk patients with inheritable channelopathies, cardiomyopathies, or congenital heart disease. Initial experience with subcutaneous ICD (S-ICD) systems has shown a high efficacy in adults. However, the use of S-ICD in children and adolescents implies some specific considerations, as the safety for these patients is unknown and recommendations among physicians may vary widely. METHODS: We reviewed the data and studied the indications for S-ICD in children and adolescents and discuss the preliminary clinical experience. RESULTS: From a cohort of 297 patients enrolled in the S-ICD "Monaldi care" registry that encompass all the patients implanted in the Monaldi Hospital of Naples, we considered 21 consecutive children and adolescents (mean age 13.9 years, range 8-18 years, mean body weight 59.3 kg, range 38-100 kg) who underwent S-ICD implant from April 2014 to June 2020. Mean follow-up was 41.9±21.9 months. Only one patient presented, 6 weeks after implantation, skin erosion at the inferior parasternal incision that resolved after antibiotic therapy, without the necessity of any system revision. Two patients experienced appropriate shocks and four inappropriate shocks, due to T wave oversensing or atrial arrhythmia. Only one patient, with arrhythmogenic right ventricular dysplasia, required a system revision after 36 months of the first implantation and then a reintervention with a replacement of the S-ICD by a conventional ICD system. CONCLUSIONS: Our experience suggests that the S-ICD device can be used in some children over the age of 8 as well as adults, with a similar rate of unwanted side effects, and early evidence of apparent efficacy.

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.).

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.