Research Topics
Genomes and Genes | Arthur A M WildeSummaryAffiliation: Academic Medical Center Country: The Netherlands Publications
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Publications
Founder mutations in the Netherlands: familial idiopathic ventricular fibrillation and DPP6P G Postema
Department of Cardiology and Heart Failure Research Center, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Neth Heart J 19:290-6. 2011..In addition, when closely coupled extrasystoles initiate ventricular fibrillation in the absence of other identifiable causes, a link to the DPP6 gene should be suspected...
Vasovagal syncope or ventricular fibrillation. Your diagnosis better be accurateArthur A M Wilde
Clin Auton Res 17:203-5. 2007
Ten years of genes in inherited arrhythmia syndromes: an example of what we have learned from patients, electrocardiograms, and computersArthur A M Wilde
Department of Clinical and Experimental Cardiology, Academic Medical Center, 1105 AZ Amsterdam, The Netherlands
J Electrocardiol 38:145-9. 2005..As such these studies serve as a good example of what can be reached in a multidisciplinary approach with clinical and basic scientists...
Proposed diagnostic criteria for the Brugada syndrome: consensus reportArthur A M Wilde
Experimental and Molecular Cardiology Group, Academic Medical Center, Amsterdam, and the Interuniversity Cardiology Institute, The Netherlands
Circulation 106:2514-9. 2002
Channelopathies in children and adultsArthur A M Wilde
Heart Failure Research Centre, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands
Pacing Clin Electrophysiol 31:S41-5. 2008..At the same time and of equal importance, unaffected family members (noncarriers) can be reassured...
Is there a genetic basis for malignant ventricular arrhythmias?Arthur A M Wilde
Department of Clinical and Experimental Cardiology, Academic Medical Center, Amsterdam, The Netherlands
Heart Rhythm 2:1145-7. 2005
Genotype-specific onset of arrhythmias in congenital long-QT syndrome: possible therapy implicationsHanno L Tan
Department of Cardiology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Circulation 114:2096-103. 2006..This disparity may point to different arrhythmia mechanisms and may affect therapy strategies. We studied whether the proportion of pause-dependent TdP onset varies among LQTS genotypes...
Is there a role for implantable cardioverter defibrillators in long QT syndrome?Arthur A M Welde
Academic Medical Center, and Interuniversity Cardiology Institute of The Netherlands, Amsterdam
J Cardiovasc Electrophysiol 13:S110-3. 2002..Some genetic subtypes, such as LQTS3, may not respond as well (or even adversely) to antiadrenergic therapy and, thus, benefit more from implantable cardioverter defibrillator therapy...
Phenotypical manifestations of mutations in the genes encoding subunits of the cardiac sodium channelArthur A M Wilde
Department of Clinical and Experimental Cardiology, Academic Medical Center, University Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Circ Res 108:884-97. 2011....
Genetics of cardiac arrhythmiasArthur A M Wilde
Experimental and Molecular Cardiology Group, Academic Medical Center, Amsterdam, The Netherlands
Heart 91:1352-8. 2005
Left cardiac sympathetic denervation for catecholaminergic polymorphic ventricular tachycardiaArthur A M Wilde
University of Amsterdam, Amsterdam
N Engl J Med 358:2024-9. 2008....
[Presymptomatic screening after a sudden cardiac death in the family]A A Wilde
Academisch Medisch Centrum, Amsterdam
Ned Tijdschr Geneeskd 143:1643-8. 1999..g. in asymptomatic persons with the Brugada syndrome, a form of right bundle branch block). Presymptomatic investigation must be performed multidisciplinary...
Inherited arrhythmia syndromesArthur Am Wilde
Experimental and Molecular Cardiology Group, Academic Medical Center, Amsterdam, The Netherlands
Circ J 71:A12-9. 2007..It is likely that this development will further increase the knowledge of the (patho-) physiology of these disease entities, but also of more common arrhythmia syndromes...
A mutation in the human cardiac sodium channel (E161K) contributes to sick sinus syndrome, conduction disease and Brugada syndrome in two familiesJeroen P P Smits
Experimental and Molecular Cardiology Group, Academic Medical Center, University of Amsterdam, The Netherlands
J Mol Cell Cardiol 38:969-81. 2005....
Familial evaluation in catecholaminergic polymorphic ventricular tachycardia: disease penetrance and expression in cardiac ryanodine receptor mutation-carrying relativesChristian van der Werf
Department of Cardiology, Heart Failure Research Center, Academic Medical Center, Amsterdam, The Netherlands
Circ Arrhythm Electrophysiol 5:748-56. 2012..Previous studies of CPVT patients mainly involved probands, so current insight into disease penetrance, expression, genotype-phenotype correlations, and arrhythmic event rates in relatives carrying the Ryr2 mutation is limited...
