Liu et al.case-control
Rare KCND3 C-terminal variants show ~8-fold enrichment in Brugada syndrome via Ito gain of function.
May support KCND3 sequencing in Brugada syndrome evaluation; extends GWAS evidence for Ito but leaves clinical adoption open.
Arthur A.M. Wilde
Electrophysiology
University of Amsterdam
About
Arthur A.M. Wilde works in Electrophysiology affiliated with University of Amsterdam.
Arthur A.M. Wilde is Full Professor of Cardiology at the Heart Centre, Department of Clinical and Experimental Cardiology, Amsterdam UMC, University of Amsterdam. He is a clinical cardiologist and translational researcher focused on electrophysiology, cardiogenetics and inherited arrhythmias. He serves as coordinator of the European Reference Network GUARD-Heart and has a visiting professorship at Columbia University Irving Medical Center.
Research focus
Inherited cardiac arrhythmiasBrugada syndromeLong QT syndromeCardiogeneticsSudden cardiac death
Impact
1,201 pubs · 74,853 cites · h-index 149
Training
MD
University of Amsterdam
1983
PhD
University of Amsterdam
1988
Affiliations
Work Experience
Full Professor of Cardiology
Amsterdam UMC, University of Amsterdam · 2003 - Present
Co-Chair, Academic Cardiovascular Science Institute
Amsterdam UMC · 2019 - Present
Visiting Professor
Columbia University Irving Medical Center · 2013 - Present
Head, Department of Clinical and Experimental Cardiology
University of Amsterdam · 2003 - 2019
Head, Laboratory of Experimental Cardiology
AMC Amsterdam · 1999 - Present
Publications
Sami Viskin
Electrophysiology · Heart Rhythm Society
the test is very easy to do and should be performed in addition to (not instead of) all the tests that we perform to confirm or exclude a diagnosis of LQTS.
The Response of the QT Interval to the Brief Tachycardia Provoked by Standing
Journal of the American College of CardiologyViskin et al.
Indian Heart JournalChockalingam et al.review
Inherited cardiovascular disorders are the primary cause of sudden cardiac death in individuals under 40 years, whereas coronary artery disease predominates over age 40.
This review highlights the essential role of molecular autopsy in unexplained sudden cardiac death and cascade clinical and genetic screening of relatives in specialized multidisciplinary clinics to prevent adverse outcomes.
Johannes Sperzel
Electrophysiology · Max Planck Society
The data available for both leadless pacemaker systems are promising and show that safety is similar to transvenous pacemakers. However, data across a larger patient population with longer term follow-up will be key in order for leadless pacemakers to fully replace transvenous pacemakers, which have been around for over 50 years and have vast amounts of data to support their safety profile.
Chronic Performance of a Leadless Cardiac Pacemaker
Journal of the American College of CardiologyKnops et al.
CirculationWilde et al.review
The review highlights that selectively targeting the late sodium current (late INa) offers a promising therapeutic strategy for arrhythmias like LQT3 without impairing normal electrical conduction driven by peak INa, and identifies emerging modulators including SGLT2 inhibitors and nicotinamide riboside.
Understanding the structural and regulatory mechanisms differentiating late INa from peak INa is essential for developing safe and selective therapies for Nav1.5-associated cardiac arrhythmias.
Gerhard Hindricks
Electrophysiology · Humboldt-Universität zu Berlin
Our findings do not mean that ICD use in myocardial infarction patients with severely reduced pump function is generally no longer beneficial. Rather, they show that the current strategy of prophylactically implanting a defibrillator in these patients cannot be a one-size-fits-all solution. Each case must be considered individually.
Mortality reduction with implanted defibrillator for primary prevention of sudden death after Myocardial Infarction: temporal trends in the PROFID study
European Heart JournalShamloo et al.
JACCPostema et al.Original Research
Patients developing ajmaline-provoked type 1 Brugada ECGs exhibit ~17 ms longer right precordial QRS durations.
Depolarization abnormalities predominate during ajmaline-induced type 1 BrS ECG; leaves open their role in risk stratification.
Supriya Shore
Heart Failure / Cardiomyopathy · University of Michigan
This study includes one of largest cohorts of ARVC relatives and provides more data on 'who, how, and when' for ARVC screening. It suggests that relatives aged 20-30 years, those with symptoms, or those with borderline ARVC need more aggressive and frequent (possibly annual) screening. Similarly, screening for relatives with possible ARVC can be spaced out to possibly every 5 years.
Individualized Family Screening for Arrhythmogenic Right Ventricular Cardiomyopathy
Journal of the American College of CardiologyMuller et al.
EHJPostema et al.editorial
The marketing authorization of mexiletine as an orphan drug for non-dystrophic myotonia has led to exorbitant price increases, threatening its availability for patients with ventricular arrhythmias.
The 2018 European marketing authorization of mexiletine as an orphan drug for non-dystrophic myotonias caused an extreme price increase to roughly €65,000 per patient per year and withdrawal of generic versions, critically endangering access for cardiology patients who rely on it to prevent life-threatening ventricular arrhythmias.
