Decreased Global Strains of LV in Asymptomatic Female Duchenne Muscular Dystrophy Gene Carriers Using CMR-FT

Kód výsledku v IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00159816%3A_____%2F21%3A00075120" target="_blank" >RIV/00159816:_____/21:00075120 - isvavai.cz</a>

Nalezeny alternativní kódy

RIV/00216224:14110/21:00120829

Výsledek na webu

<a href="https://www.sciencedirect.com/science/article/abs/pii/S1936878X20308895?via%3Dihub#" target="_blank" >https://www.sciencedirect.com/science/article/abs/pii/S1936878X20308895?via%3Dihub#</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1016/j.jcmg.2020.09.016" target="_blank" >10.1016/j.jcmg.2020.09.016</a>

Jazyk výsledku

angličtina

Název v původním jazyce

Decreased Global Strains of LV in Asymptomatic Female Duchenne Muscular Dystrophy Gene Carriers Using CMR-FT

Popis výsledku v původním jazyce

Duchenne muscular dystrophy (DMD) is the most common X-linked muscular dystrophy. Male patients can have DMD, whereas female patients can be asymptomatic carriers of DMD (aDMD-FC). They can develop cardiomyopathy, probably due to myocardial fibrosis. Early detection of functional myocardial dysfunction is critical for their prognosis. Using CMR-FT, this prospective study aimed to assess aDMD carriers&apos; left ventricular (LV) systolic function. We enrolled 37 aDMD and 20 healthy controls. We measured their global longitudinal strain (GLS), global circumferential strain (GCS), global radial strain (GRS), end-systolic (ESV), and end-diastolic (EDV) LV volumes and ejection fraction (EF) LV. aDMD and controls did not significantly differ in basic demographic data (age: 39.5 +- 9.1 vs 39.1 +- 10.7 years). There were found statistically significant differences in EF LV (56 +- 5 vs. 60 +- 3%), and all strain parameters (GLS (-19.4 +- 2.4 vs. -22.3 +- 2.2%), GCS (26.8 +- 3.3 vs. 29.5 +- 2.0%) and GRS (59.8 +- 12.9 vs. 70.9 +- 14.8%), all p&lt;0.05). aDMD carriers had significantly lower values of all strains than controls using CMR-FT, although they showed preserved EF LV. CMR-FT could benefit the early diagnosis of developing heart disease in asymptomatic patients with a DMD genetic predisposition.

Název v anglickém jazyce

Decreased Global Strains of LV in Asymptomatic Female Duchenne Muscular Dystrophy Gene Carriers Using CMR-FT

Popis výsledku anglicky

Duchenne muscular dystrophy (DMD) is the most common X-linked muscular dystrophy. Male patients can have DMD, whereas female patients can be asymptomatic carriers of DMD (aDMD-FC). They can develop cardiomyopathy, probably due to myocardial fibrosis. Early detection of functional myocardial dysfunction is critical for their prognosis. Using CMR-FT, this prospective study aimed to assess aDMD carriers&apos; left ventricular (LV) systolic function. We enrolled 37 aDMD and 20 healthy controls. We measured their global longitudinal strain (GLS), global circumferential strain (GCS), global radial strain (GRS), end-systolic (ESV), and end-diastolic (EDV) LV volumes and ejection fraction (EF) LV. aDMD and controls did not significantly differ in basic demographic data (age: 39.5 +- 9.1 vs 39.1 +- 10.7 years). There were found statistically significant differences in EF LV (56 +- 5 vs. 60 +- 3%), and all strain parameters (GLS (-19.4 +- 2.4 vs. -22.3 +- 2.2%), GCS (26.8 +- 3.3 vs. 29.5 +- 2.0%) and GRS (59.8 +- 12.9 vs. 70.9 +- 14.8%), all p&lt;0.05). aDMD carriers had significantly lower values of all strains than controls using CMR-FT, although they showed preserved EF LV. CMR-FT could benefit the early diagnosis of developing heart disease in asymptomatic patients with a DMD genetic predisposition.

Druh

J_imp - Článek v periodiku v databázi Web of Science

CEP obor

—

OECD FORD obor

30201 - Cardiac and Cardiovascular systems

Projekt

<a href="/cs/project/LQ1605" target="_blank" >LQ1605: Translační medicína</a><br>

Návaznosti

P - Projekt vyzkumu a vyvoje financovany z verejnych zdroju (s odkazem do CEP)

Rok uplatnění

2021

Kód důvěrnosti údajů

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

Název periodika

JACC-Cardiovascular Imaging

ISSN

1936-878X

e-ISSN

—

Svazek periodika

14

Číslo periodika v rámci svazku

5

Stát vydavatele periodika

US - Spojené státy americké

Počet stran výsledku

3

Strana od-do

1070-1072

Kód UT WoS článku

000647194600023

EID výsledku v databázi Scopus

—