Exercise-induced ECG changes in Brugada syndromeAhmad S Amin
Heart Failure Research Center and Department of Cardiology, Academic Medical Center, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands
Circ Arrhythm Electrophysiol 2:531-9. 2009..Yet, the effects of exercise on the BrS ECG phenotype have not been studied. We aimed to assess ECG responses to exercise in BrS and determine whether these responses are affected by the presence of an SCN5A mutation...
Expanding spectrum of human RYR2-related disease: new electrocardiographic, structural, and genetic featuresZahurul A Bhuiyan
Department of Clinical Genetics, Academic Medical Centre, University of Amsterdam, The Netherlands
Circulation 116:1569-76. 2007..Additional features include baseline bradycardia and, in some patients, right ventricular fatty displacement. The clinical spectrum is expanded by the 2 families described here...
A complex double deletion in LMNA underlies progressive cardiac conduction disease, atrial arrhythmias, and sudden deathRoos F Marsman
Heart Failure Research Center, Department of Experimental Cardiology, Amsterdam, The Netherlands
Circ Cardiovasc Genet 4:280-7. 2011..Cardiac conduction disease is a clinically and genetically heterogeneous disorder characterized by defects in electrical impulse generation and conduction and is associated with sudden cardiac death...
Role of sequence variations in the human ether-a-go-go-related gene (HERG, KCNH2) in the Brugada syndromeArie O Verkerk
Department of Experimental Cardiology, Academic Medical Center, University of Amsterdam, Meibergdreef 15, 1105 AZ Amsterdam, The Netherlands
Cardiovasc Res 68:441-53. 2005..Other causally related genes still await identification. We evaluated the role of HERG (KCNH2), which encodes the alpha-subunit of the rapid delayed rectifier K(+) channel (I(Kr)), in BrS...
Compound heterozygosity for mutations (W156X and R225W) in SCN5A associated with severe cardiac conduction disturbances and degenerative changes in the conduction systemConnie R Bezzina
Experimental and Molecular Cardiology Group, Academic Medical Center, Amsterdam, The Netherlands
Circ Res 92:159-68. 2003..The morphological changes within the heart of the deceased sibling may have occurred secondary to the Na+ channel abnormality and contributed to the severity of the disorder in this individual...
Arrhythmogenic right ventricular dysplasia/cardiomyopathy: pathogenic desmosome mutations in index-patients predict outcome of family screening: Dutch arrhythmogenic right ventricular dysplasia/cardiomyopathy genotype-phenotype follow-up studyMoniek G P J Cox
Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands
Circulation 123:2690-700. 2011..Previous genotype-phenotype relation studies involved mainly overt ARVD/C index patients, so follow-up data on relatives are scarce...
Characterization of a novel SCN5A mutation associated with Brugada syndrome reveals involvement of DIIIS4-S5 linker in slow inactivationSimona Casini
Heart Failure Research Center, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Cardiovasc Res 76:418-29. 2007..The G1319V mutation is located in the loop connecting transmembrane segments 4 and 5 in domain III (DIIIS4-S5), a region so far considered to be exclusively involved in fast inactivation...
A common polymorphism in KCNH2 (HERG) hastens cardiac repolarizationConnie R Bezzina
Experimental and Molecular Cardiology Group, Room M0 052, Academic Medical Center, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands
Cardiovasc Res 59:27-36. 2003....
Haplotype-sharing analysis implicates chromosome 7q36 harboring DPP6 in familial idiopathic ventricular fibrillationMarielle Alders
Department of Clinical Genetics, Academic Medical Center, Amsterdam, The Netherlands
Am J Hum Genet 84:468-76. 2009..Penetrance of IVF was high; 50% of risk-haplotype carriers experienced (aborted) sudden cardiac death before the age of 58 years. We propose DPP6 as a gene for IVF and increased DPP6 expression as the likely pathogenetic mechanism...
The role of the epinephrine test in the diagnosis and management of children suspected of having congenital long QT syndromeSally Ann B Clur
Department of Pediatric Cardiology, Emma Children s Hospital, Academic Medical Centre, Meibergdreef 9, Amsterdam 1105 AZ, The Netherlands
Pediatr Cardiol 31:462-8. 2010..Due to the discordance with genotyping, the epinephrine test cannot be used to diagnose genotype-positive LQTS but when used in combination with phenotype assessment and genetic screening, it could enable better management decisions...
Mechanism of right precordial ST-segment elevation in structural heart disease: excitation failure by current-to-load mismatchMark G Hoogendijk
Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Heart Rhythm 7:238-48. 2010..The Brugada sign has been associated with mutations in SCN5A and with right ventricular structural abnormalities. Their role in the Brugada sign and the associated ventricular arrhythmias is unknown...
