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

R. Panovský, M. Pešl, J. Máchal, T. Holeček, V. Feitová, L. Juříková, L. Masárová, E. Pešlová, L. Opatřil, ML. Mojica-Pisciotti, V. Kincl

. 2021 ; 16 (1) : 57. [pub] 20210130

Language English Country Great Britain

Document type Journal Article, Research Support, Non-U.S. Gov't

Persistent link   https://www.medvik.cz/link/bmc21019395

BACKGROUND: Duchenne 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 RESULTS: In 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 LVEF ≥ 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 < 0.05). The values of MAPSE (11.6 ± 1.9 v 13.7 ± 2.7 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.

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$a Panovský, Roman $u International Clinical Research Center, St. Anne's Faculty Hospital, Brno, Czech Republic. panovsky@fnusa.cz $u 1St Department of Internal Medicine/Cardioangiology, St. Anne's Faculty Hospital, Faculty of Medicine, Masaryk University Brno, Brno, Czech Republic. panovsky@fnusa.cz
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$a Quantitative assessment of left ventricular longitudinal function and myocardial deformation in Duchenne muscular dystrophy patients / $c R. Panovský, M. Pešl, J. Máchal, T. Holeček, V. Feitová, L. Juříková, L. Masárová, E. Pešlová, L. Opatřil, ML. Mojica-Pisciotti, V. Kincl
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$a BACKGROUND: Duchenne 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 RESULTS: In 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 LVEF ≥ 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 < 0.05). The values of MAPSE (11.6 ± 1.9 v 13.7 ± 2.7 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.
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$a Pešl, Martin $u International Clinical Research Center, St. Anne's Faculty Hospital, Brno, Czech Republic $u 1St Department of Internal Medicine/Cardioangiology, St. Anne's Faculty Hospital, Faculty of Medicine, Masaryk University Brno, Brno, Czech Republic $u Department of Biology, Faculty of Medicine, Masaryk University, Brno, Czech Republic
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$a Máchal, Jan $u International Clinical Research Center, St. Anne's Faculty Hospital, Brno, Czech Republic $u Department of Pathophysiology, Faculty of Medicine, Masaryk University Brno, Brno, Czech Republic
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$a Holeček, Tomáš $u International Clinical Research Center, St. Anne's Faculty Hospital, Brno, Czech Republic $u Department of Medical Imaging, St. Anne's Faculty Hospital, Brno, Brno, Czech Republic
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$a Juříková, Lenka $u Department of Pediatric Neurology, University Hospital Brno, Brno, Czech Republic
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$a Masárová, Lucia $u International Clinical Research Center, St. Anne's Faculty Hospital, Brno, Czech Republic $u 1St Department of Internal Medicine/Cardioangiology, St. Anne's Faculty Hospital, Faculty of Medicine, Masaryk University Brno, Brno, Czech Republic
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$a Pešlová, Eva $u First Department of Neurology, St. Anne's Faculty Hospital, Faculty of Medicine, Masaryk University Brno, Brno, Czech Republic
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$a Opatřil, Lukáš $u International Clinical Research Center, St. Anne's Faculty Hospital, Brno, Czech Republic $u 1St Department of Internal Medicine/Cardioangiology, St. Anne's Faculty Hospital, Faculty of Medicine, Masaryk University Brno, Brno, Czech Republic
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$a Mojica-Pisciotti, Mary Luz $u International Clinical Research Center, St. Anne's Faculty Hospital, Brno, Czech Republic
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$a Kincl, Vladimír $u International Clinical Research Center, St. Anne's Faculty Hospital, Brno, Czech Republic $u 1St Department of Internal Medicine/Cardioangiology, St. Anne's Faculty Hospital, Faculty of Medicine, Masaryk University Brno, Brno, Czech Republic
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