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.

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.

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.

End-stage heart failure in congenitally corrected transposition of the great arteries: a multicentre study.

BACKGROUND AND AIMS: For patients with congenitally corrected transposition of the great arteries (ccTGA), factors associated with progression to end-stage congestive heart failure (CHF) remain largely unclear. METHODS: This multicentre, retrospective cohort study included adults with ccTGA seen at a congenital heart disease centre. Clinical data from initial and most recent visits were obtained. The composite primary outcome was mechanical circulatory support, heart transplantation, or death. RESULTS: From 558 patients (48% female, age at first visit 36 ± 14.2 years, median follow-up 8.7 years), the event rate of the primary outcome was 15.4 per 1000 person-years (11 mechanical circulatory support implantations, 12 transplantations, and 52 deaths). Patients experiencing the primary outcome were older and more likely to have a history of atrial arrhythmia. The primary outcome was highest in those with both moderate/severe right ventricular (RV) dysfunction and tricuspid regurgitation (n = 110, 31 events) and uncommon in those with mild/less RV dysfunction and tricuspid regurgitation (n = 181, 13 events, P < .001). Outcomes were not different based on anatomic complexity and history of tricuspid valve surgery or of subpulmonic obstruction. New CHF admission or ventricular arrhythmia was associated with the primary outcome. Individuals who underwent childhood surgery had more adverse outcomes than age- and sex-matched controls. Multivariable Cox regression analysis identified older age, prior CHF admission, and severe RV dysfunction as independent predictors for the primary outcome. CONCLUSIONS: Patients with ccTGA have variable deterioration to end-stage heart failure or death over time, commonly between their fifth and sixth decades. Predictors include arrhythmic and CHF events and severe RV dysfunction but not anatomy or need for tricuspid valve surgery.

Against all odds: successful pregnancy in an adult patient with unrepaired coarctation of the aorta.

According to the modified World Health Organization classification, pregnant women with unrepaired aortic coarctation are at very high risk for both maternal and fetal complications and should, therefore, be counselled against pregnancy. The most frequent maternal complications include systemic hypertension, renal failure, preeclampsia, and aortic dissection. Herein, we describe a successful pregnancy in an adult patient with unrepaired aortic coarctation.

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.

A new score for life-threatening ventricular arrhythmias and sudden cardiac death in adults with transposition of the great arteries and a systemic right ventricle.

AIMS: To investigate the incidence of major adverse ventricular arrhythmias and related events (MAREs) and to develop a stratification tool predicting MAREs in adults with a systemic right ventricle (sRV). METHODS AND RESULTS: In a multicentre approach, all adults (≥16 years old) with a sRV undergoing follow-up between 2000 and 2018 were identified. The incidence of MAREs, defined as sudden cardiac death, sustained ventricular tachycardia, and appropriate implantable cardioverter-defibrillator (ICD) therapy, was analysed. The association of MAREs with clinical, electrical, and echocardiographic parameters was evaluated. A total of 1184 patients (median age 27.1 years; interquartile range 19.9-34.9 years; 59% male; 70% with atrial switch repair for D-transposition of the great arteries) were included. The incidence of MAREs was 6.3 per 1000 patient-years. On multivariate analysis, age, history of heart failure, syncope, QRS duration, severe sRV dysfunction and at least moderate left ventricular outflow tract obstruction were retained in the final model with a C-index of 0.78 [95% confidence interval (CI) 0.72-0.83] and a calibration slope of 0.93 (95% CI 0.64-1.21). For every five ICDs implanted in patients with a 5-year MARE risk >10%, one patient may potentially be spared from a MARE. CONCLUSION: Sudden cardiac death remains a devastating cause of death in a contemporary adult cohort with a sRV. A prediction model based on clinical, electrocardiographic, and echocardiographic parameters was devised to estimate MARE risk and to identify high-risk patients who may benefit from primary prevention ICD implantation.

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.

The Risk of Sudden Unexpected Cardiac Death in Children: Epidemiology, Clinical Causes, and Prevention.

"Sudden unexplained death (SUD) is a tragic event for both the family and community, particularly when it occurs in young individuals. Sudden cardiac death (SCD) represents the leading form of SUD and is defined as an unexpected event without an obvious extracardiac cause, occurring within 1 hour after the onset of symptoms. In children, the main causes of SCD are inherited cardiac disorders, whereas coronary artery diseases (congenital or acquired), congenital heart diseases, and myocarditis are rare. The present review examines the current state of knowledge regarding SCD in children, discussing the epidemiology, clinical causes, and prevention strategies."

Clinical Manifestations of 22q11.2 Deletion Syndrome.

DiGeorge syndrome (DGS), also known as "22q11.2 deletion syndrome" (22q11DS) (MIM # 192430 # 188400), is a genetic disorder caused by hemizygous microdeletion of the long arm of chromosome 22. In the last decades, the introduction of fluorescence in situ hybridization assays, and in selected cases the use of multiplex ligation-dependent probe amplification, has allowed the detection of chromosomal microdeletions that could not be previously identified using standard karyotype analysis. The aim of this review is to address cardiovascular and systemic involvement in children with DGS, provide genotype-phenotype correlations, and discuss their medical management and therapeutic options.

Cardiac resynchronization therapy-defibrillator implantation guided by electroanatomic mapping in a young adult patient with congenital heart disease.

Cardiac resynchronization therapy-defibrillator (CRT-D) implantation is a therapeutic option for adult patients with congenital heart disease (CHD), bundle branch block, reduced ejection fraction and symptoms of heart failure. A new implantation approach guided by the electroanatomic mapping (EAM) has been developed to overcome some issues of the standard technique: non-responder patients, high x-ray exposure and use of iodinated contrast medium for coronary sinus angiography. This approach has not been previously described in the CHD population. We report a case of EAM-guided approach for CRT-D implantation in a young adult patient with CHD.

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.

A "long-standing" malpositioned pacing lead. Long-term follow-up after extraction.

Transvenous pacemaker (PM) catheters can be unintentionally placed in the left ventricle (LV) during the implantation procedure. An 8-year-old girl was discovered with a malpositioned pm wire, seven years after the implant. Trans-thoracic echocardiogram revealed the lead traversing the inter-atrial septum, crossing the mitral valve and embedded in the basal lateral wall of the LV. This is a report of a 14-year long follow-up after the surgical extraction of the malpositioned PM lead.