Active cascade screening in primary inherited arrhythmia syndromes: does it lead to prophylactic treatment?Nynke Hofman
Department of Clinical Genetics, Academic Medical Center, Amsterdam, The Netherlands
J Am Coll Cardiol 55:2570-6. 2010..The purpose of this study was to investigate the follow-up and treatment of the mutation-carrying relatives of a proband with an inherited arrhythmia syndrome...
Fever-induced QTc prolongation and ventricular arrhythmias in individuals with type 2 congenital long QT syndromeAhmad S Amin
Heart Failure Research Center, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands
J Clin Invest 118:2552-61. 2008....
Quality of life and psychological distress in hypertrophic cardiomyopathy mutation carriers: a cross-sectional cohort studyImke Christiaans
Academic Medical Center, Department of Clinical Genetics, Amsterdam, The Netherlands
Am J Med Genet A 149:602-12. 2009..Because these variables can be addressed and adjusted during pre- and post-test counseling, genetic counseling should focus on these determinants...
Substitution of a conserved alanine in the domain IIIS4-S5 linker of the cardiac sodium channel causes long QT syndromeJeroen P P Smits
Experimental and Molecular Cardiology Group, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Cardiovasc Res 67:459-66. 2005..Substitution by a threonine shifts the voltage range of I(Na, window) activity to more positive potentials. Here the counter-acting effect of outward K+ current is reduced and may delay AP repolarization, explaining the LQT3 phenotype...
Overlap syndrome of cardiac sodium channel disease in mice carrying the equivalent mutation of human SCN5A-1795insDCarol Ann Remme
Department of Cardiology, Academic Medical Center, Room K2 110, PO Box 22700, 1100 DE Amsterdam, The Netherlands
Circulation 114:2584-94. 2006..We investigated the electrophysiological characteristics of a transgenic model of the murine equivalent mutation 1798insD...
Slow and discontinuous conduction conspire in Brugada syndrome: a right ventricular mapping and stimulation studyPieter G Postema
Cardiology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Circ Arrhythm Electrophysiol 1:379-86. 2008..The pathophysiological basis of the arrhythmias and type-1 BrS-ECG is unresolved. We studied the electrophysiological characteristics of the RV endocardium in BrS...
Type of SCN5A mutation determines clinical severity and degree of conduction slowing in loss-of-function sodium channelopathiesPaola G Meregalli
Department of Cardiology, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands
Heart Rhythm 6:341-8. 2009..The penetrance and expressivity of the disease are highly variable, and new tools for risk stratification are needed...
Quantitative trait loci for electrocardiographic parameters and arrhythmia in the mouseBrendon P Scicluna
Heart Failure Research Center, Department of Experimental Cardiology, University of Amsterdam, Amsterdam, The Netherlands
J Mol Cell Cardiol 50:380-9. 2011..The detection of QTL influencing ECG indices and arrhythmia is an essential step towards identifying genetic networks for sudden, arrhythmic, cardiac death...
New ECG criteria in arrhythmogenic right ventricular dysplasia/cardiomyopathyMoniek G P J Cox
Department of Cardiology, University Medical Center Utrecht, 3584 CX Utrecht, The Netherlands
Circ Arrhythm Electrophysiol 2:524-30. 2009..Current task force criteria (TFC) for diagnosis have limited sensitivity. The aim of this study was to assess the diagnostic value of additional criteria on activation delay and VT to improve identification of affected individuals...
An intronic mutation leading to incomplete skipping of exon-2 in KCNQ1 rescues hearing in Jervell and Lange-Nielsen syndromeZahurul A Bhuiyan
Department of Clinical Genetics, Academic Medical Centre, University of Amsterdam, Meibergdreef 15, 1105 AZ Amsterdam, The Netherlands
Prog Biophys Mol Biol 98:319-27. 2008..The novel KCNQ1 mutation found in this study is very likely a founder mutation in the southern province of Saudi Arabia emphasizing its screening in the LQT population in this region...
Common sodium channel promoter haplotype in asian subjects underlies variability in cardiac conductionConnie R Bezzina
Experimental and Molecular Cardiology Group, Department of Experimental Cardiology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Circulation 113:338-44. 2006..In this study, we tested the hypothesis that an SCN5A promoter polymorphism common in Asians modulates variability in cardiac conduction...
Arrhythmogenic right ventricular dysplasia/cardiomyopathy diagnostic task force criteria: impact of new task force criteriaMoniek G P J Cox
Department of Cardiology, University Medical Center Utrecht, 3584 CX Utrecht, The Netherlands
Circ Arrhythm Electrophysiol 3:126-33. 2010..A new international task force modified criteria to improve diagnostic yield. A comparison of diagnosis by 1994 TFC versus newly proposed criteria in 3 patient groups was conducted...
