Quantitative assessment of left ventricular longitudinal function and myocardial deformation in Duchenne muscular dystrophy patients

PANOVSKÝ, Roman, Martin PEŠL, Jan MÁCHAL, Tomáš HOLEČEK, Věra FEITOVÁ, Lenka JUŘÍKOVÁ, Lucia MASÁROVÁ, Eva PEŠLOVÁ, Lukáš OPATŘIL, Mary Luz MOJICA-PISCIOTTI and Vladimír KINCL. Quantitative assessment of left ventricular longitudinal function and myocardial deformation in Duchenne muscular dystrophy patients. Orphanet Journal of Rare Diseases. London: BMC, 2021, vol. 16, No 1, p. 1-9. ISSN 1750-1172. Available from: https://dx.doi.org/10.1186/s13023-021-01704-9.

Basic information

Original name

Quantitative assessment of left ventricular longitudinal function and myocardial deformation in Duchenne muscular dystrophy patients

Authors

PANOVSKÝ, Roman (203 Czech Republic, guarantor, belonging to the institution), Martin PEŠL (203 Czech Republic, belonging to the institution), Jan MÁCHAL (203 Czech Republic, belonging to the institution), Tomáš HOLEČEK (203 Czech Republic), Věra FEITOVÁ (203 Czech Republic), Lenka JUŘÍKOVÁ (203 Czech Republic), Lucia MASÁROVÁ (703 Slovakia, belonging to the institution), Eva PEŠLOVÁ (203 Czech Republic, belonging to the institution), Lukáš OPATŘIL (203 Czech Republic, belonging to the institution), Mary Luz MOJICA-PISCIOTTI and Vladimír KINCL (203 Czech Republic, belonging to the institution)

Edition

Orphanet Journal of Rare Diseases, London, BMC, 2021, 1750-1172

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Other information

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30101 Human genetics

Country of publisher

United Kingdom of Great Britain and Northern Ireland

Confidentiality degree

není předmětem státního či obchodního tajemství

References:

URL

Impact factor

Impact factor: 4.303

RIV identification code

RIV/00216224:14110/21:00121084

Organization unit

Faculty of Medicine

DOI

http://dx.doi.org/10.1186/s13023-021-01704-9

UT WoS

000616474600005

Keywords in English

Cardiac magnetic resonance; Duchenne muscular dystrophy; Feature tracking; Strain analysis

Tags

14110115, 14110127, 14110513, 14110518, rivok

Tags

International impact, Reviewed

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Abstract

V originále

BackgroundDuchenne muscular dystrophy (DMD) manifests in males mainly by skeletal muscle impairment, but also by cardiac dysfunction. The assessment of the early phases of cardiac involvement using echocardiography is often very difficult to perform in these patients. The aim of the study was to use cardiac magnetic resonance (CMR) strain analysis and mitral annular plane systolic excursion (MAPSE) in the detection of early left ventricular (LV) dysfunction in DMD patients. Methods and resultsIn total, 51 male DMD patients and 18 matched controls were examined by CMR. MAPSE measurement and functional analysis using feature tracking (FT) were performed. Three groups of patients were evaluated: A/ patients with LGE and LV EF<50% (n=8), B/ patients with LGE and LVEF50% (n=13), and C/ patients without LGE and LVEF >= 50% (n=30). MAPSE and global LV strains of the 3 DMD groups were compared to controls (n=18). Groups A and B had significantly reduced values of MAPSE, global longitudinal strain (GLS), global circumferential strain (GCS), and global radial strain (GRS) in comparison to controls (p<0.05). The values of MAPSE (11.61.9 v 13.72.7 mm) and GCS (- 26.2 +/- 4.2 v - 30.0 +/- 5.1%) were significantly reduced in group C compared to the controls (p<0.05).Conclusion DMD patients had decreased LV systolic function measured by MAPSE and global LV strain even in the case of normal LV EF and the absence of LGE. FT and MAPSE measurement provide sensitive assessment of early cardiac involvement in DMD patients.