JACCViskin et al.Cross-Sectional Study
Quinidine is readily available in only 14% of countries, with unavailability linked to fatal arrhythmias.
Descriptive study reveals widespread global unavailability of life-saving quinidine in Brugada syndrome, highlighting critical international drug supply barriers.
EP EuropacePostema et al.Guideline
Essential antiarrhythmic drug accessibility worldwide: a multi-survey study and comprehensive evaluation of access and supply challenges
AIMS: Antiarrhythmic drugs (AADs) are essential for preventing symptoms, morbidity, and mortality associated with cardiac arrhythmias. However, concerns are increasing about their accessibility. We aimed to assess insights into current access to AADs. METHODS AND RESULTS: Online surveys were distributed to members of the European Heart Rhythm Association, the Association for European Paediatric and Congenital Cardiology, the European Reference Network GUARD-Heart, and national heart rhythm societies. Responses were obtained from 558 respondents across 80 countries. Among countries included in the main analysis (≥5 respondents; 16 countries within and 7 outside the European Union, n = 298 respondents), only 16-59% of surveyed AADs were considered readily accessible. Accessibility varied substantially by drug: oral flecainide and propranolol were available in 88% of countries, whereas mexiletine solution was available in only 10%. Marked differences in accessibility were reported both between and within countries, with availability also changing over time. Higher-income countries generally reported better access and a greater number of market authorization holders than lower-income countries. National societies' perception was that limited access negatively affected patients' quality of life (72%) and life expectancy (35%), while 73% expected these challenges to persist or worsen in the coming years. CONCLUSION: Physicians worldwide frequently encounter problems accessing guideline-recommended antiarrhythmic drugs for their patients, which putatively impacts their patients' quality of life and life expectancy. This highlights systemic vulnerabilities that extend across national and institutional boundaries, urging close collaboration among clinicians, policymakers, manufacturers, and institutions to guarantee timely and equitable treatment for all patients.
The Lancet Regional Health - EuropeDollfus et al.Cross-Sectional Study
Crisis readiness for rare disease populations: learnings and recommendations by the European Reference Networks
Cross-sectional survey assesses crisis experiences of European Reference Networks for rare diseases, suggesting essential preparedness improvements.
JACCChockalingam et al.cohort
For breakthrough cardiac events in symptomatic patients on metoprolol compared to propranolol/nadolol.
Metoprolol was associated with higher breakthrough events in symptomatic LQT1/2; challenges beta-blocker equivalence but remains hypothesis-generating pending randomized data.
EHJRivaud et al.cohort
38 mutation-positive patients experienced SCD or life-threatening ventricular arrhythmia, with a high proportion of those >40 years having hypertension/hypertrophy; in mice, hypertrophy induction caused SCD, which was prevented by inhibiting the late sodium current.
Co-existing cardiac hypertrophy modulates arrhythmia risk and reduces pacemaker efficacy in inherited sodium channelopathy, highlighting the need for rigorous treatment of hypertension and hypertrophy in these patients.
JACCMedeiros‐Domingo et al.cohort
Putative disease-causing RYR2 mutations were identified in 73 of 155 cases, with 11 cases hosting mutations outside the three canonical domains.
Comprehensive RYR2 genetic testing reveals mutations in nearly half of patients with exertional syncope and normal QTc, supporting a tiered genetic testing strategy.
EP EuropaceSarquella‐Brugada et al.Guideline
Diagnosis and management of very rare primary arrhythmia syndromes in children and adults: a Clinical Consensus Statement of the European Heart Rhythm Association of the ESC and the Association of Cardiovascular Nursing & Allied Professions of the ESC, endorsed by the Association for European Paediatric and Congenital Cardiology
Offers pragmatic guidance for rare inherited arrhythmia syndromes; extends guidelines to previously unaddressed entities.
JACCLaurent et al.case_series
All affected subjects carried the c.665G>A transition in the SCN5A gene, leading to a gain of function of the sodium channel that was responsive to hydroquinidine.
Identifies a novel familial arrhythmia syndrome (MEPPC) caused by an SCN5A gain-of-function mutation that is responsive to hydroquinidine.
JACCBaker et al.preclinical
Electrophysiologic mapping shows VT activation delay follows a zigzag route over surviving myocyte bundles.
Surviving myocardial tissue in infarcted human hearts forms complex branching bundles that cause activation delay and facilitate ventricular tachycardia via a zigzag route of activation.
Journal of Cardiovascular Translational ResearchDrie et al.Original Research
Tracing the pathogenic PLN p.(Arg14del) variant across the globe; more than just a local curiosity
Study traces the PLN p.(Arg14del) variant distribution in multiple countries, indicating its global significance.
JACCRijsingen et al.cohort
Malignant ventricular arrhythmias affect ~18% of LMNA carriers, occurring exclusively with two or more risk factors.
In LMNA mutation carriers, the presence of at least two risk factors (NSVT, LVEF <45%, male sex, non-missense mutations) identifies patients at high risk for malignant ventricular arrhythmias who may benefit from an ICD.