Exercise related ventricular arrhythmias are related to cardiac fibrosis in hypertrophic cardiomyopathy mutation carriers
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.
We studied the prevalence and origin of ERVA and related these with fibrosis on CMR in a population of 31 HCM mutation carriers.
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.
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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- Exercise related ventricular arrhythmias are related to cardiac fibrosis in hypertrophic cardiomyopathy mutation carriers
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Netherlands Heart Journal
Volume 19, Issue 4 , pp 168-174
- Cover Date
- Print ISSN
- Online ISSN
- Bohn Stafleu van Loghum
- Additional Links
- Hypertrophic cardiomyopathy
- Magnetic resonance imaging
- Author Affiliations
- 1. Department of Cardiology, Maastricht University Medical Center, Maastricht, the Netherlands
- 5. Department of Cardiology, Academic Medical Center, PO Box 22660, 1100 DD, Amsterdam, the Netherlands
- 2. Department of Radiology, Maastricht University Medical Center, Maastricht, the Netherlands
- 3. Department of Genetics, Maastricht University Medical Center, Maastricht, the Netherlands
- 4. Department of Molecular Genetics, Maastricht University Medical Center, Maastricht, the Netherlands