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 …

Global longitudinal strain, myocardial storage and hypertrophy in Fabry disease

R Vijapurapu, S Nordin, S Baig, B Liu, S Rosmini… - Heart, 2019 - heart.bmj.com
Introduction Detecting early cardiac involvement in Fabry disease (FD) is important because
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 …

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 …

[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 …

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 …

Reduced global longitudinal strain as a marker for early detection of Fabry cardiomyopathy

DY Lu, WM Huang, WT Wang, SC Hung… - European Heart …, 2022 - academic.oup.com
Aims Fabry cardiomyopathy (FC) is characterized by progressive left ventricular hypertrophy
(LVH). Conventional echocardiography is not sensitive in detecting preclinical FC before the …

54 Characterisation of systolic myocardial strain in patients with fabry disease

R Vijapurapu, S Nordin, S Baig, B Liu, R Kozor… - Heart, 2018 - search.proquest.com
Background Fabry disease (FD) is an X-linked lysosomal storage disorder leading to
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 …

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 …