Structural and functional cardiac analyses using modern and sensitive myocardial techniques in adult Pompe disease

Kód výsledku v 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>

Výsledek na webu

<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>

Jazyk výsledku

angličtina

Název v původním jazyce

Structural and functional cardiac analyses using modern and sensitive myocardial techniques in adult Pompe disease

Popis výsledku v původním jazyce

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.

Název v anglickém jazyce

Structural and functional cardiac analyses using modern and sensitive myocardial techniques in adult Pompe disease

Popis výsledku anglicky

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.

Druh

J_x - Nezařazeno - Článek v odborném periodiku (Jimp, Jsc a Jost)

CEP obor

FA - Kardiovaskulární nemoci včetně kardiochirurgie

OECD FORD obor

—

Projekt

—

Návaznosti

N - Vyzkumna aktivita podporovana z neverejnych zdroju

Rok uplatnění

2015

Kód důvěrnosti údajů

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Název periodika

International journal of cardiovascular imaging

ISSN

1569-5794

e-ISSN

—

Svazek periodika

31

Číslo periodika v rámci svazku

5

Stát vydavatele periodika

US - Spojené státy americké

Počet stran výsledku

10

Strana od-do

947-956

Kód UT WoS článku

000355151300007

EID výsledku v databázi Scopus

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