The Experts below are selected from a list of 156 Experts worldwide ranked by ideXlab platform
A.a.m. Wilde - One of the best experts on this subject based on the ideXlab platform.
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Characterisation of familial idiopathic ventricular fibrillation linked to DPP6
European Heart Journal, 2013Co-Authors: Pieter G. Postema, J Ten N Sande, Imke Christiaans, M Boekholdt, Connie R Bezzina, X. Ling, Marielle Alders, András Varró, Stanley Nattel, A.a.m. WildeAbstract:Purpose: Idiopathic ventricular fibrillation (IVF) is a rare but notoriously difficult to manage arrhythmia syndrome as there are no clinical identifiers to establish the risk for a premature cardiac arrest. However, we recently uncovered a link between familial IVF and a risk haplotype on the new arrhythmia gene DPP6 on chromosome 7q36 in several related IVF families. DPP6 is putatively involved in the Ito current in heart. In this study we characterise both the extended bench and bedside evaluations in IVF linked to DPP6. Methods: We studied 235 carriers and 267 non-carriers of the DPP6 risk haplotype. Clinical parameters and a combination of all cause mortality and cardiac arrest were evaluated. Additional expression studies in human cardiac tissue samples and adenovirus-mediated gene transfer studies in cultured canine Purkinje cells and cardiomyocytes were performed. Results: Successfully resuscitated patients did not have any sign of other inheritable arrhythmia syndromes. Recorded IVF was always initiated by monomorphic short-coupled Extrasystoles from the right ventricular apex/lower free wall. Ablation of Extrasystole preceding Purkinje potentials and/or the administration quinidine led to suppression of subsequent arrhythmias. Carriers and non-carriers had normal and similar electrocardiographic and echocardiographic indices. Cardiac MRI showed a trend to slightly larger right ventricular volumes in carriers, but these were still within normal limits and not clinically useful. Death and/or cardiac arrest occurred in 84 carriers and 10 non-carriers. Median survival of carriers was 68 years (p
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characterisation of familial idiopathic ventricular fibrillation linked to dpp6
European Heart Journal, 2013Co-Authors: Pieter G. Postema, J Ten N Sande, Imke Christiaans, M Boekholdt, Connie R Bezzina, X. Ling, Marielle Alders, András Varró, Stanley Nattel, A.a.m. WildeAbstract:Purpose: Idiopathic ventricular fibrillation (IVF) is a rare but notoriously difficult to manage arrhythmia syndrome as there are no clinical identifiers to establish the risk for a premature cardiac arrest. However, we recently uncovered a link between familial IVF and a risk haplotype on the new arrhythmia gene DPP6 on chromosome 7q36 in several related IVF families. DPP6 is putatively involved in the Ito current in heart. In this study we characterise both the extended bench and bedside evaluations in IVF linked to DPP6. Methods: We studied 235 carriers and 267 non-carriers of the DPP6 risk haplotype. Clinical parameters and a combination of all cause mortality and cardiac arrest were evaluated. Additional expression studies in human cardiac tissue samples and adenovirus-mediated gene transfer studies in cultured canine Purkinje cells and cardiomyocytes were performed. Results: Successfully resuscitated patients did not have any sign of other inheritable arrhythmia syndromes. Recorded IVF was always initiated by monomorphic short-coupled Extrasystoles from the right ventricular apex/lower free wall. Ablation of Extrasystole preceding Purkinje potentials and/or the administration quinidine led to suppression of subsequent arrhythmias. Carriers and non-carriers had normal and similar electrocardiographic and echocardiographic indices. Cardiac MRI showed a trend to slightly larger right ventricular volumes in carriers, but these were still within normal limits and not clinically useful. Death and/or cardiac arrest occurred in 84 carriers and 10 non-carriers. Median survival of carriers was 68 years (p<0.01 vs non-carriers), with events starting from age 16 with a worse phenotype in males (median survival 59 vs 82 years, p<0.01 vs females). Biopsy studies uncovered DPP6 mRNA overexpression in carriers (p<0.01 vs controls). Studies in non-diseased explanted human hearts revealed higher DPP6 mRNA expression in Purkinje fibers than in other regions (p<0.01 vs ventricular myocardium) while DPP6-overexpression significantly increased Ito density in canine Purkinje cells (∼52%, p<0.05 vs control) but not in cardiomyocytes, further underpinning the importance of the Purkinje network in the DPP6 risk haplotype. Conclusions: A new arrhythmia gene DPP6 is linked to familial IVF and shows a malignant phenotype. Our results point to a pivotal role of DPP6 overexression in Purkinje fibers eliciting short-coupled Extrasystoles which trigger IVF. For the first time, only genetic predisposition can be used as a risk-marker for future events in IVF.
