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Published in: Netherlands Heart Journal 4/2011

Open Access 01-04-2011 | Original Article

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

Authors: I. A. W. van Rijsingen, S. C. A. M. Bekkers, S. Schalla, J. F. Hermans-van Ast, G. Snoep, B. S. N. Alzand, Y. H. J. M. Arens, A. van den Wijngaard, H. J. G. M. Crijns, Y. M. Pinto

Published in: Netherlands Heart Journal | Issue 4/2011

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Abstract

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.
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Metadata
Title
Exercise related ventricular arrhythmias are related to cardiac fibrosis in hypertrophic cardiomyopathy mutation carriers
Authors
I. A. W. van Rijsingen
S. C. A. M. Bekkers
S. Schalla
J. F. Hermans-van Ast
G. Snoep
B. S. N. Alzand
Y. H. J. M. Arens
A. van den Wijngaard
H. J. G. M. Crijns
Y. M. Pinto
Publication date
01-04-2011
Publisher
Bohn Stafleu van Loghum
Published in
Netherlands Heart Journal / Issue 4/2011
Print ISSN: 1568-5888
Electronic ISSN: 1876-6250
DOI
https://doi.org/10.1007/s12471-011-0090-8

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