Exclusion of multiple candidate genes and large genomic rearrangements in SCN5A in a Dutch Brugada syndrome cohortTamara T Koopmann
Center for Heart Failure Research, Experimental and Molecular Cardiology Group, University of Amsterdam, Amsterdam, The Netherlands
Heart Rhythm 4:752-5. 2007..Genetic heterogeneity and/or undetected SCN5A mutations, such as exon duplications and deletions, could be involved in the remaining 80% mutation-negative patients...
Carriers of the hypertrophic cardiomyopathy MYBPC3 mutation are characterized by reduced myocardial efficiency in the absence of hypertrophy and microvascular dysfunctionStefan A J Timmer
Department of Cardiology, VU University Medical Center, De Boelelaan 1117, Amsterdam, The Netherlands
Eur J Heart Fail 13:1283-9. 2011..Therefore, the aim was to investigate myocardial perfusion and energetics in genotype-positive, phenotype-negative HCM subjects (carriers)...
Mortality risk of untreated myosin-binding protein C-related hypertrophic cardiomyopathy: insight into the natural historyEline A Nannenberg
Department of Clinical Genetics, Academic Medical Center, Amsterdam, The Netherlands
J Am Coll Cardiol 58:2406-14. 2011..The goal of this study was to assess the mortality of hypertrophic cardiomyopathy (HCM), partly in times when the disease was not elucidated and patients were untreated...
How do hypertrophic cardiomyopathy mutations affect myocardial function in carriers with normal wall thickness? Assessment with cardiovascular magnetic resonanceTjeerd Germans
Department of Cardiology, VU University Medical Center, Amsterdam, The Netherlands
J Cardiovasc Magn Reson 12:13. 2010..We aimed to confirm the presence of functional abnormalities using cardiovascular magnetic resonance (CMR), and to investigate if sensitive functional assessment could be employed to identify carriers...
Manifest disease, risk factors for sudden cardiac death, and cardiac events in a large nationwide cohort of predictively tested hypertrophic cardiomyopathy mutation carriers: determining the best cardiological screening strategyImke Christiaans
Department of Clinical Genetics, Academic Medical Centre, Amsterdam, The Netherlands
Eur Heart J 32:1161-70. 2011....
Value of history-taking in syncope patients: in whom to suspect long QT syndrome?Nancy Colman
Department of Cardiology B2, Academic Medical Centre, Meibergdreef 15, 1105 AZ, Amsterdam, The Netherlands
Europace 11:937-43. 2009..Long QT syndrome (LQTS), a potentially fatal disorder, has to be distinguished from non-fatal conditions. Our aim was to investigate whether history-taking can be used in identifying patients likely to have LQTS...
Incidence, causes, and outcomes of out-of-hospital cardiac arrest in children. A comprehensive, prospective, population-based study in the NetherlandsAbdennasser Bardai
Heart Failure Research Center, Department of Cardiology, Academic Medical Center, University of Amsterdam, Meibergdreef 9, Amsterdam, The Netherlands
J Am Coll Cardiol 57:1822-8. 2011....
Pathophysiological mechanisms of Brugada syndrome: depolarization disorder, repolarization disorder, or more?Paola G Meregalli
Department of Cardiology, Room M0 105, Academic Medical Center, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands
Cardiovasc Res 67:367-78. 2005....
Contribution of inherited heart disease to sudden cardiac death in childhoodNynke Hofman
Department of Clinical Genetics, Academic Medical Center, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands
Pediatrics 120:e967-73. 2007..We aimed to establish the cause of sudden cardiac death in the children of whom the family was referred to our cardiogenetics department and the diagnostic yield of these investigations...
Cardiac sodium channel overlap syndromes: different faces of SCN5A mutationsCarol Ann Remme
Department of Experimental Cardiology, Heart Failure Research Center, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Trends Cardiovasc Med 18:78-87. 2008..Here, we provide an overview of current knowledge on SCN5A mutations associated with sodium channel overlap syndromes and discuss a possible role for modifiers in determining disease expressivity in the individual patient...
A cardiac sodium channel mutation cosegregates with a rare connexin40 genotype in familial atrial standstillW Antoinette Groenewegen
Department of Medical Physiology, University Medical Center, Utrecht, The Netherlands
Circ Res 92:14-22. 2003..We propose that, although the functional effect of each genetic change is relatively benign, the combined effect of genetic changes eventually progresses to total AS...
Sudden unexplained death: heritability and diagnostic yield of cardiological and genetic examination in surviving relativesHanno L Tan
Department of Cardiology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Circulation 112:207-13. 2005..9 presymptomatic carriers per family. Simple procedures (examining many relatives) and routine tests (resting/exercise ECG) constitute excellent diagnostic strategies. Molecular genetics provide strong supportive information...
