Normal left-atrial structure and function despite concentric left-ventricular remodelling in a cohort of patients with Anderson-Fabry disease

European Heart Journal Cardiovascular Imaging
Brendan N PutkoGavin Y Oudit

Abstract

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. 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 ...Continue Reading

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Citations

Oct 27, 2017·European Heart Journal Cardiovascular Imaging·Thor EdvardsenBogdan A Popescu

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