O'Brien Ciara, Britton Ian, Karur Gauri R, Iwanochko Robert M, Morel Chantal F, Nguyen Elsie T, Thavendiranathan Paaladinesh, Woo Anna, Hanneman Kate
Toronto Joint Department of Medical Imaging, Toronto General Hospital, Peter Munk Cardiac Centre, University Health Network, University of Toronto, 585 University Ave, 1 PMB-298, Toronto, ON, Canada M5G 2N2 (C.O., G.R.K., E.T.N., P.T., K.H.); Division of Cardiology, Peter Munk Cardiac Centre, University Health Network, University of Toronto, Toronto, Canada (I.B., R.M.I., P.T., A.W.); and Fred A. Litwin Centre in Genetic Medicine, University Health Network & Mount Sinai Hospital, University of Toronto, Toronto, Canada (C.F.M.).
Radiol Cardiothorac Imaging. 2020 Jun 11;2(3):e190149. doi: 10.1148/ryct.2020190149. eCollection 2020 Jun.
To compare transthoracic echocardiography (TTE) and cardiac MRI measurements of left ventricular mass (LVM) and maximum wall thickness (MWT) in patients with Fabry disease and evaluate the clinical significance of discrepancies between modalities.
Seventy-eight patients with Fabry disease (mean age, 46 years ± 14 [standard deviation]; 63% female) who underwent TTE and cardiac MRI within a 6-month interval between 2008 and 2018 were included in this retrospective cohort study. The clinical significance of measurement discrepancies was evaluated with respect to diagnosis of left ventricular hypertrophy (LVH), eligibility for disease-specific therapy, and prognosis. Statistical analysis included paired-sample test, Cox proportional hazard models, Akaike information criterion (AIC), and intraclass correlation coefficients.
LVM indexed to body surface area (LVMI) and MWT were significantly higher at TTE compared with MRI (105 g/m ± 48 vs 78 g/m ± 36, < .001 and 14 mm ± 4 vs 13 mm ± 5, = .008, respectively). LVH classification was discordant between modalities in 23 patients (29%) ( < .001). Eligibility for disease-specific therapy based on MWT was discordant between modalities in 20 patients (26%) ( < .001). LVMI assessed with MRI was a better predictor of the combined endpoint compared with LVMI assessed with TTE (AIC, 127 vs 131). Interobserver agreement for LVMI and MWT was higher for MRI (intraclass correlation coefficient, 0.951 and 0.912, respectively) compared with TTE (intraclass correlation coefficient, 0.940 and 0.871; respectively).
TTE overestimates LVM and MWT and has lower reproducibility compared with cardiac MRI in Fabry disease. Measurement discrepancies between modalities are clinically significant with respect to diagnosis of LVH, prognosis, and treatment decisions.© RSNA, 2020.
比较法布里病患者经胸超声心动图（TTE）和心脏磁共振成像（MRI）对左心室质量（LVM）和最大壁厚（MWT）的测量结果，并评估不同检查方法测量结果差异的临床意义。
本回顾性队列研究纳入了2008年至2018年间在6个月内接受TTE和心脏MRI检查的78例法布里病患者（平均年龄46岁±14[标准差]；63%为女性）。从左心室肥厚（LVH）诊断、疾病特异性治疗的适用性和预后方面评估测量差异的临床意义。统计分析包括配对样本检验、Cox比例风险模型、赤池信息准则（AIC）和组内相关系数。
与MRI相比，TTE测得的体表面积指数左心室质量（LVMI）和MWT显著更高（分别为105 g/m²±48与78 g/m²±36，P<.001；14 mm±4与13 mm±5，P=.008）。23例患者（29%）的LVH分类在两种检查方法之间不一致（P<.001）。基于MWT的疾病特异性治疗适用性在20例患者（26%）中两种检查方法之间不一致（P<.001）。与TTE评估的LVMI相比，MRI评估的LVMI是联合终点更好的预测指标（AIC，127对131）。与TTE（组内相关系数分别为0.940和0.871）相比，MRI对LVMI和MWT的观察者间一致性更高（组内相关系数分别为(0.951)和(0.912)）。
在法布里病中，TTE高估了LVM和MWT，与心脏MRI相比再现性更低。不同检查方法之间的测量差异在LVH诊断、预后和治疗决策方面具有临床意义。©RSNA，2020。