Obtaining insurance after DNA diagnostics: a survey among hypertrophic cardiomyopathy mutation carriersImke Christiaans
Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands
Eur J Hum Genet 18:251-3. 2010....
SCN5A mutations in Brugada syndrome are associated with increased cardiac dimensions and reduced contractilityFrans van Hoorn
Department of Radiology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
PLoS ONE 7:e42037. 2012..We aimed to determine whether clinical evidence may be obtained that Na(v)1.5 is involved in maintaining cardiac structural integrity...
Risk factors for malignant ventricular arrhythmias in lamin a/c mutation carriers a European cohort studyIngrid A W van Rijsingen
Department of Cardiology, Heart Failure Research Center, Academic Medical Center, Amsterdam, The Netherlands
J Am Coll Cardiol 59:493-500. 2012..The purpose of this study was to determine risk factors that predict malignant ventricular arrhythmias (MVA) in Lamin A/C (LMNA) mutation carriers...
Variants in the 3' untranslated region of the KCNQ1-encoded Kv7.1 potassium channel modify disease severity in patients with type 1 long QT syndrome in an allele-specific mannerAhmad S Amin
Heart Failure Research Center, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Eur Heart J 33:714-23. 2012..We aimed to study whether this can be explained by single nucleotide polymorphisms (SNPs) in KCNQ1's 3' untranslated region (3'UTR)...
Facilitatory and inhibitory effects of SCN5A mutations on atrial fibrillation in Brugada syndromeAhmad S Amin
Heart Failure Research Center, Department of Experimental Cardiology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Europace 13:968-75. 2011..Here, we examined the relation between SCN5A mutations, atrial conduction velocity, atrial structural changes, and atrial ectopic activity in BrS...
Cardiac ion channels in health and diseaseAhmad S Amin
Heart Failure Research Center, University of Amsterdam, Amsterdam, The Netherlands
Heart Rhythm 7:117-26. 2010..This review discusses ion channels that contribute to action potential formation in healthy hearts and their role in inherited and acquired diseases...
A gain-of-function TBX5 mutation is associated with atypical Holt-Oram syndrome and paroxysmal atrial fibrillationAlex V Postma
Heart Failure Research Center, L2 108 1, Academic Medical Center, Meibergdreef 15, 1105 AZ, Amsterdam, The Netherlands
Circ Res 102:1433-42. 2008....
Genome-wide association study identifies a susceptibility locus at 21q21 for ventricular fibrillation in acute myocardial infarctionConnie R Bezzina
Department of Experimental Cardiology, University of Amsterdam, Amsterdam, The Netherlands
Nat Genet 42:688-91. 2010..This locus has not previously been implicated in arrhythmia susceptibility...
Cardiac sodium channels and inherited electrophysiologic disorders: a pharmacogenetic overviewJeroen P P Smits
University of Amsterdam, Academic Medical Center, Department of Cardiology, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands
Expert Opin Pharmacother 9:537-49. 2008..An overview is given of the known risks of development of the previously mentioned complications of commonly prescribed drugs in patients affected with Na channel-related diseases and the underlying mechanisms...
Predictive genetic testing for cardiovascular diseases: impact on carrier childrenTineke M Meulenkamp
Medical Psychology, Academic Medical Center University of Amsterdam, and Pediatric Lipid Clinic, Emma s Children Hospital, Amsterdam, The Netherlands
Am J Med Genet A 146:3136-46. 2008..Nevertheless, dealing with the daily implications of their condition remains difficult in some situations, warranting continued availability of psychosocial support...
[Premature sudden death--consider serious familial heart rhythm disturbances]Pieter G Postema
Academisch Medisch Centrum, afd Cardiologie, Hartfaal Centrum, Amsterdam, The Netherlands
Ned Tijdschr Geneeskd 155:A3391. 2011..Timely recognition of persons affected allows appropriate treatment and may implicate an implantable cardioverter defibrillator...
The yield of risk stratification for sudden cardiac death in hypertrophic cardiomyopathy myosin-binding protein C gene mutation carriers: focus on predictive screeningImke Christiaans
Department of Clinical Genetics, Academic Medical Centre, Amsterdam, The Netherlands
Eur Heart J 31:842-8. 2010....
Sodium channelopathies: do we really understand what's going on?Pieter G Postema
Department of Cardiology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
J Cardiovasc Electrophysiol 22:590-3. 2011..One son who carried the ΔKPQ mutation but not the I1660V mutation did not show the expected Long-QT phenotype but, unexpectedly, showed a conduction disease/Brugada phenotype...
