Normal left-atrial structure and function despite concentric left-ventricular remodelling in a cohort of patients with Anderson-Fabry disease.
OBJECTIVE
Anderson-Fabry Disease (AFD) is an important cause of cardiomyopathy characterized by concentric left-ventricular hypertrophy (LVH). We evaluated the extent of left-atrial (LA) structural and functional remodelling in this group of patients given that LA remodelling is a marker of adverse outcomes in the presence of LVH.


RESULTS
Clinical profiles were obtained and cardiac MRI was performed in cohorts of patients with AFD (n = 31), healthy controls (n = 23), and a positive control cohort with known concentric remodelling and LVH (CR/H, n = 21). Of patients with AFD, 58% were on enzyme-replacement therapy (ERT), 84% were on renin-angiotensin system antagonism, and 65% were on statins. Despite a similar increase in LV mass index in the AFD when compared with the CR/H cohort, mean LA volumes for the AFD group were similar to those seen in the healthy control group. Following from this, we observed that the percentage contribution to LV stroke volume due to elastic/passive and active LA emptying was similar in the AFD and healthy control groups, while passive emptying was significantly lower in the CR/H group. The consequences of LVH in the AFD cohort were manifested in atrioventricular uncoupling, whereby the extent of elastic/passive and active LA emptying was not a function of the extent of longitudinal movement of the mitral annular plane, as it was in healthy control subjects.


CONCLUSIONS
Left-atrial structure and function were relatively normal in our cohort of patients with AFD, who were also judiciously treated with a contemporary strategy that includes renin-angiotensin system antagonism, statins, and ERT.
Division of Cardiology, Department of Medicine, Mazankowski Alberta Heart Institute, University of Alberta, 2C2 Walter Mackenzie Health Sciences Centre, Edmonton, Alberta, Canada.
Brendan N
BN
Putko
Division of Cardiology, Department of Medicine, Mazankowski Alberta Heart Institute, University of Alberta, 2C2 Walter Mackenzie Health Sciences Centre, Edmonton, Alberta, Canada.
Haran
H
Yogasundaram
Department of Biomedical Engineering, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada.
Kelvin
K
Chow
Department of Biomedical Engineering, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada.
Joseph
J
Pagano
Department of Medical Genetics and Pediatrics, University of Calgary, Calgary, Alberta, Canada.
Aneal
A
Khan
Division of Cardiology, Department of Medicine, Mazankowski Alberta Heart Institute, University of Alberta, 2C2 Walter Mackenzie Health Sciences Centre, Edmonton, Alberta, Canada.
D Ian
DI
Paterson
Department of Biomedical Engineering, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada.
Richard B
RB
Thompson
Division of Cardiology, Department of Medicine, Mazankowski Alberta Heart Institute, University of Alberta, 2C2 Walter Mackenzie Health Sciences Centre, Edmonton, Alberta, Canada gavin.oudit@ualberta.ca.
Gavin Y
GY
Oudit
D000328:Adult; D016275:Atrial Function / Q000502:physiology*; D016022:Case-Control Studies; D000795:Fabry Disease / Q000188:drug therapy / Q000503:physiopathology*; D005260:Female; D006325:Heart Atria / Q000033:anatomy & histology*; D006801:Humans; D017379:Hypertrophy, Left Ventricular / Q000188:drug therapy / Q000503:physiopathology*; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008875:Middle Aged; D012720:Severity of Illness Index; D020257:Ventricular Remodeling / Q000502:physiology*
Anderson-Fabry disease; Cardiomyopathy; Left atrium; Left ventricular hypertrophy classification