Rachel ter Bekke

Maastricht Universitair Medisch Centrum · Cardiology

Background: Implantation of a permanent pacemaker and atrioventricular (AV) node ablation (pace-and-ablate) is an established approach for rate and symptom control in elderly patients with symptomatic atrial fibrillation (AF). Left bundle branch area pacing (LBBAP) is a physiological pacing strategy that might overcome right ventricular pacing-ind...

Background Juvenile onset of extensive atrial electromechanical failure, including atrial standstill, is a rare disease entity that may precede ventricular cardiomyopathy. Genetic variants associated with early-onset atrioventricular (AV) cardiomyopathy are increasingly recognized. Case summary A 16-year-old patient presented with atrial brady- an...

Funding Acknowledgements Type of funding sources: None. Background Heart failure with preserved ejection fraction (HFpEF) frequently coexists with atrial fibrillation (AF). The gold-standard test for HFpEF diagnosis remains invasive assessment of left ventricular end-diastolic pressures but noninvasive diagnostic scoring systems have been develope...

Funding Acknowledgements Type of funding sources: None. Introduction This study sought to examine the prognostic role of quantifying areas of interest in preinterventional cardiac magnetic resonance (CMR) in patients with structural heart disease (SHD) referred for ablation of ventricular tachycardia (VT). Background The role of preinterventional...

Funding Acknowledgements Type of funding sources: None. Background Catheter ablation (CA) has been established as an effective therapeutic option to achieve rhythm control in patients with atrial fibrillation (AF). However, AF recurrence occurs in approximately 45% of the patients undergoing CA. The association between AF recurrence after CA and l...

Background The “double fire” (DF) atrioventricular (AV) nodal response is a rare mechanism of two ventricular electrical activations following a single atrial beat due to dual AV node physiology. DF AV nodal response is often misdiagnosed and may lead to unnecessary and sometimes risky interventions. Case summary We describe a series of three case...

Sudden cardiac death is often caused by ventricular arrhythmias driven by reentry. Comprehensive characterization of the potential triggers and substrate in survivors of sudden cardiac arrest has provided insights into the trigger-substrate interaction leading to reentry. Previously, a “Triangle of Arrhythmogenesis”, reflecting interactions between...

Objective: To compare observer confidence for myocardial scar detection using 3 different late gadolinium enhancement (LGE) data sets by 2 observers with different levels of experience. Materials and methods: Forty-one consecutive patients, who were referred for 3D dark-blood LGE MRI before implantable cardioverter-defibrillator implantation or...

The EU Horizon 2020 Framework-funded Standardized Treatment and Outcome Platform for Stereotactic Therapy Of Re-entrant tachycardia by a Multidisciplinary (STOPSTORM) consortium has been established as a large research network for investigating STereotactic Arrhythmia Radioablation (STAR) for ventricular tachycardia (VT). The aim is to provide a po...

Introduction Patients with ventricular tachyarrhythmias (VT) are at high risk of sudden cardiac death. When appropriate, catheter ablation is modestly effective, with relatively high VT recurrence and complication rates. Personalized models that incorporate imaging and computational approaches have advanced VT management. However, 3D patient-specif...

Background: The Worm Study, derived from a multi-generation pedigree segregating a single amino-acid deletion in SCN5A (c.4850_4852delTCT, p.(Phe1617del), rs749697698), is characterized by substantial phenotypic heterogeneity and overlap of sudden cardiac death, long-QT syndrome, cardiac conduction disease, Brugada syndrome and isorhythmic atriove...

Background In patients with VT after myocardial infarction (MI), substrate-based ablation is superior to approaches that target clinical and tolerated VTs only. Different substrate modification strategies have been reported. However, proposed ablation targets are prone to operator interpretation (e.g. abnormal electrograms). Accordingly, ablation r...

IntroductionContinuous progress in atrial fibrillation (AF) ablation techniques has led to an increasing number of procedures with improved outcome. However, about 30–50% of patients still experience recurrences within 1 year after their ablation. Comprehensive translational research approaches integrated in clinical care pathways may improve our u...

Funding Acknowledgements Type of funding sources: None. Background Substrate ablation is superior to approaches that target clinical and tolerated VTs in patients with post-myocardial infarction (MI) VT. Different substrate modification strategies have been reported. However, proposed ablation targets are prone to operator interpretation (e.g. abn...

