Advanced CMR techniques in Anderson-Fabry disease: state of the art
A Ponsiglione, M De Giorgi, R Ascione, C Nappi… - Diagnostics, 2023 - mdpi.com
Anderson-Fabry disease (AFD) is a rare multisystem X-linked lysosomal storage disorder
caused by α-galactosidase A enzyme deficiency. Long-term cardiac involvement in AFD …
caused by α-galactosidase A enzyme deficiency. Long-term cardiac involvement in AFD …
Cardiac involvement in Anderson–Fabry disease. The role of advanced echocardiography
L Spinelli, A Bianco, E Riccio, A Pisani… - Frontiers in …, 2024 - frontiersin.org
Anderson–Fabry disease (AFD) is a lysosomal storage disorder, depending on defects in
alpha galactosidase A activity, due to a mutation in the galactosidase alpha gene …
alpha galactosidase A activity, due to a mutation in the galactosidase alpha gene …
Left atrial remodeling in hypertrophic cardiomyopathy and Fabry disease: A CMR-based head-to-head comparison and outcome analysis
Background Hypertrophic cardiomyopathy (HCM) and Fabry disease cardiomyopathy (FD)
are phenocopies, as they show left ventricular hypertrophy (LVH). The left atrium (LA) is …
are phenocopies, as they show left ventricular hypertrophy (LVH). The left atrium (LA) is …
Impact of enzyme replacement therapy and migalastat on left atrial strain and cardiomyopathy in patients with Fabry disease
C Pogoda, SM Brand, T Duning… - Frontiers in …, 2023 - frontiersin.org
Aims Cardiomyopathy in Fabry disease (FD) is a major determinant of morbidity and
mortality. This study investigates the effects of FD-specific treatment using enzyme …
mortality. This study investigates the effects of FD-specific treatment using enzyme …
Feature tracking cardiac magnetic resonance imaging to assess cardiac manifestations of systemic diseases
Feature-tracking cardiac magnetic resonance (FT-CMR), with the ability to quantify
myocardial deformation, has a unique role in the evaluation of subclinical myocardial …
myocardial deformation, has a unique role in the evaluation of subclinical myocardial …
Cardiopulmonary determinants of reduced exercise tolerance in Fabry disease
O De Marco, J Gambardella, A Bianco… - Frontiers in …, 2024 - frontiersin.org
Fabry disease (FD), also known as Anderson-Fabry disease, is a hereditary disorder of
glycosphingolipid metabolism, caused by a deficiency of the lysosomal alpha-galactosidase …
glycosphingolipid metabolism, caused by a deficiency of the lysosomal alpha-galactosidase …
Screening for Fabry disease in patients with hypertrophic cardiomyopathy using cardiac magnetic resonance imaging
J Li, L Pu, Z Xu, K Wan, Y Xu, J Wang, Y Han… - European Radiology, 2024 - Springer
Background Fabry disease (FD) usually mimics hypertrophic cardiomyopathy (HCM).
Decreased native T1 mapping and a unique late gadolinium enhancement (LGE) pattern by …
Decreased native T1 mapping and a unique late gadolinium enhancement (LGE) pattern by …
[HTML][HTML] Reproducibility assessment of rapid strains in cardiac MRI: Insights and recommendations for clinical application
MC Halfmann, LHGA Hopman, H Körperich… - European Journal of …, 2024 - Elsevier
Purpose Studies have shown the incremental value of strain imaging in various cardiac
diseases. However, reproducibility and generalizability has remained an issue of concern …
diseases. However, reproducibility and generalizability has remained an issue of concern …
Left atrial strain in fetal echocardiography–could it be introduced to everyday clinical practice?
Objectives Prenatal cardiology is a part of preventive cardiology based on fetal
echocardiography and fetal interventional cardiology, which facilitates treatment of …
echocardiography and fetal interventional cardiology, which facilitates treatment of …
Left atrial strain analysis in the realm of pediatric cardiology: Advantages and implications
BACKGROUND: Left atrial (LA) strain analysis has emerged as a noninvasive technique for
assessing LA function and early detection of myocardial deformation. Recently, its …
assessing LA function and early detection of myocardial deformation. Recently, its …