Genetically determined differences in sodium current characteristics modulate conduction disease severity in mice with cardiac sodium channelopathyCarol Ann Remme
Heart Failure Research Center, Department of Experimental Cardiology, Academic Medical Center, University of Amsterdam, Room K2 110, PO Box 22700, 1100 DE Amsterdam, The Netherlands
Circ Res 104:1283-92. 2009..In particular, the sodium channel subunit beta4 (SCN4B) may constitute a potential genetic modifier of conduction and cardiac sodium channel disease...
Right ventricular fibrosis and conduction delay in a patient with clinical signs of Brugada syndrome: a combined electrophysiological, genetic, histopathologic, and computational studyRuben Coronel
Experimental and Molecular Cardiology Group, Academic Medical Center, Amsterdam, The Netherlands
Circulation 112:2769-77. 2005..The mechanism of ECG changes and arrhythmogenesis in Brugada syndrome (BS) patients is unknown...
Electrocardiographic factors playing a role in ischemic ventricular fibrillation in ST elevation myocardial infarction are related to the culprit arteryMiguel E Lemmert
Department of Cardiology, University Hospital Maastricht, Maastricht, The Netherlands
Heart Rhythm 5:71-8. 2008..Sudden cardiac death caused by ischemic ventricular fibrillation (VF) associated with ST elevation myocardial infarction (STEMI) is one of the most frequent causes of death...
Do patients with long QT syndrome remain at risk for sudden cardiac death after 40 years of age?Pieter G Postema
Department of Clinical and Experimental Cardiology at the Academic Medical Centre, Amsterdam, The Netherlands
Nat Clin Pract Cardiovasc Med 5:602-3. 2008..65-9.92). Clearly, patients with LQTS remain at increased risk of lethal events after 40 years of age, indicating that continuous, age-independent awareness for QT prolongation is essential...
ECG quantification of myocardial scar does not differ between primary and secondary prevention ICD recipients with ischemic heart diseaseKarin Kraaier
Department of Cardiology, Medisch Spectrum Twente, Haaksbergerstraat 55, Enschede, The Netherlands
Pacing Clin Electrophysiol 33:192-7. 2010..In this study, we tested the hypothesis that QRS scores among ICD recipients for secondary prevention are higher than QRS scores in primary prevention patients...
Genotype-phenotype relationship in Brugada syndrome: electrocardiographic features differentiate SCN5A-related patients from non-SCN5A-related patientsJeroen P P Smits
Experimental and Molecular Cardiology Group, Academic Medical Center, University of Amsterdam, 1100 DE Amsterdam, The Netherlands
J Am Coll Cardiol 40:350-6. 2002....
Diagnostic performance of various QTc interval formulas in a large family with long QT syndrome type 3: Bazett's formula not so bad after allJan Brouwer
Department of Cardiology, Thorax Center, University Hospital Groningen, Groningen, The Netherlands
Ann Noninvasive Electrocardiol 8:269-74. 2003..In the diagnosis of this form of LQTS3, Bazett's formula was at least as good as other proposed corrections of the QT interval to heart rate...
Gender differences in the long QT syndrome: effects of beta-adrenoceptor blockadeChantal E Conrath
Department of Cardiology, University Medical Center, Utrecht, The Netherlands
Cardiovasc Res 53:770-6. 2002..CONCLUSIONS: These findings indicate that, in addition to underlying differences in repolarization between men and women, cardiac electrophysiological responses to beta-adrenoceptor blockade can be modulated by gender-related factors...
Delay in right ventricular activation contributes to Brugada syndromeRaymond Tukkie
Department of Cardiology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Circulation 109:1272-7. 2004..CONCLUSIONS: These results indicate that both proposed mechanisms of Brugada syndrome may be operative...
A family with Andersen-Tawil syndrome and dilated cardiomyopathyBas A Schoonderwoerd
Department of Cardiology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
Heart Rhythm 3:1346-50. 2006
Nodoventricular accessory pathways in PRKAG2-dependent familial preexcitation syndrome reveal a disorder in cardiac developmentHanno L Tan
Heart Failure Research Center, Department of Cardiology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Circ Arrhythm Electrophysiol 1:276-81. 2008..Yet, histopathologic proof is still lacking. We aimed to provide such proof...
Cardiac involvement in carriers of Duchenne and Becker muscular dystrophyE M Hoogerwaard
Department of Neurology, Academic Medical Centre, University of Amsterdam, The Netherlands
Neuromuscul Disord 9:347-51. 1999..If left ventricle dilatation or dilated cardiomyopathy is present a yearly follow up is needed, in order to start timely therapy...
Premature ventricular contractions during triggered imaging with ultrasound contrastP A van der Wouw
Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands
J Am Soc Echocardiogr 13:288-94. 2000..Further investigation into the relation of this phenomenon to both ultrasound energy and the contrast agent was carried out during a subsequent bolus-versus-infusion study...
