Alì Marco, Monti Caterina Beatrice, Melazzini Luca, Cardani Rosanna, Fossati Barbara, Cavalli Michele, Chow Kelvin, Secchi Francesco, Meola Giovanni, Sardanelli Francesco
Unit of Diagnostic Imaging and Stereotactic Radiosurgery, C.D.I. Centro Diagnostico Italiano S.p.A., Milan, Italy.
Unit of Radiology, IRCCS Policlinico San Donato, San Donato Milanese, Italy.
Front Neurol. 2020 Mar 19;11:192. doi: 10.3389/fneur.2020.00192. eCollection 2020.
To evaluate myocardial strain and extracellular volume in myotonic dystrophy type 1 (DM1) patients as potential imaging biomarkers of subclinical cardiac pathology. 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 -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. Global cardiac strain (median -19.1%; IQR -20.5%, -16.5%) in DM1 patients was lower ( = 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 ( = 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, = 0.016) and with both global (ρ = 0.733 = 0.025) and septal extracellular volume (ρ = 0.767, = 0.016). 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.
评估1型强直性肌营养不良（DM1）患者的心肌应变和细胞外容积，作为亚临床心脏病变的潜在影像学生物标志物。我们回顾性分析了9例在本中心接受心脏磁共振检查且无明显心脏病的DM1患者。患者在年龄和性别上与健康对照匹配。采用曼-惠特尼U检验比较两组间的心脏应变。采用t检验比较DM1患者与健康受试者的细胞外容积。使用斯皮尔曼相关系数ρ研究影像参数之间的关联。DM1患者的整体心脏应变（中位数-19.1%；四分位间距-20.5%，-16.5%）低于对照组（中位数-21.7%；四分位间距-22.7%，-21.3%）（P = 0.011）。DM1患者的整体细胞外容积（中位数32.3%；四分位间距29.3%，36.8%）显著高于文献报道的健康受试者（中位数25.6%；四分位间距19.9%，31.9%）（P = 0.008）。整体心脏应变与室间隔应变（ρ = 0.767，P = 0.016）以及整体（ρ = 0.733，P = 0.025）和室间隔细胞外容积（ρ = 0.767，P = 0.016）均呈强正相关。心脏细胞外容积增加和应变降低是DM1早期心脏病变的迹象。治疗DM1的医生可考虑将心脏磁共振作为一种筛查工具，以识别该疾病早期的心脏受累情况。
