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View article: Integrating Genetic, Clinical, and Histopathological Data for Definitive Diagnosis of PRKAG2-Related Disease
Integrating Genetic, Clinical, and Histopathological Data for Definitive Diagnosis of PRKAG2-Related Disease Open
Background: PRKAG2-related disease is an autosomal dominant disorder caused by pathogenic variants in the PRKAG2 gene, leading to glycogen accumulation in cardiomyocytes. It is characterized by left ventricular hypertrophy (LVH), ventricul…
View article: Sacubitril/Valsartan and Dapagliflozin in Patients with a Failing Systemic Right Ventricle: Effects on the Arrhythmic Burden
Sacubitril/Valsartan and Dapagliflozin in Patients with a Failing Systemic Right Ventricle: Effects on the Arrhythmic Burden Open
Background/Objectives: 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 r…
View article: Comprehensive Diagnostic Work-Up for Uncovering the Causes of Sudden Cardiac Death: The Role of Family Members
Comprehensive Diagnostic Work-Up for Uncovering the Causes of Sudden Cardiac Death: The Role of Family Members Open
Background: The aim of this study was to evaluate the performance of the diagnostic pathway proposed by the European Society of Cardiology (ESC) guidelines for identifying the underlying aetiology of sudden cardiac death (SCD) through the …
View article: Congenital Atrio-Ventricular Block: looking for optimal implantation timing. A single tertiary referral centre experience
Congenital Atrio-Ventricular Block: looking for optimal implantation timing. A single tertiary referral centre experience Open
Background Congenital atrio-ventricular block (CAVB) affects 1 in 15,000 live births with a mortality rate of 16–30%. It can be associated with congenital heart disease (CHD) or maternal autoantibodies and, less commonly, with viral infect…
View article: Antiarrhythmic therapy for narrow QRS supraventricular tachyarrhythmias in newborns and infants in the first year of life: Potent tools to be handled with care
Antiarrhythmic therapy for narrow QRS supraventricular tachyarrhythmias in newborns and infants in the first year of life: Potent tools to be handled with care Open
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 experienc…
View article: Prevalence and Clinical Significance of Intraventricular Conduction Disturbances in Hospitalized Children
Prevalence and Clinical Significance of Intraventricular Conduction Disturbances in Hospitalized Children Open
Introduction: Data on the prevalence and clinical significance of interventricular conduction disturbances (IVCDs) in children are scarce. While incomplete right bundle branch blocks (IRBBBs) seem to be the most frequent and benign finding…
View article: New Guidelines of Pediatric Cardiac Implantable Electronic Devices: What Is Changing in Clinical Practice?
New Guidelines of Pediatric Cardiac Implantable Electronic Devices: What Is Changing in Clinical Practice? Open
Guidelines are important tools to guide the diagnosis and treatment of patients to improve the decision-making process of health professionals. They are periodically updated according to new evidence. Four new Guidelines in 2021, 2022 and …
View article: Myocardial work in systemic right ventricle: new insights on right ventricular adaptation to systolic flow
Myocardial work in systemic right ventricle: new insights on right ventricular adaptation to systolic flow Open
Background Myocardial work (MW) is an innovative tool, which combines global longitudinal analysis with non-invasively assessed ventricular pressure to provide a measure of ventricular performance. This technique has recently been used in …
View article: Dapaglifozin in adults with a systemic right ventricle:initial results from the DAPA-SERVE trial
Dapaglifozin in adults with a systemic right ventricle:initial results from the DAPA-SERVE trial Open
Background Sodium-glucose Cotransporter-2(SGLT2) inhibitors are currently recommended as first line therapy for all patients with heart failure with reduced ejection fraction (HFrEF).Adults with a systemic right ventricle (sRV) may develop…
View article: Remote monitoring of implantable loop recorders reduces time to diagnosis in patients with unexplained syncope: a multicenter propensity score-matched study
Remote monitoring of implantable loop recorders reduces time to diagnosis in patients with unexplained syncope: a multicenter propensity score-matched study Open
Background There are little data on remote monitoring (RM) of implantable loop recorders (ILRs) in patients with unexplained syncope and whether it confers enhanced diagnostic power. Objective To evaluate the effect of RM in ILR recipients…
View article: Diagnosis and treatment of fetal and pediatric age patients (0–12 years) with Wolff–Parkinson–White syndrome and atrioventricular accessory pathways
Diagnosis and treatment of fetal and pediatric age patients (0–12 years) with Wolff–Parkinson–White syndrome and atrioventricular accessory pathways Open
Overt or concealed accessory pathways are the anatomic substrates of ventricular preexcitation (VP), Wolff–Parkinson–White syndrome (WPW) and paroxysmal supraventricular tachycardia (PSVT). These arrhythmias are commonly observed in pediat…
View article: Contact force sensing radiofrequency catheter ablation guided by electroanatomic mapping in pediatric patients with permanent junctional reciprocating tachycardia: A case series
Contact force sensing radiofrequency catheter ablation guided by electroanatomic mapping in pediatric patients with permanent junctional reciprocating tachycardia: A case series Open
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 d…
View article: Combined subcutaneous implantable cardioverter defibrillator and pacemaker devices in complex congenital heart disease. A single-center experienced based study.
