Rare Disease: Cardiac Risk Assessment With MRI in Patients With Myotonic Dystrophy Type 1

Frontiers in Neurology

卷:11

Francesco Sardanelli¹  Francesco Secchi¹  Giovanni Meola¹  Barbara Fossati²  Rosanna Cardani³  Caterina Beatrice Monti⁴  Luca Melazzini⁴  Michele Cavalli⁵  Kelvin Chow⁶  Marco Alì⁹ 

[1] Department of Biomedical Sciences for Health, University of Milan, San Donato Milanese, Italy;

[2] Department of Neurorehabilitation Sciences, Casa di Cura del Policlinico, Milan, Italy;

[3] Laboratory of Muscle Histopathology and Molecular Biology, IRCCS Policlinico San Donato, San Donato Milanese, Italy;

[4] PhD Course in Integrative Biomedical Research, Department of Biomedical Sciences for Health, University of Milan, Milan, Italy;

[5] Postgraduate School in Neurology, University of Milan, Milan, Italy;

[6] Siemens Medical Solutions USA Inc., Chicago, IL, United States;

[7] Unit of Diagnostic Imaging and Stereotactic Radiosurgery, C.D.I. Centro Diagnostico Italiano S.p.A., Milan, Italy;

[8] Unit of Neurology, IRCCS Policlinico San Donato, San Donato Milanese, Italy;

[9] Unit of Radiology, IRCCS Policlinico San Donato, San Donato Milanese, Italy;

关键词: myotonic dystrophy;    cardiac magnetic resonance;    myocardial strain imaging;    extracellular volume;    cardiac fibrosis;   

DOI  :  10.3389/fneur.2020.00192

来源: DOAJ

【 摘 要 】

Introduction: To evaluate myocardial strain and extracellular volume in myotonic dystrophy type 1 (DM1) patients as potential imaging biomarkers of subclinical cardiac pathology.Materials and methods: We retrospectively analyzed 9 DM1 patients without apparent cardiac disease who had undergone cardiac magnetic resonance at our center. Patients were age- and sex-matched with healthy controls. The Mann-Whitney U test was used to compare cardiac strain between the two groups. The t-test was used to compare the extracellular volume obtained in DM1 patients with that in healthy subject. Spearman's ρ was used for studying the associations among imaging parameters.Results: Global cardiac strain (median −19.1%; IQR −20.5%, −16.5%) in DM1 patients was lower (p = 0.011) than that in controls (median−21.7%; IQR−22.7%,-21.3%). Global extracellular volume in DM1 patients (median 32.3%; IQR 29.3%,36.8%) was significantly (p = 0.008) higher than that reported in literature in healthy subjects (median 25.6%; IQR 19.9%,31.9%). Global cardiac strain showed a strong, positive correlation with septal strain (ρ = 0.767, p = 0.016) and with both global (ρ = 0.733 p = 0.025) and septal extracellular volume (ρ = 0.767, p = 0.016).Discussion: The increase in cardiac extracellular volume and decrease in strain are signs of early cardiac pathology in DM1. Physicians dealing with DM1 may take into consideration cardiac magnetic resonance as a screening tool to identify early cardiac involvement in this condition.

【 授权许可】

Unknown