Publications by authors named "Tomas Robyns"

Background: Female athletes exhibit lower rates of atrial fibrillation (AF) and sudden cardiac death compared with male athletes, but the mechanisms behind this sex disparity in arrhythmia risk remain unclear.

Methods: This study analyzed findings from Holter monitors, echocardiograms, and cardiac magnetic resonance imaging in a cohort of 397 endurance athletes enriched with prevalent AF. Athletes with cardiomyopathies, channelopathies, pre-excitation, and/or myocardial infarction were excluded.

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Background And Aims: Cardiomyopathies are a heterogeneous group of genetic disorders requiring specialised, multidisciplinary management to optimize patient outcomes. A critical aspect of care is the transition of paediatric patients to adult services, which varies significantly across healthcare systems.This study assessed current practices in care transition and multidisciplinary management of inherited and rare cardiomyopathies across specialised European centres within the European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart (ERN GUARD-Heart) network.

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Aims: While studies have shown worse clinical outcomes in patients with an implantable cardioverter-defibrillator (ICD) who underwent an upgrade to cardiac resynchronization therapy (CRT) when compared to de novo CRT implantations, little is known about the clinical trajectory of these patients prior to upgrading to CRT.

Methods And Results: This single-centre retrospective study included patients with ischaemic or non-ischaemic cardiomyopathy with a QRS duration <130 ms who underwent ICD implantation between 2010 and 2022. By reviewing the evolution of QRS duration, QRS morphology, and right ventricular pacing (RVP) percentages, it was assessed whether the patients developed an indication for CRT according to contemporary European Society of Cardiology guidelines during the follow-up.

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Background: A greater prevalence of arrhythmias has been described in endurance athletes, but it remains unclear whether this risk persists after detraining. We aimed to evaluate the prevalence of arrhythmias and their relationship with cardiac remodeling in lifelong and retired master endurance athletes compared to non-athletic controls.

Methods: We performed a cross-sectional analysis of observational studies that used echocardiography and cardiac magnetic resonance to detail cardiac structure and function, and Holter monitors to identify atrial and ventricular arrhythmias in 185 endurance athletes and 81 non-athletic controls aged ≥40 years.

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Aims: Non-ischaemic ring-like late gadolinium enhancement (LGE) in the left ventricle (LV) detected by cardiac magnetic resonance (CMR) is an emerging biomarker associated with adverse outcomes. Data regarding ring-like LGE are limited to small patient cohorts. We aimed to assess the prevalence of ring-like LGE, its association with morpho-functional phenotypes, aetiologic background, and prognostic implications.

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Ventricular depolarization refers to the electrical activation and subsequent contraction of the ventricles, visible as the QRS complex on a 12-lead electrocardiogram (ECG). A well-organized and efficient depolarization is critical for cardiac function. Abnormalities in ventricular depolarization may indicate various pathologies and can be present in all leads if the condition is general, or in a subgroup of anatomically contiguous leads if the condition is limited to the corresponding anatomic location of the heart.

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Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare, potentially life-threatening genetic heart disease. Nonselective beta-blockers (BBs) are highly effective in reducing CPVT-triggered arrhythmic events. However, some patients suffer from unacceptable BB side effects and might require strategies without a BB.

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Purpose: Reflex syncope is a burdensome disease with considerable repercussions on the quality of life. Tilt training is a therapeutic option, but evidence on this topic is scarce and outdated. Hyperventilation is oftentimes associated with reflex syncope.

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Background: Left ventricular (LV) hypertrophy is a common clinical finding. Differential diagnosis includes Fabry disease, a rare and progressive, but treatable storage disease caused by deficiency of α-galactosidase A. However, diagnosis of Fabry is often hampered by its clinical heterogeneity, LV hypertrophy phenocopies and unawareness of the clinician.

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Mitral valve prolapse (MVP) and mitral annular disjunction (MAD) are common valvular abnormalities that have been associated with ventricular arrhythmias (VA). Cardiac magnetic resonance imaging (CMR) has a key role in risk stratification of VA, including assessment of late gadolinium enhancement (LGE). Single-center retrospective analysis of patients with MVP or MAD who had >1 CMR and >1 24 h Holter registration available.

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Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) may cause sudden cardiac death (SCD) despite medical therapy. Therefore, implantable cardioverter-defibrillators (ICDs) are commonly advised. However, there is limited data on the outcomes of ICD use in children.

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Background: Exercise-induced cardiac remodeling can be profound, resulting in clinical overlap with dilated cardiomyopathy, yet the significance of reduced ejection fraction (EF) in athletes is unclear. The aim is to assess the prevalence, clinical consequences, and genetic predisposition of reduced EF in athletes.

Methods: Young endurance athletes were recruited from elite training programs and underwent comprehensive cardiac phenotyping and genetic testing.

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Background: In severely affected patients with catecholaminergic polymorphic ventricular tachycardia, beta-blockers are often insufficiently protective. The purpose of this study was to evaluate whether flecainide is associated with a lower incidence of arrhythmic events (AEs) when added to beta-blockers in a large cohort of patients with catecholaminergic polymorphic ventricular tachycardia.

