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.
Rare Disease : Cardiac Risk Assessment With MRI in Patients With Myotonic Dystrophy Type 1 / M. Ali, C.B. Monti, L. Melazzini, R. Cardani, B. Fossati, M. Cavalli, K. Chow, F. Secchi, G. Meola, F. Sardanelli. - In: FRONTIERS IN NEUROLOGY. - ISSN 1664-2295. - 11(2020 Mar 19), pp. 192.1-192.7.
|Titolo:||Rare Disease : Cardiac Risk Assessment With MRI in Patients With Myotonic Dystrophy Type 1|
ALI', MARCO (Primo)
MELAZZINI, LUCA (Corresponding)
SARDANELLI, FRANCESCO (Ultimo)
|Parole Chiave:||cardiac fibrosis; cardiac magnetic resonance; extracellular volume; myocardial strain imaging; myotonic dystrophy|
|Settore Scientifico Disciplinare:||Settore MED/36 - Diagnostica per Immagini e Radioterapia|
|Data di pubblicazione:||19-mar-2020|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.3389/fneur.2020.00192|
|Appare nelle tipologie:||01 - Articolo su periodico|