[The heartache of muscular dystrophy]E M Hoogerwaard
afd Neurologie, Academisch Medisch Centrum Universiteit van Amsterdam
Ned Tijdschr Geneeskd 144:2181-4. 2000..Patients with dilated cardiomyopathy and with a positive family history for dilated cardiomyopathy, muscle weakness or high serum creatine kinase activity should be screened for a mutation in the dystrophin gene...
K(ATP) channel opening during ischemia: effects on myocardial noradrenaline release and ventricular arrhythmiasC A Remme
Experimental and Molecular Cardiology Group, Academic Medical Center, University of Amsterdam, The Netherlands
J Cardiovasc Pharmacol 38:406-16. 2001....
Symptoms and signs of syncope: a review of the link between physiology and clinical cluesWouter Wieling
Department of Internal Medicine, Academic Medical Centre University of Amsterdam, Amsterdam, The Netherlands
Brain 132:2630-42. 2009..Complete loss of consciousness occurs with the 'turning up' of the eyeballs. Profound cerebral hypoperfusion may be accompanied by myoclonic jerks...
Drugs and Brugada syndrome patients: review of the literature, recommendations, and an up-to-date website (www.brugadadrugs.org)Pieter G Postema
Department of Cardiology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Heart Rhythm 6:1335-41. 2009..Importantly, many drugs have been reported to induce the characteristic Brugada syndrome-linked ECG abnormalities and/or (fatal) ventricular tachyarrhythmias...
Genetic control of sodium channel functionHanno L Tan
Experimental and Molecular Cardiology Group, Department of Cardiology, Academic Medical Center, Room M0 052, P O Box 22700, 1100 DE, Amsterdam, The Netherlands
Cardiovasc Res 57:961-73. 2003....
Prevalence and characterization of ECG abnormalities after intracerebral hemorrhageMaurits D R van Bree
Department of Neurology, Academic Medical Centre Amsterdam, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands
Neurocrit Care 12:50-5. 2010..The purpose of this study is to investigate the prevalence and type of ECG abnormalities in a consecutive series of ICH patients, and their possible association with pre-defined neurological and radiological parameters...
Mechanisms of inherited cardiac conduction diseaseJeroen P P Smits
Department of Clinical and Experimental Cardiology, Experimental and Molecular Cardiology Group, Academic Medical Center, University of Amsterdam, Room M 0 107, Meibergdreef 9, PO Box 22700, 1100 DE Amsterdam, The Netherlands
Europace 7:122-37. 2005..In this review we aim to find evidence for the idea that functional and structural ICCD are fundamentally different diseases and, if so, whether this has diagnostic and clinical consequences...
Contribution of sodium channel mutations to bradycardia and sinus node dysfunction in LQT3 familiesMarieke W Veldkamp
Experimental and Molecular Cardiology Group, Department of Clinical and Experimental Cardiology, Academic Medical Center, University of Amsterdam, The Netherlands
Circ Res 92:976-83. 2003....
Desmoglein-2 and desmocollin-2 mutations in dutch arrhythmogenic right ventricular dysplasia/cardiomypathy patients: results from a multicenter studyZahurul A Bhuiyan
Department of Clinical Genetics, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Circ Cardiovasc Genet 2:418-27. 2009..We also aimed to distinguish relevant clinical and ECG parameters...
High diagnostic yield and accuracy of history, physical examination, and ECG in patients with transient loss of consciousness in FAST: the Fainting Assessment studyNynke van Dijk
Departments of Internal Medicine, Academic Medical Center, University of Amsterdam AMC UvA, Amsterdam, The Netherlands
J Cardiovasc Electrophysiol 19:48-55. 2008..Transient loss of consciousness (TLOC) is a common clinical problem...
Clinical and genetic analysis of long QT syndrome in children from six families in Saudi Arabia: are they different?Zahurul A Bhuiyan
Department of Clinical Genetics, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Pediatr Cardiol 30:490-501. 2009..3208 C > T in the KCNH2 gene, are presumably founder mutations in the Assir province of Saudi Arabia. Further, all LQTS causing mutations detected in this study are novel and have not been reported in other populations...
Familial sudden death is an important risk factor for primary ventricular fibrillation: a case-control study in acute myocardial infarction patientsLukas R C Dekker
Department of Cardiology, B2 116, Academic Medical Center, PO Box 22660, 1100 DD, Amsterdam, The Netherlands
Circulation 114:1140-5. 2006..Primary ventricular fibrillation (VF) accounts for the majority of deaths during the acute phase of myocardial infarction. Identification of patients at risk for primary VF remains very poor...
