Cargando…

Exercise related ventricular arrhythmias are related to cardiac fibrosis in hypertrophic cardiomyopathy mutation carriers

AIMS: Hypertrophic cardiomyopathy (HCM) is a frequent cause of sudden cardiac death (SCD) due to exercise-related ventricular arrhythmias (ERVA); however the pathological substrate is uncertain. The aim was to determine the prevalence of ERVA and their relation with fibrosis as determined by cardiac...

Descripción completa

Detalles Bibliográficos
Autores principales: van Rijsingen, I. A. W., Bekkers, S. C. A. M., Schalla, S., Hermans-van Ast, J. F., Snoep, G., Alzand, B. S. N., Arens, Y. H. J. M., van den Wijngaard, A., Crijns, H. J. G. M., Pinto, Y. M.
Formato: Texto
Lenguaje:English
Publicado: Bohn Stafleu van Loghum 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3058538/
https://www.ncbi.nlm.nih.gov/pubmed/21475680
http://dx.doi.org/10.1007/s12471-011-0090-8
_version_ 1782200371447332864
author van Rijsingen, I. A. W.
Bekkers, S. C. A. M.
Schalla, S.
Hermans-van Ast, J. F.
Snoep, G.
Alzand, B. S. N.
Arens, Y. H. J. M.
van den Wijngaard, A.
Crijns, H. J. G. M.
Pinto, Y. M.
author_facet van Rijsingen, I. A. W.
Bekkers, S. C. A. M.
Schalla, S.
Hermans-van Ast, J. F.
Snoep, G.
Alzand, B. S. N.
Arens, Y. H. J. M.
van den Wijngaard, A.
Crijns, H. J. G. M.
Pinto, Y. M.
author_sort van Rijsingen, I. A. W.
collection PubMed
description AIMS: Hypertrophic cardiomyopathy (HCM) is a frequent cause of sudden cardiac death (SCD) due to exercise-related ventricular arrhythmias (ERVA); however the pathological substrate is uncertain. The aim was to determine the prevalence of ERVA and their relation with fibrosis as determined by cardiac magnetic resonance imaging (CMR) in carriers of an HCM causing mutation. METHODS: We studied the prevalence and origin of ERVA and related these with fibrosis on CMR in a population of 31 HCM mutation carriers. RESULTS: ERVA occurred in seven patients (23%) who all showed evidence of fibrosis (100% ERVA(+) vs. 58% ERVA(-), p = 0.04). No ventricular tachycardia or ventricular fibrillation occurred. In patients with ERVA, the extent of fibrosis was significantly larger (8 ± 4% vs. 3 ± 4%, p = 0.02). ERVA originated from areas with a high extent of fibrosis or regions directly adjacent to these areas. CONCLUSIONS: ERVA in HCM mutation carriers arose from the area of fibrosis detected by CMR; ERVA seems closely related to cardiac fibrosis. Fibrosis as detected by CMR should be evaluated as an additional risk factor to further delineate risk of SCD in carriers of an HCM causing mutation.
format Text
id pubmed-3058538
institution National Center for Biotechnology Information
language English
publishDate 2011
publisher Bohn Stafleu van Loghum
record_format MEDLINE/PubMed
spelling pubmed-30585382011-04-05 Exercise related ventricular arrhythmias are related to cardiac fibrosis in hypertrophic cardiomyopathy mutation carriers van Rijsingen, I. A. W. Bekkers, S. C. A. M. Schalla, S. Hermans-van Ast, J. F. Snoep, G. Alzand, B. S. N. Arens, Y. H. J. M. van den Wijngaard, A. Crijns, H. J. G. M. Pinto, Y. M. Neth Heart J Original Article AIMS: Hypertrophic cardiomyopathy (HCM) is a frequent cause of sudden cardiac death (SCD) due to exercise-related ventricular arrhythmias (ERVA); however the pathological substrate is uncertain. The aim was to determine the prevalence of ERVA and their relation with fibrosis as determined by cardiac magnetic resonance imaging (CMR) in carriers of an HCM causing mutation. METHODS: We studied the prevalence and origin of ERVA and related these with fibrosis on CMR in a population of 31 HCM mutation