Transcatheter Pulmonary Valve Implantation in Congenital Heart Diseases: Current Advances and Future Prospectives.

Pulmonary disfunction is frequent in repaired congenital heart diseases. Both pulmonary regurgitation and pulmonary stenosis are possible complications over time. In the past, the surgical approach was the only feasible management but exposed the patient to a redo-surgery with its consequent risks.

Sacubitril/Valsartan and Dapagliflozin in Patients with a Failing Systemic Right Ventricle: Effects on the Arrhythmic Burden.

: Angiotensin receptor neprilysin inhibitor (ARNI) and sodium-glucose co-transporter 2 inhibitors (SGLT2i) are essential medications in heart failure (HF) therapy, and their potential antiarrhythmic effects have been reported. Recently, ARNI and SGLT2i use for HF in adult congenital heart disease (ACHD) has been studied. However, whether any beneficial effects may be achieved on the arrhythmic burden in the complex population of ACHD with a systemic right ventricle (sRV) is still to be determined.

The Unique Challenge of Coronary Artery Disease in Adult Patients with Congenital Heart Disease.

Advances in medical and surgical interventions have resulted in a steady increase in the number of patients with congenital heart disease (CHD) reaching adult age. Unfortunately, this ever-growing population faces an added challenge: an increased risk of acquiring coronary artery disease. This review provides insight into the complex interactions between coronary artery disease and CHD in adults.

Clinical presentation and genetic characterization of early-onset atrial fibrillation in patients affected by long QT syndrome: A single-center experience.

Introduction: Early-onset atrial fibrillation (AF) has already been observed in approximately 2% of patients with genetically proven long QT syndrome (LQTS). This frequency is higher than population-based estimates of early-onset AF. However, the concomitant expression of AF in LQTS is likely underestimated.

Antiarrhythmic therapy for narrow QRS supraventricular tachyarrhythmias in newborns and infants in the first year of life: Potent tools to be handled with care.

Supraventricular tachyarrhythmias pose a significant challenge in neonates and infants, particularly within the first year of life, where prompt and effective management is crucial. By synthesizing available evidence and clinical experience, this review aims to provide a comprehensive overview of antiarrhythmic therapy in this vulnerable population, with a focus on narrow QRS supraventricular tachyarrhythmias. This review examines the current understanding of supraventricular tachyarrhythmia management and discusses the challenges associated with antiarrhythmic therapy in newborns and infants during the critical first year of life, evaluating the efficacy and safety of various antiarrhythmic agents commonly utilized in this population, including dosing considerations, adverse effects, and strategies for acute management and prophylactic long-term antiarrhythmic treatment.

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.

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.

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.

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.

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.

Contact force sensing radiofrequency catheter ablation guided by electroanatomic mapping in pediatric patients with permanent junctional reciprocating tachycardia: A case series.

Limited data are available on the use of irrigated contact force (CF) catheters for radiofrequency (RF) ablation of permanent junctional reciprocating tachycardia (PJRT) in children. We considered five consecutive pediatric patients with diagnosis of PJRT who underwent RF ablation with irrigated CF catheter guided by electroanatomic mapping, obtaining a low number of RF pulses and minimal fluoroscopy exposure. High CF values are not necessary to obtain effective RF lesions and successful ablation.

Myocardial work in children with Wolff-Parkinson-White syndrome.

Wolff-Parkinson-White Syndrome (WPW) has been associated with reduced local myocardial deformation, and when left ventricular dysfunction is present, catheter ablation of the accessory pathway may be required, even in asymptomatic patients. We aimed to evaluate the diagnostic value of non-invasive myocardial work in predicting subtle abnormalities in myocardial performance in children with WPW.Seventy-five paediatric patients (age 8.

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.

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.

Pharmacological therapy in adult congenital heart disease with coronary artery disease and atrial fibrillation.

Thanks to advances in both medical care and surgical techniques, over recent years, survival for patients with congenital heart disease (CHD) has increased significantly, with about 90% of patients now reaching adulthood. However, as CHD patients grow older, their relative risk of developing coronary artery disease (CAD) increases. Moreover, it has been demonstrated that the majority of adult congenital heart disease (ACHD) patients has at least one cardiovascular risk factor.

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.

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.

Leadless pacemaker in Twiddler's syndrome: A case report.

Twiddler's syndrome is a rare cause of pacemaker lead dislodgement. We present the case of a 49-year-old male patient with Down's syndrome implanted with a dual chamber pacemaker showing high ventricular impedance, no sensing, and complete loss of capture for both leads at the 3-month follow-up due to Twiddler Syndrome. The dislocated device was removed, and an endocardial leadless pacing system was implanted.

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.

Early evaluation of atrial high rate episodes using remote monitoring in pacemaker patients: Results from the RAPID study.

Aim: Remote monitoring (RM) of implantable cardiac devices has enabled continuous surveillance of atrial high rate episodes (AHREs) with well-recognized clinical benefits. We aimed to add evidence on the role of the RM as compared to conventional follow-up by investigating the interval from AHRE onset to physician's evaluation and reaction time in actionable episodes.

Methods And Results: A total of 97 dual-chamber pacemaker recipients were followed with RM (RM-ON group;  = 64) or conventional in-office visits (RM-OFF group;  = 33) for 18 months.

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.

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. ().