Pieter G. Postema - One of the best experts on this subject based on the ideXlab platform.
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Characterisation of familial idiopathic ventricular fibrillation linked to DPP6
European Heart Journal, 2013Co-Authors: Pieter G. Postema, J Ten N Sande, Imke Christiaans, M Boekholdt, Connie R Bezzina, X. Ling, Marielle Alders, András Varró, Stanley Nattel, A.a.m. WildeAbstract:Purpose: Idiopathic ventricular fibrillation (IVF) is a rare but notoriously difficult to manage arrhythmia syndrome as there are no clinical identifiers to establish the risk for a premature cardiac arrest. However, we recently uncovered a link between familial IVF and a risk haplotype on the new arrhythmia gene DPP6 on chromosome 7q36 in several related IVF families. DPP6 is putatively involved in the Ito current in heart. In this study we characterise both the extended bench and bedside evaluations in IVF linked to DPP6. Methods: We studied 235 carriers and 267 non-carriers of the DPP6 risk haplotype. Clinical parameters and a combination of all cause mortality and cardiac arrest were evaluated. Additional expression studies in human cardiac tissue samples and adenovirus-mediated gene transfer studies in cultured canine Purkinje cells and cardiomyocytes were performed. Results: Successfully resuscitated patients did not have any sign of other inheritable arrhythmia syndromes. Recorded IVF was always initiated by monomorphic short-coupled Extrasystoles from the right ventricular apex/lower free wall. Ablation of Extrasystole preceding Purkinje potentials and/or the administration quinidine led to suppression of subsequent arrhythmias. Carriers and non-carriers had normal and similar electrocardiographic and echocardiographic indices. Cardiac MRI showed a trend to slightly larger right ventricular volumes in carriers, but these were still within normal limits and not clinically useful. Death and/or cardiac arrest occurred in 84 carriers and 10 non-carriers. Median survival of carriers was 68 years (p
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characterisation of familial idiopathic ventricular fibrillation linked to dpp6
European Heart Journal, 2013Co-Authors: Pieter G. Postema, J Ten N Sande, Imke Christiaans, M Boekholdt, Connie R Bezzina, X. Ling, Marielle Alders, András Varró, Stanley Nattel, A.a.m. WildeAbstract:Purpose: Idiopathic ventricular fibrillation (IVF) is a rare but notoriously difficult to manage arrhythmia syndrome as there are no clinical identifiers to establish the risk for a premature cardiac arrest. However, we recently uncovered a link between familial IVF and a risk haplotype on the new arrhythmia gene DPP6 on chromosome 7q36 in several related IVF families. DPP6 is putatively involved in the Ito current in heart. In this study we characterise both the extended bench and bedside evaluations in IVF linked to DPP6. Methods: We studied 235 carriers and 267 non-carriers of the DPP6 risk haplotype. Clinical parameters and a combination of all cause mortality and cardiac arrest were evaluated. Additional expression studies in human cardiac tissue samples and adenovirus-mediated gene transfer studies in cultured canine Purkinje cells and cardiomyocytes were performed. Results: Successfully resuscitated patients did not have any sign of other inheritable arrhythmia syndromes. Recorded IVF was always initiated by monomorphic short-coupled Extrasystoles from the right ventricular apex/lower free wall. Ablation of Extrasystole preceding Purkinje potentials and/or the administration quinidine led to suppression of subsequent arrhythmias. Carriers and non-carriers had normal and similar electrocardiographic and echocardiographic