Graphical Abstract Schematic representation of the different global (left) and regional (right) electrical (upper panels) and mechanical (lower panels) components that contribute to electromechanical reciprocity both in the intact heart and at the cardiac tissue level. Stretch, passive mechanical stretching of the myocardium caused by changes in vo...

Background Age and comorbidities increase COVID-19 related in-hospital mortality risk, but the extent by which comorbidities mediate the impact of age remains unknown. Methods In this multicenter retrospective cohort study with data from 45 Dutch hospitals, 4806 proven COVID-19 patients hospitalized in Dutch hospitals (between February and July 20...

Background The sequence of myocardial activation and recovery can be studied in detail by invasive catheter recordings of cardiac electrograms (EGMs), or noninvasive inverse reconstructions thereof with electrocardiographic imaging (ECGI). Local activation and recovery times are obtained from a unipolar EGM by the moment of maximum downslope of the...

A comprehensive understanding of the interaction between triggers and electrical substrates leading to ventricular fibrillation (VF) and sudden cardiac arrest is lacking, and electrical substrates are difficult to detect and localize with current clinical tools. Here, we created repolarization time (RT) dispersion by regional drug infusion in perfu...

Background Sudden cardiac arrest is often due to ventricular fibrillation (VF). In 5–10% of cases, no cause can be identified despite extensive cardiac examination, hence the designation idiopathic VF. Early repolarization with down sloping ST segments has been previously identified in patients with idiopathic VF. Early repolarization may increase...

Background: Age and comorbidities increase COVID-19 related in-hospital mortality risk, but the extent by which comorbidities mediate the impact of age remains unknown. Methods: In this multicenter retrospective cohort study of 45 Dutch hospitals, 4,806 proven COVID-19 patients hospitalized in Dutch hospitals (between February and July 2020) from t...

Background The current standard of care for acute atrial fibrillation (AF) focuses primarily on immediate restoration of sinus rhythm by cardioversion, although AF often terminates spontaneously. Objective To identify determinants of early spontaneous conversion (SCV) in patients presenting at the emergency department (ED) because of AF. Methods...

Background: Symptomatic idiopathic ventricular arrhythmias (VA), including premature beats (VPB) and nonsustained ventricular tachycardia (VT) are commonly encountered arrhythmias. Although these VA are usually benign, their treatment can be a challenge to primary and secondary health care providers. Mainstay treatment is comprised of antiarrhythm...

Materials and methods: Fifty consecutive patients with previous cardiac arrhythmias, scheduled for high-resolution 3D LGE MRI, were prospectively enrolled between October 2017 and February 2020. Free-breathing 3D dark-blood LGE MRI with high isotropic resolution (1.6 × 1.6 × 1.6 mm) was performed using a conventional fixed TI (n = 25) or a dynamic...

A feared complication of acute myocardial infarction is the formation of a cardiac pseudoaneurysm. We report a case of a gargantuan, arrhythmogenic left-ventricular pseudoaneurysm with contradictory morphological characteristics. The integrative use of high-resolution 3-dimensional magnetic resonance imaging and computed tomography proved essential...

Background Sudden cardiac arrest (SCA) is most often due to ventricular fibrillation (VF). When no cause is found during diagnostic follow-up, fibrillation is classified as idiopathic (iVF). We hypothesize that a critical functional substrate-trigger interaction underlies iVF. Purpose To study electrophysiological triggers and substrate for iVF in...

Background: In the 123-study we prospectively assessed, in a randomized fashion, the minimal cryoballoon application time necessary to achieve pulmonary vein (PV) isolation (PVI) in patients with paroxysmal atrial fibrillation (AF) with the aim to reduce complications by shortening the application duration. The first results of this study demonstr...

The autonomic nervous system with its sympathetic and parasympathetic limbs and its neurohormones (nor)epinephrine and acetylcholine is a well-recognized modulator of cardiac electrophysiology. Inherited or congenital inhomogeneities of cardiac autonomic innervation, sympathovagal imbalance, autonomic nervous system hyperactivity, autonomic conflic...

Aims In the past few years, promising results were described in targeting the arrhythmogenic substrate of the epicardial right ventricular outflow tract (RVOT) region in patients with Brugada syndrome (BrS). In this report, we describe our experience with endo- and epicardial substrate mapping and ablation in a series of highly symptomatic BrS pati...

