Comparison of echocardiographic parameters in Fabry cardiomyopathy and light-chain cardiac amyloidosis
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11110%2F18%3A10382967" target="_blank" >RIV/00216208:11110/18:10382967 - isvavai.cz</a>
Alternative codes found
RIV/00064165:_____/18:10382967
Result on the web
<a href="https://doi.org/10.1111/echo.14144" target="_blank" >https://doi.org/10.1111/echo.14144</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1111/echo.14144" target="_blank" >10.1111/echo.14144</a>
Alternative languages
Result language
angličtina
Original language name
Comparison of echocardiographic parameters in Fabry cardiomyopathy and light-chain cardiac amyloidosis
Original language description
Background: Fabry cardiomyopathy (FC) and light-chain amyloid cardiomyopathy (AL) present with concentric left ventricular (LV) hypertrophy/remodeling and diastolic rather than systolic dysfunction. Direct comparisons are difficult due to rarity and confounded by variability of LV thickness. Aims: To compare LV diastolic and systolic properties between patients with FC and AL in a cohort matched for interventricular septal thickness (IVS). Methods: A two-center echocardiographic analysis was performed, comprising 118 patients with IVS >=12 mm (FC and AL 59 patients each) matched by IVS. Results: Fabry cardiomyopathy patients had larger LV end-diastolic diameter (47.7 [44.0-50.9] vs 45.0 [41.5-49.0] mm, P = 0.002), better LV ejection fraction (EF 68.7 [63.4-74.0] vs 63.0 [54.0-70.0]%, P = 0.001) and midwall fractional shortening (midFS 14.8 [13.0-16.1] vs 12.1 [8.9-15.0]%, P = 0.006). LV EF <40% was rare in both (2% vs 7%, P = 0.17). AL patients expressed higher LV diastolic dysfunction grade (III in 26% vs 4%, II in 21% vs 12% and I in 54% vs 84%, P = 0.004), with higher E/e' ratio (13.6 [10.2-18.8] vs 9.8 [7.5-12.3], P < 0.0001). Average E/e' ratio and midFS were significantly associated with NYHA severity in both groups (P < 0.05 for all). Conclusions: Matched AL patients had worse LV diastolic function than FC, driven by E/e'. Significant LV systolic dysfunction was rare overall. MidFS and E/e' were associated with heart failure severity in both groups.
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
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Continuities
I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace
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
Echocardiography
ISSN
0742-2822
e-ISSN
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Volume of the periodical
35
Issue of the periodical within the volume
11
Country of publishing house
US - UNITED STATES
Number of pages
9
Pages from-to
1755-1763
UT code for WoS article
000449697000007
EID of the result in the Scopus database
2-s2.0-85053733610