indices. Cardiac MRI showed a trend to slightly larger right ventricular volumes in carriers, but these were still within normal limits and not clinically useful. Death and/or cardiac arrest occurred in 84 carriers and 10 non-carriers. Median survival of carriers was 68 years (p<0.01 vs non-carriers), with events starting from age 16 with a worse phenotype in males (median survival 59 vs 82 years, p<0.01 vs females). Biopsy studies uncovered DPP6 mRNA overexpression in carriers (p<0.01 vs controls). Studies in non-diseased explanted human hearts revealed higher DPP6 mRNA expression in Purkinje fibers than in other regions (p<0.01 vs ventricular myocardium) while DPP6-overexpression significantly increased Ito density in canine Purkinje cells (∼52%, p<0.05 vs control) but not in cardiomyocytes, further underpinning the importance of the Purkinje network in the DPP6 risk haplotype. Conclusions: A new arrhythmia gene DPP6 is linked to familial IVF and shows a malignant phenotype. Our results point to a pivotal role of DPP6 overexression in Purkinje fibers eliciting short-coupled Extrasystoles which trigger IVF. For the first time, only genetic predisposition can be used as a risk-marker for future events in IVF.
X. Ling - One of the best experts on this subject based on the ideXlab platform.
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Characterisation of familial idiopathic ventricular fibrillation linked to DPP6
European Heart Journal, 2013Co-Authors: Pieter G. Postema, J Ten N Sande, Imke Christiaans, M Boekholdt, Connie R Bezzina, X. Ling, Marielle Alders, András Varró, Stanley Nattel, A.a.m. WildeAbstract:Purpose: Idiopathic ventricular fibrillation (IVF) is a rare but notoriously difficult to manage arrhythmia syndrome as there are no clinical identifiers to establish the risk for a premature cardiac arrest. However, we recently uncovered a link between familial IVF and a risk haplotype on the new arrhythmia gene DPP6 on chromosome 7q36 in several related IVF families. DPP6 is putatively involved in the Ito current in heart. In this study we characterise both the extended bench and bedside evaluations in IVF linked to DPP6. Methods: We studied 235 carriers and 267 non-carriers of the DPP6 risk haplotype. Clinical parameters and a combination of all cause mortality and cardiac arrest were evaluated. Additional expression studies in human cardiac tissue samples and adenovirus-mediated gene transfer studies in cultured canine Purkinje cells and cardiomyocytes were performed. Results: Successfully resuscitated patients did not have any sign of other inheritable arrhythmia syndromes. Recorded IVF was always initiated by monomorphic short-coupled Extrasystoles from the right ventricular apex/lower free wall. Ablation of Extrasystole preceding Purkinje potentials and/or the administration quinidine led to suppression of subsequent arrhythmias. Carriers and non-carriers had normal and similar electrocardiographic and echocardiographic indices. Cardiac MRI showed a trend to slightly larger right ventricular volumes in carriers, but these were still within normal limits and not clinically useful. Death and/or cardiac arrest occurred in 84 carriers and 10 non-carriers. Median survival of carriers was 68 years (p
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characterisation of familial idiopathic ventricular fibrillation linked to dpp6
