Structural and functional cardiac analyses using modern and sensitive myocardial techniques in adult Pompe disease
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00023001%3A_____%2F15%3A00059689" target="_blank" >RIV/00023001:_____/15:00059689 - isvavai.cz</a>
Result on the web
<a href="http://link.springer.com/article/10.1007%2Fs10554-015-0629-7" target="_blank" >http://link.springer.com/article/10.1007%2Fs10554-015-0629-7</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1007/s10554-015-0629-7" target="_blank" >10.1007/s10554-015-0629-7</a>
Alternative languages
Result language
angličtina
Original language name
Structural and functional cardiac analyses using modern and sensitive myocardial techniques in adult Pompe disease
Original language description
The purpose of this study was to analyze comprehensively the heart using modern and sensitive myocardial techniques in order to determine if structural or functional cardiac alterations are present in adult Pompe disease. Twelve patients with adult Pompe disease and a control group of 187 healthy subjects of similar age and gender were included. Structural and functional cardiac characteristics were analyzed by conventional and 2D speckle-tracking echocardiography. In addition, in a subgroup of adult Pompe patients, we analyzed the myocardial and musculoskeletal features by means of cardiac and whole-body muscle magnetic resonance imaging. Patients with Pompe disease had significant structural and functional musculoskeletal alterations such as atrophy with fatty replacement and weakness in trunk and extremities. In contrast, Pompe patients had similar structural and functional myocardial features to healthy subjects (LV strain -20.7 +/- A 1.9 vs. -21.3 +/- A 2.1 %; RV strain -24.2 +/- A 5.3 vs. -24.8 +/- A 3.8 %; LA strain 41.5 +/- A 10.3 vs. 44.8 +/- A 11.0 %; P > 0.05; and no evidence of LV and RV hypertrophy or LA enlargement). In addition, there was no evidence of valvular cardiac alterations, electrocardiographic abnormalities, or myocardial fibrosis in Pompe patients. In the current study analyzing the heart with modern and sensitive myocardial techniques, we evidenced that functional and structural cardiac alterations are not present when Pompe disease begins in adulthood. Therefore, these findings suggest that adult Pompe disease should not be taken into consideration in the differential diagnostic of structural or functional cardiac disorders.
Czech name
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Czech description
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Classification
Type
J<sub>x</sub> - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)
CEP classification
FA - Cardiovascular diseases including cardio-surgery
OECD FORD branch
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Result continuities
Project
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Continuities
N - Vyzkumna aktivita podporovana z neverejnych zdroju
Others
Publication year
2015
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
International journal of cardiovascular imaging
ISSN
1569-5794
e-ISSN
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Volume of the periodical
31
Issue of the periodical within the volume
5
Country of publishing house
US - UNITED STATES
Number of pages
10
Pages from-to
947-956
UT code for WoS article
000355151300007
EID of the result in the Scopus database
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