TY - JOUR
T1 - Exercise related ventricular arrhythmias are related to cardiac fibrosis in hypertrophic cardiomyopathy mutation carriers
AU - van Rijsingen, I. A. W.
AU - Bekkers, S. C. A. M.
AU - Schalla, S.
AU - Hermans-van Ast, J. F.
AU - Snoep, G.
AU - Alzand, Becker S. N.
AU - Arens, Y. H. J. M.
AU - van den Wijngaard, A.
AU - Crijns, H. J. G. M.
AU - Pinto, Y. M.
PY - 2011/4
Y1 - 2011/4
N2 - 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.
AB - 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.
KW - Hypertrophic cardiomyopathy
KW - Magnetic resonance imaging
KW - Exercise
KW - Fibrosis
KW - Arrhythmias
U2 - 10.1007/s12471-011-0090-8
DO - 10.1007/s12471-011-0090-8
M3 - Article
C2 - 21475680
SN - 1568-5888
VL - 19
SP - 168
EP - 174
JO - Netherlands Heart Journal
JF - Netherlands Heart Journal
IS - 4
ER -