European Heart Journal, 2013Co-Authors: Pieter G. Postema, J Ten N Sande, Imke Christiaans, M Boekholdt, Connie R Bezzina, X. Ling, Marielle Alders, András Varró, Stanley Nattel, A.a.m. WildeAbstract:Purpose: Idiopathic ventricular fibrillation (IVF) is a rare but notoriously difficult to manage arrhythmia syndrome as there are no clinical identifiers to establish the risk for a premature cardiac arrest. However, we recently uncovered a link between familial IVF and a risk haplotype on the new arrhythmia gene DPP6 on chromosome 7q36 in several related IVF families. DPP6 is putatively involved in the Ito current in heart. In this study we characterise both the extended bench and bedside evaluations in IVF linked to DPP6. Methods: We studied 235 carriers and 267 non-carriers of the DPP6 risk haplotype. Clinical parameters and a combination of all cause mortality and cardiac arrest were evaluated. Additional expression studies in human cardiac tissue samples and adenovirus-mediated gene transfer studies in cultured canine Purkinje cells and cardiomyocytes were performed. Results: Successfully resuscitated patients did not have any sign of other inheritable arrhythmia syndromes. Recorded IVF was always initiated by monomorphic short-coupled Extrasystoles from the right ventricular apex/lower free wall. Ablation of Extrasystole preceding Purkinje potentials and/or the administration quinidine led to suppression of subsequent arrhythmias. Carriers and non-carriers had normal and similar electrocardiographic and echocardiographic indices. Cardiac MRI showed a trend to slightly larger right ventricular volumes in carriers, but these were still within normal limits and not clinically useful. Death and/or cardiac arrest occurred in 84 carriers and 10 non-carriers. Median survival of carriers was 68 years (p<0.01 vs non-carriers), with events starting from age 16 with a worse phenotype in males (median survival 59 vs 82 years, p<0.01 vs females). Biopsy studies uncovered DPP6 mRNA overexpression in carriers (p<0.01 vs controls). Studies in non-diseased explanted human hearts revealed higher DPP6 mRNA expression in Purkinje fibers than in other regions (p<0.01 vs ventricular myocardium) while DPP6-overexpression significantly increased Ito density in canine Purkinje cells (∼52%, p<0.05 vs control) but not in cardiomyocytes, further underpinning the importance of the Purkinje network in the DPP6 risk haplotype. Conclusions: A new arrhythmia gene DPP6 is linked to familial IVF and shows a malignant phenotype. Our results point to a pivotal role of DPP6 overexression in Purkinje fibers eliciting short-coupled Extrasystoles which trigger IVF. For the first time, only genetic predisposition can be used as a risk-marker for future events in IVF.
Stanley Nattel - One of the best experts on this subject based on the ideXlab platform.
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Characterisation of familial idiopathic ventricular fibrillation linked to DPP6
European Heart Journal, 2013Co-Authors: Pieter G. Postema, J Ten N Sande, Imke Christiaans, M Boekholdt, Connie R Bezzina, X. Ling, Marielle Alders, András Varró, Stanley Nattel, A.a.m. WildeAbstract:Purpose: Idiopathic ventricular fibrillation (IVF) is a rare but notoriously difficult to manage arrhythmia syndrome as there are no clinical identifiers to establish the risk for a premature cardiac arrest. However, we recently uncovered a link between familial IVF and a risk haplotype on the new arrhythmia gene DPP6 on chromosome 7q36 in several related IVF families. DPP6 is putatively involved in the Ito current in heart. In this study we characterise both the extended bench and bedside evaluations in IVF linked to DPP6. Methods: We studied 235 carriers and 267 non-carriers of the DPP6 risk haplotype. Clinical parameters and a combination of all cause mortality and cardiac arrest were evaluated. Additional expression studies in human cardiac tissue samples and adenovirus-mediated gene transfer studies in cultured canine Purkinje cells and cardiomyocytes were performed. Results: