Heterozygous mutations in the C-terminal domain of COPA underlie a complex autoinflammatory syndrome.

Mutations in the N-terminal WD40 domain of coatomer protein complex subunit α (COPA) cause a type I interferonopathy, typically characterized by alveolar hemorrhage, arthritis, and nephritis. We described 3 heterozygous mutations in the C-terminal domain (CTD) of COPA (p.C1013S, p.

Dynamic changes of left atrial substrate over time following pulmonary vein isolation: the Progress-AF study.

Aims: Little is known about dynamic changes of the left atrial (LA) substrate over time in patients with atrial fibrillation (AF). This study aims to evaluate substrate changes following pulmonary vein isolation (PVI).

Methods And Results: In our prospective observational study, consecutive patients undergoing first PVI-only and redo ablation were included.

Atrial fibrillation cycle-length (AF-CL) inside the pulmonary veins predicts paroxysmal AF ablation success- the INDUCE-AF study.

Background: A novel method to measure atrial fibrillation cycle length (AF-CL) was recently described, based on the average of 10 consecutive signals (FARS10). FARS10 accurately identified pulmonary vein isolation (PVI)-responders among patients with persistent AF. Whether this method is applicable to patients with paroxysmal AF is unknown.

Inactivating TDP2 missense mutation in siblings with congenital abnormalities reminiscent of fanconi anemia.

Mutations in TDP2, encoding tyrosyl-DNA phosphodiesterase 2, have been associated with a syndromal form of autosomal recessive spinocerebellar ataxia, type 23 (SCAR23). This is a very rare and progressive neurodegenerative disorder described in only nine patients to date, and caused by splice site or nonsense mutations that result in greatly reduced or absent TDP2 protein. TDP2 is required for the rapid repair of DNA double-strand breaks induced by abortive DNA topoisomerase II (TOP2) activity, important for genetic stability in post-mitotic cells such as neurons.

Gender-Related Differences in Atrial Substrate in Patients with Atrial Fibrillation.

Gender-related differences have been reported in patients who underwent pulmonary vein isolation (PVI). Atrial substrate plays a role in the outcomes after ablation but gender-related differences in atrial substrate have never been described in detail. We sought to analyze gender-related differences in atrial remodeling (spontaneous low-voltage zones [LVZs]) and their clinical relevance after PVI.

Left Bundle Branch Area Defibrillator (LBBAD): A First-in-Human Feasibility Study.

Background: Left bundle branch area (LBBA) pacing is a promising pacing technique. LBBA implantable cardioverter-defibrillator (ICD) lead implantation reduces the number of leads in patients with both pacing and ICD indications, reducing cost and potentially increasing safety. LBBA positioning of ICD leads has not previously been described.

Conduction system pacing, a European survey: insights from clinical practice.

Aims: The field of conduction system pacing (CSP) is evolving, and our aim was to obtain a contemporary picture of European CSP practice.

Methods And Results: A survey was devised by a European CSP Expert Group and sent electronically to cardiologists utilizing CSP. A total of 284 physicians were invited to contribute of which 171 physicians (60.

Anterior mitral line in patients with persistent atrial fibrillation and anterior scar: A multicenter matched comparison-The MiLine study.

Background: The benefit of an anterior mitral line (AML) in patients with persistent atrial fibrillation (AF) and anterior atrial scar undergoing ablation has never been investigated.

Objective: The purpose of this study was to evaluate the outcomes of AML in addition to standard treatment compared to standard treatment alone (no AML) in this subset of patients.

Methods: Patients with persistent AF and anterior low-voltage zone (LVZ) treated with AML in 3 centers were retrospectively enrolled.

Electrocardiographic phenotype of exercise-induced arrhythmogenic cardiomyopathy: A retrospective observational study.

Introduction: The right ventricle can be susceptible to pathologic alterations with exercise. This can cause changes to the ECG. Our aim was to identify the electrocardiographic phenotype of exercise induced (ExI) arrhythmogenic cardiomyopathy (ACM).

Left bundle branch area pacing outcomes: the multicentre European MELOS study.

Aims: Permanent transseptal left bundle branch area pacing (LBBAP) is a promising new pacing method for both bradyarrhythmia and heart failure indications. However, data regarding safety, feasibility and capture type are limited to relatively small, usually single centre studies. In this large multicentre international collaboration, outcomes of LBBAP were evaluated.

Rescue left bundle branch area pacing in coronary venous lead failure or nonresponse to biventricular pacing: Results from International LBBAP Collaborative Study Group.

Background: Cardiac resynchronization therapy (CRT) using biventricular pacing (BVP) is effective in patients with heart failure, left bundle branch block (LBBB), and reduced left ventricular function. Left bundle branch area pacing (LBBAP) has been reported as an alternative option for CRT.

Objective: The purpose of this study was to assess the feasibility and outcomes of LBBAP in patients who failed conventional BVP because of coronary venous (CV) lead complications or who were nonresponders to BVP.

A new prediction model for left ventricular systolic function recovery after catheter ablation of atrial fibrillation in patients with heart failure: The ANTWOORD Study.

Background: in patients with heart failure (HF) and atrial fibrillation (AF), AF ablation improves left ventricular ejection fraction (LVEF), along with prognosis, in a variable percentage of patients. We aimed to investigate the predictors of LVEF recovery after AF ablation and to develop a prediction model for individualized assessment.

Methods: we conducted an observational, retrospective, single-centre study on 111 consecutive patients with AF and HF with impaired LVEF (<50%) undergoing ablation.

