Multimodality imaging in Fabry cardiomyopathy: from early diagnosis to therapeutic targets
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11110%2F18%3A10386248" target="_blank" >RIV/00216208:11110/18:10386248 - isvavai.cz</a>
Alternative codes found
RIV/00064165:_____/18:10386248
Result on the web
<a href="https://doi.org/10.1093/ehjci/jey132" target="_blank" >https://doi.org/10.1093/ehjci/jey132</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1093/ehjci/jey132" target="_blank" >10.1093/ehjci/jey132</a>
Alternative languages
Result language
angličtina
Original language name
Multimodality imaging in Fabry cardiomyopathy: from early diagnosis to therapeutic targets
Original language description
Fabry disease (FD) is a rare genetic X-linked disorder that can impact multiple organs. Cardiac involvement influences the prognosis of patients with FD, being one of the main causes of mortality. Cardiac imaging has proven essential in all aspects of Fabry cardiomyopathy evaluation, such as diagnosis (including detection of early organ changes), disease progression, and guideline for starting enzyme therapy. Imaging techniques used in cardiac evaluation of FD range from 2D and deformation studies in echocardiography to cardiac magnetic resonance (CMR), cardiac scintigraphy and positron emission tomography. The present review summarizes the imaging 'red flags' demonstrated to be able to differentiate early cardiac FD from normal controls and Fabry cardiomyopathy from other causes of hypertrophic cardiomyopathy. Also, it discusses the current evidence for the role of CMR in myocardial tissue characterization, as well as other imaging methods that have shown promise in FD. Current knowledge of the evaluation of the presence and extent of cardiac involvement at baseline and during follow-up of enzyme therapy efficiency are further presented. Multiple studies demonstrated that imaging parameters can be reliably used in establishing diagnosis and monitoring therapy in FD. Given the rarity of this disorder, we conclude that awareness should be raised about these imaging 'red flags' and likely patients sent for evaluation in expert centres.
Czech name
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Czech description
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Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
30201 - Cardiac and Cardiovascular systems
Result continuities
Project
—
Continuities
V - Vyzkumna aktivita podporovana z jinych verejnych zdroju
Others
Publication year
2018
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
European Heart Journal: Cardiovascular Imaging
ISSN
2047-2404
e-ISSN
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Volume of the periodical
19
Issue of the periodical within the volume
12
Country of publishing house
GB - UNITED KINGDOM
Number of pages
10
Pages from-to
1313-1322
UT code for WoS article
000455360200001
EID of the result in the Scopus database
2-s2.0-85056802005