Successfully resuscitated patients did not have any sign of other inheritable arrhythmia syndromes. Recorded IVF was always initiated by monomorphic short-coupled Extrasystoles from the right ventricular apex/lower free wall. Ablation of Extrasystole preceding Purkinje potentials and/or the administration quinidine led to suppression of subsequent arrhythmias. Carriers and non-carriers had normal and similar electrocardiographic and echocardiographic indices. Cardiac MRI showed a trend to slightly larger right ventricular volumes in carriers, but these were still within normal limits and not clinically useful. Death and/or cardiac arrest occurred in 84 carriers and 10 non-carriers. Median survival of carriers was 68 years (p
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characterisation of familial idiopathic ventricular fibrillation linked to dpp6
European Heart Journal, 2013Co-Authors: Pieter G. Postema, J Ten N Sande, Imke Christiaans, M Boekholdt, Connie R Bezzina, X. Ling, Marielle Alders, András Varró, Stanley Nattel, A.a.m. WildeAbstract:Purpose: Idiopathic ventricular fibrillation (IVF) is a rare but notoriously difficult to manage arrhythmia syndrome as there are no clinical identifiers to establish the risk for a premature cardiac arrest. However, we recently uncovered a link between familial IVF and a risk haplotype on the new arrhythmia gene DPP6 on chromosome 7q36 in several related IVF families. DPP6 is putatively involved in the Ito current in heart. In this study we characterise both the extended bench and bedside evaluations in IVF linked to DPP6. Methods: We studied 235 carriers and 267 non-carriers of the DPP6 risk haplotype. Clinical parameters and a combination of all cause mortality and cardiac arrest were evaluated. Additional expression studies in human cardiac tissue samples and adenovirus-mediated gene transfer studies in cultured canine Purkinje cells and cardiomyocytes were performed. Results: Successfully resuscitated patients did not have any sign of other inheritable arrhythmia syndromes. Recorded IVF was always initiated by monomorphic short-coupled Extrasystoles from the right ventricular apex/lower free wall. Ablation of Extrasystole preceding Purkinje potentials and/or the administration quinidine led to suppression of subsequent arrhythmias. Carriers and non-carriers had normal and similar electrocardiographic and echocardiographic indices. Cardiac MRI showed a trend to slightly larger right ventricular volumes in carriers, but these were still within normal limits and not clinically useful. Death and/or cardiac arrest occurred in 84 carriers and 10 non-carriers. Median survival of carriers was 68 years (p<0.01 vs non-carriers), with events starting from age 16 with a worse phenotype in males (median survival 59 vs 82 years, p<0.01 vs females). Biopsy studies uncovered DPP6 mRNA overexpression in carriers (p<0.01 vs controls). Studies in non-diseased explanted human hearts revealed higher DPP6 mRNA expression in Purkinje fibers than in other regions (p<0.01 vs ventricular myocardium) while DPP6-overexpression significantly increased Ito density in canine Purkinje cells (∼52%, p<0.05 vs control) but not in cardiomyocytes, further underpinning the importance of the Purkinje network in the DPP6 risk haplotype. Conclusions: A new arrhythmia gene DPP6 is linked to familial IVF and shows a malignant phenotype. Our results point to a pivotal role of DPP6 overexression in Purkinje fibers eliciting short-coupled Extrasystoles which trigger IVF. For the first time, only genetic predisposition can be used as a risk-marker for future events in IVF.
András Varró - One of the best experts on this subject based on the ideXlab platform.