carriers. RESULTS: ERVA occurred in seven patients (23%) who all showed evidence of fibrosis (100% ERVA(+) vs. 58% ERVA(-), p = 0.04). No ventricular tachycardia or ventricular fibrillation occurred. In patients with ERVA, the extent of fibrosis was significantly larger (8 ± 4% vs. 3 ± 4%, p = 0.02). ERVA originated from areas with a high extent of fibrosis or regions directly adjacent to these areas. CONCLUSIONS: ERVA in HCM mutation carriers arose from the area of fibrosis detected by CMR; ERVA seems closely related to cardiac fibrosis. Fibrosis as detected by CMR should be evaluated as an additional risk factor to further delineate risk of SCD in carriers of an HCM causing mutation. Bohn Stafleu van Loghum 2011-02-17 2011-04 /pmc/articles/PMC3058538/ /pubmed/21475680 http://dx.doi.org/10.1007/s12471-011-0090-8 Text en © The Author(s) 2011 https://creativecommons.org/licenses/by-nc/4.0/This article is distributed under the terms of the Creative Commons Attribution Noncommercial License which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author(s) and source are credited.
spellingShingle Original Article
van Rijsingen, I. A. W.
Bekkers, S. C. A. M.
Schalla, S.
Hermans-van Ast, J. F.
Snoep, G.
Alzand, B. S. N.
Arens, Y. H. J. M.
van den Wijngaard, A.
Crijns, H. J. G. M.
Pinto, Y. M.
Exercise related ventricular arrhythmias are related to cardiac fibrosis in hypertrophic cardiomyopathy mutation carriers
title Exercise related ventricular arrhythmias are related to cardiac fibrosis in hypertrophic cardiomyopathy mutation carriers
title_full Exercise related ventricular arrhythmias are related to cardiac fibrosis in hypertrophic cardiomyopathy mutation carriers
title_fullStr Exercise related ventricular arrhythmias are related to cardiac fibrosis in hypertrophic cardiomyopathy mutation carriers
title_full_unstemmed Exercise related ventricular arrhythmias are related to cardiac fibrosis in hypertrophic cardiomyopathy mutation carriers
title_short Exercise related ventricular arrhythmias are related to cardiac fibrosis in hypertrophic cardiomyopathy mutation carriers
title_sort exercise related ventricular arrhythmias are related to cardiac fibrosis in hypertrophic cardiomyopathy mutation carriers
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3058538/
https://www.ncbi.nlm.nih.gov/pubmed/21475680
http://dx.doi.org/10.1007/s12471-011-0090-8
work_keys_str_mv AT vanrijsingeniaw exerciserelatedventriculararrhythmiasarerelatedtocardiacfibrosisinhypertrophiccardiomyopathymutationcarriers
AT bekkersscam exerciserelatedventriculararrhythmiasarerelatedtocardiacfibrosisinhypertrophiccardiomyopathymutationcarriers
AT schallas exerciserelatedventriculararrhythmiasarerelatedtocardiacfibrosisinhypertrophiccardiomyopathymutationcarriers
AT hermansvanastjf exerciserelatedventriculararrhythmiasarerelatedtocardiacfibrosisinhypertrophiccardiomyopathymutationcarriers
AT snoepg exerciserelatedventriculararrhythmiasarerelatedtocardiacfibrosisinhypertrophiccardiomyopathymutationcarriers
AT alzandbsn exerciserelatedventriculararrhythmiasarerelatedtocardiacfibrosisinhypertrophiccardiomyopathymutationcarriers
AT arensyhjm exerciserelatedventriculararrhythmiasarerelatedtocardiacfibrosisinhypertrophiccardiomyopathymutationcarriers
AT vandenwijngaarda exerciserelatedventriculararrhythmiasarerelatedtocardiacfibrosisinhypertrophiccardiomyopathymutationcarriers
AT crijnshjgm exerciserelatedventriculararrhythmiasarerelatedtocardiacfibrosisinhypertrophiccardiomyopathymutationcarriers
AT pintoym exerciserelatedventriculararrhythmiasarerelatedtocardiacfibrosisinhypertrophiccardiomyopathymutationcarriers