Methods: From 2 international registries, this multicenter case cross-over study included patients with a clinical or genetic diagnosis of catecholaminergic polymorphic ventricular tachycardia in whom flecainide was added to beta-blocker therapy.

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Background: Recently, an expert consensus statement proposed indications where implantation of a primary prevention implantable cardioverter-defibrillator (ICD) may be reasonable in patients with mitral valve prolapse (MVP). The objective was to evaluate the proposed risk stratification by the expert consensus statement.

Methods: Consecutive patients with MVP without alternative arrhythmic substrates with cardiac magnetic resonance imaging (CMR) were included in a single-center retrospective registry.

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Article Synopsis
  • Calmodulinopathy is caused by mutations in CALM genes and leads to serious arrhythmias, particularly in young people; the ICalmR aims to connect clinical symptoms with molecular causes.
  • The ICalmR has gathered data from 140 patients, showing a notable presence of CALM-LQTS and CALM-CPVT, and has observed a decrease in the frequency of serious cardiac events compared to past data.
  • The condition presents a wide range of symptoms, from severe arrhythmias to no symptoms at all; while therapy options are limited and based on current practices, management often involves medication and devices like defibrillators.
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Background: In sudden cardiac arrest survivors without an immediately identifiable cause, additional extensive yet individualised testing is required.

Methods: We describe 3 survivors of sudden cardiac arrest in whom exercise stress testing was not performed during the initial hospital admission.

Results: All 3 patients were incorrectly diagnosed with long QT syndrome based on temporary sudden cardiac arrest-related heart rate-corrected QT interval prolongation, and exercise stress testing was not performed during the initial work-up.

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Aims: Diagnosis of Long QT syndrome (LQTS) is based on prolongation of the QT interval corrected for heart rate (QTc) on surface ECG and genotyping. However, up to 25% of genotype positive patients have a normal QTc interval. We recently showed that individualized QT interval (QTi) derived from 24 h holter data and defined as the QT value at the intersection of an RR interval of 1,000 ms with the linear regression line fitted through QT-RR data points of each individual patient was superior over QTc to predict mutation status in LQTS families.

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Aims: Papillary muscle (PM) abnormalities are considered part of the phenotypic spectrum of hypertrophic cardiomyopathy (HCM). The aim of this study was to evaluate the presence and frequency of PM displacement in different HCM phenotypes.

Methods And Results: We retrospectively analysed cardiovascular magnetic resonance (CMR) findings in 156 patients (25% females, median age 57 years).

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Article Synopsis
  • The study identifies and compares the clinical and MRI features of truncating variants in Titin (TTNtv) and Filamin C (FLNCtv), revealing 3.6% and 12.3% occurrences respectively in the Belgian population.
  • FLNCtv carriers mostly present with arrhythmogenic cardiomyopathy (ACM), while TTNtv carriers exhibit either ACM or dilated cardiomyopathy (DCM) phenotypes, with both groups experiencing non-sustained Ventricular Tachycardia.
  • Cardiac MRI shows TTNtv patients have lower Left Ventricular ejection fraction but limited fibrosis, while FLNCtv patients display significant myocardial fibrosis with a ring-like pattern.
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Background: Congenital long QT syndrome (LQTS) is a rare heart disease caused by various underlying mutations. Most general cardiologists do not routinely see patients with congenital LQTS and may not always recognize the accompanying ECG features. In addition, a proportion of disease carriers do not display obvious abnormalities on their ECG.

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Article Synopsis
  • Brugada syndrome (BrS) is a serious heart condition linked to sudden death in young adults, with few known genetic factors beyond the SCN5A gene.
  • A large study involving 2,820 BrS cases and 10,001 controls revealed 21 genetic signals across 12 locations, suggesting a strong genetic component to the disorder.
  • Key findings highlight the importance of transcription regulation in BrS development and introduce microtubule-related mechanisms that affect the expression of a key cardiac protein, shedding light on the disorder's genetic and molecular basis.
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Background: Symptomatic children with catecholaminergic polymorphic ventricular tachycardia (CPVT) are at risk for recurrent arrhythmic events. β-Blockers decrease this risk, but studies comparing individual β-blockers in sizeable cohorts are lacking. We aimed to assess the association between risk for arrhythmic events and type of β-blocker in a large cohort of symptomatic children with CPVT.

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Article Synopsis
  • Cryoballoon ablation (CRYO) is an effective alternative to radiofrequency catheter ablation (RFCA) for treating atrial fibrillation (AF), with a focus on improving quality of life (QoL) for patients.
  • A review of clinical studies showed consistent significant improvement in QoL for patients undergoing CRYO after 12 months, regardless of the QoL measurement tools used.
  • Limitations in direct comparisons between CRYO and RFCA, along with the need for more long-term data, highlight the importance of prioritizing QoL assessment in future research.
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