Two-dimensional speckle tracking as a non-invasive tool for identification of myocardial fibrosis in Fabry disease

This cross-sectional study aimed to analyse myocardial deformation in patients with Fabry disease (FD) in order to evaluate speckle tracking as a method for non-invasive determination of myocardial fibrosis. Myocardial fibrosis is common in Fabry cardiomyopathy and is associated with disease progres...

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Bibliographic Details
Published in:European heart journal 2013-06, Vol.34 (21), p.1587-1596
Main Authors: Krämer, Johannes, Niemann, Markus, Liu, Dan, Hu, Kai, Machann, Wolfram, Beer, Meinrad, Wanner, Christoph, Ertl, Georg, Weidemann, Frank
Format: Article
Language:English
Subjects:
Cross-Sectional Studies
Echocardiography - methods
Fabry Disease - pathology
Fabry Disease - physiopathology
Female
Fibrosis - pathology
Fibrosis - physiopathology
Humans
Hypertrophy, Left Ventricular - pathology
Hypertrophy, Left Ventricular - physiopathology
Magnetic Resonance Angiography - methods
Male
Middle Aged
Myocardium - pathology
ROC Curve
Stress, Physiological
Systole
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Summary:This cross-sectional study aimed to analyse myocardial deformation in patients with Fabry disease (FD) in order to evaluate speckle tracking as a method for non-invasive determination of myocardial fibrosis. Myocardial fibrosis is common in Fabry cardiomyopathy and is associated with disease progression and severe prognosis. In 101 consecutive Fabry patients (39.8 ± 12.9 years; 42 males), the quantitative measurement of myocardial fibrosis with magnetic resonance imaging was compared with regional myocardial deformation assessed by speckle-tracking imaging. Patients were analysed in relation to per cent of late-enhancement (LE)-positive areas of left-ventricular (LV) mass. Fifty-two patients (51%) displayed LE with a mean volume of 1.2 ± 1.8% of total LV mass. Predominantly basal lateral and posterior segments were affected. Patients with LE had lower global systolic longitudinal strain than those without (LE -14.8 ± 3.5% and -18.9 ± 2.1%, respectively; P < 0.001). Loss of global deformation, quantified by speckle tracking, was predominantly caused by basal posterior (P = 0.049) and lateral (P = 0.005) segments and global systolic strain correlated with the amount of LE (r = 0.543; P < 0.001). Patients with severe LE (>2%, n = 22) showed the lowest deformation values (-5.9 ± 8.4%) in basal postero-lateral segments when compared with those with mild (
ISSN:0195-668X
1522-9645
DOI:10.1093/eurheartj/eht098