Background The use of temporary mechanical circulatory support (tMCS) during arrhythmias is increasing, although available evidence for this indication is limited, with significant gaps of knowledge regarding appropriate timing, management and configuration. This systematic review sought to analyze the use of tMCS in patients with life-threatening...

In this study, we performed homozygosity mapping to further characterize a Dutch-German pedigree segregating an SCN5A mutation that we previously described as the "Worm population". We show how heterogeneity of cardiac phenotypes in this pedigree can be explained by the sum of effects from a number of genomic regions, independent from the effects o...

Background Left-stellate ganglion stimulation (LSGS) can modify regional dispersion of ventricular refractoriness, promote triggered activity, and reduce the threshold for ventricular fibrillation (VF). Sympathetic hyperactivity precipitates torsades de pointes (TdP) and VF in susceptible patients with long-QT syndrome type 1 (LQT1). We investigate...

Background The second generation cryoballoon significantly improves outcome of pulmonary vein (PV) isolation (PVI) but may cause more complications than the first generation. Currently no consensus regarding optimal cryoballoon application time exists. The 123‐study aimed to assess the minimal cryoballoon application duration necessary to achieve P...

Background: Heritable cardiac sodium-channel dysfunction is associated with various arrhythmia syndromes, some predisposing to ventricular fibrillation. Phenotypic diversity among carriers of identical-by-descent mutations is often remarkable, suggesting influences of genetic modifiers. Objective: We identified a unique SCN5A-mutation founder po...

Background: In patients with aortic stenosis, subtle alterations in myocardial mechanics can be detected by speckle-tracking echocardiography before reduction of left ventricular ejection fraction (LVEF). Methods: In this prospective study, 162 patients with aortic stenosis with an average aortic valve area of 0.7 ± 0.2 cm(2) and a mean LVEF of...

In the western world, ∼20% of all deaths among people between 20 and 80 years old are sudden and unexpected and this has not changed much during recent decades, whereas mortality from cardiac disease has diminished substantially.1 Sudden death is most often caused by the onset of ventricular fibrillation (VF) or ventricular tachycardia, interfering...

Gal et al. [1] reported the results of the 50 patients treated for atrial fibrillation (AF) using the endoscopic laser balloon ablation system (EAS) in the Netherlands, who were recruited for the study between December 2011 and December 2013. The second-generation EAS uses a compliant balloon to improve balloon/tissue contact, which improves the en...

Gastrointestinal functional and motility disorders, like irritable bowel syndrome (IBS), have a high prevalence in the Western population and cause significant morbidity and loss of quality of life leading to considerable costs for health care. A decade ago, it has been demonstrated that interstitial cells of Cajal and intestinal smooth muscle cell...

Aim: Prolonged and dispersed left-ventricular (LV) contraction is present in patients with long-QT syndrome (LQTS). Electrical and mechanical abnormalities appear most pronounced in symptomatic individuals. We focus on the 'electromechanical window' (EMW; duration of LV-mechanical systole minus QT interval) in patients with genotyped LQTS. Profoun...

Arrhythmogenic right-ventricular cardiomyopathy (ARVC) can lead to RV dilatation. We hypothesized that electrocardiographic characteristics including QRS amplitudes in the extremity- and precordial leads, the S amplitude in lead V1 , and extent of T-wave negativity over the precordial leads are related to RV dilatation in this condition. In 42 ARVC...

Purpose: Methods: Results: Conclusions: Table 1 Patient Characteristics

Since the first linkage of the long-QT syndrome to the Harvey ras-1 gene in 1991 ample research has been performed to decipher the molecular-biophysical basis of congenital repolarization defects and the electrophysiological mechanisms of torsades-de-pointes arrhythmias in this condition. Mechanistic knowledge is mostly derived from cellular experi...

Transcatheter valve-in-valve implantation is a promising treatment option for degenerated bioprosthetic heart valves in high-risk patients.1 Successful percutaneous techniques have been described for pulmonary and aortic valves.2,3 We present the first successful percutaneous tricuspid valve-in-valve implantation. The patient was a fragile 74-year...

A case of a 48-year-old male is described with recurrent paroxysmal ventricular tachycardia originating near the region of the posterior fascicle, as an initial presentation of a primary norepinephrine producing pheochromocytoma, including reversible catecholamine-induced cardiomyopathy. The ventricular arrhythmia was terminated by procainamide and...