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Characterisation of familial idiopathic ventricular fibrillation linked to DPP6
European Heart Journal, 2013Co-Authors: Pieter G. Postema, J Ten N Sande, Imke Christiaans, M Boekholdt, Connie R Bezzina, X. Ling, Marielle Alders, András Varró, Stanley Nattel, A.a.m. WildeAbstract:Purpose: Idiopathic ventricular fibrillation (IVF) is a rare but notoriously difficult to manage arrhythmia syndrome as there are no clinical identifiers to establish the risk for a premature cardiac arrest. However, we recently uncovered a link between familial IVF and a risk haplotype on the new arrhythmia gene DPP6 on chromosome 7q36 in several related IVF families. DPP6 is putatively involved in the Ito current in heart. In this study we characterise both the extended bench and bedside evaluations in IVF linked to DPP6. Methods: We studied 235 carriers and 267 non-carriers of the DPP6 risk haplotype. Clinical parameters and a combination of all cause mortality and cardiac arrest were evaluated. Additional expression studies in human cardiac tissue samples and adenovirus-mediated gene transfer studies in cultured canine Purkinje cells and cardiomyocytes were performed. Results: Successfully resuscitated patients did not have any sign of other inheritable arrhythmia syndromes. Recorded IVF was always initiated by monomorphic short-coupled Extrasystoles from the right ventricular apex/lower free wall. Ablation of Extrasystole preceding Purkinje potentials and/or the administration quinidine led to suppression of subsequent arrhythmias. Carriers and non-carriers had normal and similar electrocardiographic and echocardiographic indices. Cardiac MRI showed a trend to slightly larger right ventricular volumes in carriers, but these were still within normal limits and not clinically useful. Death and/or cardiac arrest occurred in 84 carriers and 10 non-carriers. Median survival of carriers was 68 years (p
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characterisation of familial idiopathic ventricular fibrillation linked to dpp6
European Heart Journal, 2013Co-Authors: Pieter G. Postema, J Ten N Sande, Imke Christiaans, M Boekholdt, Connie R Bezzina, X. Ling, Marielle Alders, András Varró, Stanley Nattel, A.a.m. WildeAbstract:Purpose: Idiopathic ventricular fibrillation (IVF) is a rare but notoriously difficult to manage arrhythmia syndrome as there are no clinical identifiers to establish the risk for a premature cardiac arrest. However, we recently uncovered a link between familial IVF and a risk haplotype on the new arrhythmia gene DPP6 on chromosome 7q36 in several related IVF families. DPP6 is putatively involved in the Ito current in heart. In this study we characterise both the extended bench and bedside evaluations in IVF linked to DPP6. Methods: We studied 235 carriers and 267 non-carriers of the DPP6 risk haplotype. Clinical parameters and a combination of all cause mortality and cardiac arrest were evaluated. Additional expression studies in human cardiac tissue samples and adenovirus-mediated gene transfer studies in cultured canine Purkinje cells and cardiomyocytes were performed. Results: Successfully resuscitated patients did not have any sign of other inheritable arrhythmia syndromes. Recorded IVF was always initiated by monomorphic short-coupled Extrasystoles from the right ventricular apex/lower free wall. Ablation of Extrasystole preceding Purkinje potentials and/or the administration quinidine led to suppression of subsequent arrhythmias. Carriers and non-carriers had normal and similar electrocardiographic and echocardiographic indices. Cardiac MRI showed a trend to slightly larger right ventricular volumes in carriers, but these were still within normal limits and not clinically useful. Death and/or cardiac arrest occurred in 84 carriers and 10 non-carriers. Median survival of carriers was 68 years (p<0.01 vs non-carriers), with events starting from age 16 with a worse phenotype in males (median survival 59 vs 82 years, p<0.01 vs females). Biopsy studies uncovered DPP6 mRNA overexpression in carriers (p<0.01 vs controls). Studies in non-diseased explanted human hearts revealed higher DPP6 mRNA expression in Purkinje fibers than in other regions (p<0.01 vs ventricular myocardium) while DPP6-overexpression significantly increased Ito density in canine Purkinje cells (∼52%, p<0.05 vs control) but not in cardiomyocytes, further underpinning the importance of the Purkinje network in the DPP6 risk haplotype. Conclusions: A new arrhythmia gene DPP6 is linked to familial IVF and shows a malignant phenotype. Our results point to a pivotal role of DPP6 overexression in Purkinje fibers eliciting short-coupled Extrasystoles which trigger IVF. For the first time, only genetic predisposition can be used as a risk-marker for future events in IVF.
