Biventricular strain assessment indicates progressive impairment of myocardial contractility in phenotypically negative patients with Fabry's disease
MC Halfmann, S Benz, UJ Schoepf… - European Journal of …, 2022 - Elsevier
Purpose The accumulation of sphingolipids in Fabry's disease (FD) leads to left ventricular
(LV) hypertrophy and shortened T1 in cardiac magnetic resonance (CMR). Early detection of …
(LV) hypertrophy and shortened T1 in cardiac magnetic resonance (CMR). Early detection of …
Global longitudinal strain, myocardial storage and hypertrophy in Fabry disease
Introduction Detecting early cardiac involvement in Fabry disease (FD) is important because
therapy may alter disease progression. Cardiovascular magnetic resonance (CMR) can …
therapy may alter disease progression. Cardiovascular magnetic resonance (CMR) can …
[HTML][HTML] Quantification of myocardial deformation in patients with Fabry disease by cardiovascular magnetic resonance feature tracking imaging
L Zhao, C Zhang, J Tian, M Saiedi, C Ma… - Cardiovascular …, 2021 - ncbi.nlm.nih.gov
Background Cardiac involvement is a major contributor of morbidity and mortality in Fabry
disease (FD). Early detection and accurate evaluation of the disease progression is …
disease (FD). Early detection and accurate evaluation of the disease progression is …
T1 mapping in patients with Fabry disease predicts functional and morphological abnormalities in cardiac MRI
C Gatterer, G Mundigler, S Graf, D Beitzke… - European Heart …, 2020 - academic.oup.com
Abstract Introduction About 50% of Fabry disease (FD) patients develop cardiac
manifestation, also known as Fabry cardiomyopathy. Beside echocardiography, cardiac …
manifestation, also known as Fabry cardiomyopathy. Beside echocardiography, cardiac …
[HTML][HTML] A disproportionate contribution of papillary muscles and trabeculations to total left ventricular mass makes choice of cardiovascular magnetic resonance …
R Kozor, F Callaghan, M Tchan… - Journal of …, 2015 - Elsevier
Abstract Background Sphingolipid deposition in Fabry disease causes left ventricular (LV)
hypertrophy, of which the accurate assessment is essential. Cardiovascular magnetic …
hypertrophy, of which the accurate assessment is essential. Cardiovascular magnetic …
The myocardial phenotype of Fabry disease pre-hypertrophy and pre-detectable storage
JB Augusto, N Johner, D Shah, S Nordin… - European Heart …, 2021 - academic.oup.com
Abstract Aims Cardiac involvement in Fabry disease (FD) occurs prior to left ventricular
hypertrophy (LVH) and is characterized by low myocardial native T1 with sphingolipid …
hypertrophy (LVH) and is characterized by low myocardial native T1 with sphingolipid …
Reduced global longitudinal strain as a marker for early detection of Fabry cardiomyopathy
Aims Fabry cardiomyopathy (FC) is characterized by progressive left ventricular hypertrophy
(LVH). Conventional echocardiography is not sensitive in detecting preclinical FC before the …
(LVH). Conventional echocardiography is not sensitive in detecting preclinical FC before the …
54 Characterisation of systolic myocardial strain in patients with fabry disease
Background Fabry disease (FD) is an X-linked lysosomal storage disorder leading to
intracellular accumulation of glycosphingolipid affecting multiple organs including the heart …
intracellular accumulation of glycosphingolipid affecting multiple organs including the heart …
Myocardial lipid content in Fabry disease: a combined 1H-MR spectroscopy and MR imaging study at 3 Tesla
B Petritsch, H Koestler, AM Weng, M Horn… - BMC Cardiovascular …, 2016 - Springer
Background Fabry disease is characterized by a progressive deposition of sphingolipids in
different organ systems, whereby cardiac involvement leads to death. We hypothesize that …
different organ systems, whereby cardiac involvement leads to death. We hypothesize that …
Value of left ventricular feature tracking strain analysis for detection of early cardiac involvement in Fabry disease (FD)
FC Roller, A Brose, M Richter, A Schüssler… - Journal of Clinical …, 2021 - mdpi.com
Purpose: Detection of cardiac involvement in Fabry disease (FD) is of high importance for
treatment management. Native T1 mapping especially showed great potential for detection …
treatment management. Native T1 mapping especially showed great potential for detection …
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