Recurrent intrauterine fetal loss due to near absence of HERG: clinical and functional characterization of a homozygous nonsense HERG Q1070X mutationZahurul A Bhuiyan
Department of Clinical Genetics, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Heart Rhythm 5:553-61. 2008..Resolving the molecular genetic nature of these rare cases provides significant insight into the role of the affected proteins in arrhythmogenesis and (extra-) cardiac development...
Con: Newborn screening to prevent sudden cardiac death?Irene M van Langen
Department of Clinical Genetics, Academic Medical Centre, Amsterdam, The Netherlands
Heart Rhythm 3:1356-9. 2006
Health-related quality of life of children with a positive carrier status for inherited cardiovascular diseasesEllen M A Smets
Department of Medical Psychology, Academic Medical Center University of Amsterdam, Amsterdam, The Netherlands
Am J Med Genet A 146:700-7. 2008..Yet, attention to possible problems in these children remains warranted...
Voltage-gated sodium channels: action players with many facesTamara T Koopmann
Experimental and Molecular Cardiology Group, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Ann Med 38:472-82. 2006..In this review article, mutations in these genes leading to various inherited disorders are discussed...
A novel early onset lethal form of catecholaminergic polymorphic ventricular tachycardia maps to chromosome 7p14-p22Zahurul A Bhuiyan
Department of Clinical Genetics, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands
J Cardiovasc Electrophysiol 18:1060-6. 2007..In this study, we aimed to elucidate clinical phenotypes of a new variant of CPVT (3) in an inbred Arab family and also delineate the chromosomal location of the gene causing CPVT (3)...
Adenosine induced ventricular arrhythmias in the emergency roomH L Tan
Department of Clinical and Experimental Cardiology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Pacing Clin Electrophysiol 24:450-5. 2001..These arrhythmias appeared to frequently originate from the inferior left ventricular septum, suggesting that this area may be particularly susceptible to the proarrhythmic effects of adenosine...
Diagnosis and long-term follow-up of the Brugada syndrome in patients with idiopathic ventricular fibrillationC A Remme
Department of Cardiology, Heart-Lung Centre Utrecht, The Netherlands
Eur Heart J 22:400-9. 2001..For all idiopathic ventricular fibrillation patients, the ventricular tachyarrhythmia recurrence rate was substantial during an average follow-up of more than 6 years...
Possible bradycardic mode of death and successful pacemaker treatment in a large family with features of long QT syndrome type 3 and Brugada syndromeM P van den Berg
Department of Cardiology, University Hospital Groningen, The Netherlands
J Cardiovasc Electrophysiol 12:630-6. 2001..The purpose of this study was to detail the clinical features and efficacy of pacemaker therapy in preventing sudden death in this family...
Alterations in potassium channel gene expression in atria of patients with persistent and paroxysmal atrial fibrillation: differential regulation of protein and mRNA levels for K+ channelsB J Brundel
Department of Cardiology, Thoraxcenter University Hospital Groningen, The Netherlands
J Am Coll Cardiol 37:926-32. 2001..Our purpose was to determine whether patients with persistent atrial fibrillation (AF) and patients with paroxysmal AF show alterations in potassium channel expression...
LocaLisa: new technique for real-time 3-dimensional localization of regular intracardiac electrodesF H Wittkampf
Heart Lung Institute, Department of Cardiology, University Hospital Utrecht, Utrecht, The Netherlands
Circulation 99:1312-7. 1999..Estimation of the 3-dimensional (3D) position of ablation electrodes from fluoroscopic images is inadequate if a systematic lesion pattern is required in the treatment of complex arrhythmogenic substrates...
Genomic organisation and chromosomal localisation of two members of the KCND ion channel family, KCND2 and KCND3A V Postma
Experimental and Molecular Cardiology Group, Cardiovascular Research Institute Amsterdam, The Netherlands
Hum Genet 106:614-9. 2000..2, respectively. This information should facilitate the systematic screening of KCND2 and KCND3 exons for mutations in (inherited) arrhythmia syndromes, such as LQTS and Brugada...
A single Na(+) channel mutation causing both long-QT and Brugada syndromesC Bezzina
Departments of Clinical Genetics, Academic Medical Center, Amsterdam, The Netherlands
Circ Res 85:1206-13. 1999..The functional consequence of both shifts is likely to be a reduced Na(+) current during the upstroke of the action potential. LQT(3) and Brugada syndrome are allelic disorders but may also share a common genotype...
Persistent precordial "hyperacute" T-waves signify proximal left anterior descending artery occlusionN J Verouden
Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands
Heart 95:1701-6. 2009..To describe patients with a distinct electrocardiogram (ECG) pattern without ST-segment elevation in the presence of an acute occlusion of the proximal left anterior descending (LAD) artery...
