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

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...

Ausführliche Beschreibung

Gespeichert in:
Bibliographische Detailangaben
Veröffentlicht in:Orphanet journal of rare diseases 2021-01, Vol.16 (1), p.57-57, Article 57
Hauptverfasser: Panovsky, Roman, Pesl, Martin, Machal, Jan, Holecek, Tomas, Feitova, Vera, Jurikova, Lenka, Masarova, Lucia, Peslova, Eva, Opatril, Lukas, Mojica-Pisciotti, Mary Luz, Kincl, Vladimir
Format: Artikel
Sprache:eng
Schlagworte:
Online-Zugang:Volltext
Tags: Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
Beschreibung
Zusammenfassung: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=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
ISSN:1750-1172
1750-1172
DOI:10.1186/s13023-021-01704-9