Combined subcutaneous implantable cardioverter defibrillator and pacemaker devices in complex congenital heart disease. A single-center experienced based study. Open
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 i…
View article: Implantable Loop Recorder with Long Sensing Vector: Safety, Acceptability, and Sensing Performance in Pediatric Patients
Implantable Loop Recorder with Long Sensing Vector: Safety, Acceptability, and Sensing Performance in Pediatric Patients Open
View article: COVID-19 vaccination in adults with congenital heart disease: results of 1-year prospective study
COVID-19 vaccination in adults with congenital heart disease: results of 1-year prospective study Open
Background Adults with congenital heart disease (ACHD) are a vulnerable population. Routine vaccination is the only strategy to prevent a life-threatening infection. However, concerns on the cardiac safety and efficacy of COVID-19 vaccines…
View article: Myocardial work impairment in children with Wolff-Parkinson-White syndrome
Myocardial work impairment in children with Wolff-Parkinson-White syndrome Open
Background Wolf-Parkinson-White Syndrome (WPW) has been associated with reduced local myocardium deformation during systole. According to guidelines, when the left ventricle (LV) dysfunction is present catheter ablation of the accessory pa…
View article: Effects of sacubitril/valsartan in patients with a systemic right ventricle
Effects of sacubitril/valsartan in patients with a systemic right ventricle Open
Background Sacubitril/valsartan has been proved to reduce mortality in heart failure and reduced ejection fraction (EF) and is currently recommended as first-line therapy. However, effects in patients with a systemic right ventricle (sRV) …
View article: Severe Lymphatic Disorder and Multifocal Atrial Tachycardia Treated with Trametinib in a Patient with Noonan Syndrome and SOS1 Mutation
Severe Lymphatic Disorder and Multifocal Atrial Tachycardia Treated with Trametinib in a Patient with Noonan Syndrome and SOS1 Mutation Open
Noonan syndrome (NS) is a multisystemic disorder caused by germline mutations in the Ras/MAPK cascade, causing a broad spectrum of phenotypical abnormalities, including abnormal facies, developmental delay, bleeding diathesis, congenital h…
View article: Subcutaneous finger cardioverter-defibrillator in low weight paediatric patients: a case series
Subcutaneous finger cardioverter-defibrillator in low weight paediatric patients: a case series Open
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 vario…
View article: Wearable cardioverter-defibrillator (life-vest): A feasible bridging treatment in adult congenital heart disease
Wearable cardioverter-defibrillator (life-vest): A feasible bridging treatment in adult congenital heart disease Open
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 surger…
View article: Myocardial work indices and ventricular dyssynchrony in adults with aortic coarctation
Myocardial work indices and ventricular dyssynchrony in adults with aortic coarctation Open
Funding Acknowledgements Type of funding sources: None. Background Adults with coarctation of the aorta (CoA) may present residual aortic obstruction and develop arterial vasculopathy and subclinical left ventricular (LV) dysfunction. Myoc…
View article: Cardiac resynchronization therapy-defibrillator implantation guided by electroanatomic mapping in a young adult patient with congenital heart disease
Cardiac resynchronization therapy-defibrillator implantation guided by electroanatomic mapping in a young adult patient with congenital heart disease Open
View article: Safety of Omitting Defibrillation Efficacy Testing With Subcutaneous Defibrillators: A Propensity-Matched Case-Control Study
Safety of Omitting Defibrillation Efficacy Testing With Subcutaneous Defibrillators: A Propensity-Matched Case-Control Study Open
No abstract available
View article: 276 Wolff–Parkinson–White syndrome was associated with reduced myocardial work
276 Wolff–Parkinson–White syndrome was associated with reduced myocardial work Open
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…
View article: Prognostic relevance of thyroid disease in adults with congenital heart disease
Prognostic relevance of thyroid disease in adults with congenital heart disease Open
Background Adults with congenital heart disease (ACHD) are frequently affected by thyroid diseases (TD). However, the clinical relevance of TD in ACHD remains unknown. Purpose We aimed to describe the prevalence of TD in the ACHD populatio…
View article: Effects of sacubitril/valsartan in patients with a systemic right ventricle: early evidence of exercise tolerance and systolic function improvement
Effects of sacubitril/valsartan in patients with a systemic right ventricle: early evidence of exercise tolerance and systolic function improvement Open
Background Sacubitril/valsartan has been shown to reduce mortality and morbidity inpatients with heart failure and reduced systolic function. However, the effects of this novel association in patients with congenital heart disease and a sy…
View article: Coronavirus disease 2019 in patients with Fontan circulation
Coronavirus disease 2019 in patients with Fontan circulation Open
View article: Subcutaneous implantable cardioverter defibrillator in complex adult congenital heart disease. Results from the S-ICD “Monaldi Care” registry
Subcutaneous implantable cardioverter defibrillator in complex adult congenital heart disease. Results from the S-ICD “Monaldi Care” registry Open
View article: Pulmonary valve endocarditis in adults with congenital heart disease: the role of echocardiography in a case series
Pulmonary valve endocarditis in adults with congenital heart disease: the role of echocardiography in a case series Open
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 descr…
View article: Challenges and Growth as a Mental Health Professional from Volunteering Experiences in the Community Gambling Awareness Campaign
Challenges and Growth as a Mental Health Professional from Volunteering Experiences in the Community Gambling Awareness Campaign Open
Adults with congenital heart disease (ACHD) constitute a growing population with complex cardiac physiopathology and frequent extra-cardiac involvement. The recent dramatic improvement of their life expectancy has resulted in an increasing…