Reproducibility and predictive value of a simple novel method to measure atrial fibrillation cycle length in persistent atrial fibrillation - FARS-AF study.

Introduction: Different methods are used for atrial fibrillation (AF) cycle length (CL) measurement with variable results. Previous studies of pulmonary vein (PV) CL measurement showed contradictory results on predicting PV isolation (PVI) efficacy. A novel simple method of measuring the average of 10 consecutive Fastest Atrial Repetitive Similar morphology signal (FARS )-CL to characterize local atrial activity rate was evaluated prospectively.

Etiology and Outcome of Catheter Ablation in Patients With Onset of Atrial Fibrillation <45 Years of Age.

Etiologic factors and long-term outcomes of catheter ablation of atrial fibrillation (AF) in young patients have not been well characterized. This study aimed to analyze the etiologic factors and outcomes of pulmonary vein isolation (PVI) in patients with young-onset AF (young-AF, defined as having first documented episode <45 years). Consecutive patients with young-AF undergoing PVI (n = 197) in 2 academic centers were enrolled and followed for 36.

Left bundle branch-optimized cardiac resynchronization therapy (LOT-CRT): Results from an international LBBAP collaborative study group.

Background: Cardiac resynchronization therapy (CRT) based on the conventional biventricular pacing (BiV-CRT) technique sometimes results in broad QRS complex and suboptimal response.

Objective: We aimed to assess the feasibility and outcomes of CRT based on left bundle branch area pacing (LBBAP, in lieu of the right ventricular lead) combined with coronary venous left ventricular pacing in an international multicenter study.

Methods: LBBAP-optimized CRT (LOT-CRT) was attempted in nonconsecutive patients with CRT indications.

Intensive recreational athletes in the prospective multinational ICD Sports Safety Registry: Results from the European cohort.

Background: In the ICD Sports Safety Registry, death, arrhythmia- or shock-related physical injury did not occur in athletes who continue competitive sports after implantable cardioverter-defibrillator (ICD) implantation. However, data from non-competitive ICD recipients is lacking. This report describes arrhythmic events and lead performance in intensive recreational athletes with ICDs enrolled in the European recreational arm of the Registry, and compares their outcome with those of the competitive athletes in the Registry.

Differential presentation of atrioventricular nodal re-entrant tachycardia in athletes and non-athletes.

Aims: Prolonged participation in exercise results in structural and electrical cardiac remodelling. The development of an athlete's heart is recognized as a risk factor for atrial arrhythmias. This study aims to evaluate the impact of athlete heart remodelling on the presentation of atrioventricular nodal re-entrant tachycardia (AVNRT).

Less Pulmonary Vein Reconnection at Redo Procedures Following Radiofrequency Point-by-Point Antral Pulmonary Vein Isolation With the Use of Contemporary Catheter Ablation Technologies.

Objectives: This study investigated whether real-world use of contemporary technologies changed pulmonary vein (PV) reconnection and redo pulmonary vein isolation (PVI) procedure frequencies.

Background: Previous studies consistently reported that following PVI recurrence of PV conduction is observed in >80% of patients.

Methods: Consecutive patients undergoing 529 first and/or redo radiofrequency point-by-point PVI between January 2013 and December 2016 were included.

Case report, aetiology, and treatment of an acquired long-QT syndrome.

Acquired long-QT syndrome is an iatrogenic disorder, usually induced by drugs, which can cause life-threatening arrhythmias. We present a case report on an acquired long-QT syndrome with an interesting confluence of circumstances, and comment on aetiology and treatment.

Undersensing of ventricular fibrillation due to interference between a pacemaker and defibrillator in the same patient.

Sensing in pacemakers (PMs) and implantable cardioverter defibrillators (ICDs) is crucial to normal device behaviour. Since both devices treat different arrhythmias, the technical approach to signal detection is also completely different. A PM has a fixed threshold of sensing, above which events are sensed and therapy of the device withheld.

Pacemaker-mediated tachycardia with varying cycle length: what is the mechanism?

As new algorithms are being developed to promote intrinsic atrioventricular conduction in preventing the deleterious effects of right ventricular pacing, more complex rhythm strips can be encountered. In our patient with a dual-chamber implantable cardioverter-defibrillator, such an algorithm resulted in a pacemaker-mediated tachycardia with several changes in cycle length.

Adenosine-induced ventricular asystole or rapid ventricular pacing to enhance three-dimensional rotational imaging during cardiac ablation procedures.

Aims: Rotational angiography with digital three-dimensional reconstruction (3DRA) allows per-procedural 3D imaging to facilitate cardiac ablation procedures. We developed a new approach that allows per-procedural 3D imaging of the atria and ventricles with a single C-arm rotation, combining higher 3D image quality with a lower contrast and radiation dose.

Methods And Results: Forty patients underwent 3DRA of the left atrium (LA, n = 26), right atrium (RA, n = 11), left ventricle (LV, n = 2), or right ventricle (RV, n = 1) during ablation procedures performed under general anaesthesia.

Biplane three-dimensional augmented fluoroscopy as single navigation tool for ablation of atrial fibrillation: accuracy and clinical value.

Background: We developed new methods for real time biplane integration of three-dimensional (3D) left atrial models with fluoroscopic images to assist in catheter ablation of atrial fibrillation (AF).

Objective: The purpose of this study was to quantitatively assess the accuracy of 3D fluoroscopy integration and to evaluate its clinical value when used as a single navigation tool for AF ablation.

Methods: Sixty patients underwent AF ablation under biplane fluoroscopic guidance after selective angiography of the four pulmonary veins.