Faculty of Graduate Studies, Department of Medical Genetics and Pediatrics, University of Calgary Cumming School of Medicine, Alberta Children's Hospital Research Institute, Calgary, Alberta, Canada.
Departments of Cardiac Sciences and Medicine, Cumming School of Medicine, Calgary, Alberta, Canada.
Can J Cardiol. 2018 Aug;34(8):1041-1047. doi: 10.1016/j.cjca.2018.03.011. Epub 2018 Mar 29.
Cardiac hypertrophy in Fabry disease can be assessed using the left ventricular mass index (LVMI) with either echocardiography (LVMI-ECHO) or magnetic resonance imaging (LVMI-CMR).
A retrospective case series of patients with Fabry disease in Alberta involved a cross-sectional analysis of 32 patients and a longitudinal analysis of 14 of these patients with at least 4 serial CMR measurements.
The cross-sectional analysis showed the mean LVMI-ECHO was 97.8 ± 26.0 g/m, which was higher compared with LVMI-CMR at 81.1 ± 26.9 g/m with a mean bias of 16.7 g/m (P < 0.001). In the longitudinal analysis, LVMI-ECHO was higher, with an estimated marginal mean of 96.21 ± 6.13 (mean ± standard error of the mean [SEM]) compared with 71.18 ± 5.99 for LVMI-CMR (P < 0.01; generalized estimating equations). There was an association between an increase in LVMI-CMR over time with the presence of cardiac fibrosis, and patients treated with enzyme replacement therapy (ERT) had slower increases than those without therapy. LVMI-ECHO failed to detect these associations owing to the higher variability and tendency to overestimate the LVMI.
We propose the preferred method for measuring LVMI is CMR in patients with Fabry disease.
法布瑞氏病患者的心脏肥大可以通过超声心动图（LVMI-ECHO）或磁共振成像（LVMI-CMR）的左心室质量指数（LVMI）来评估。
艾伯塔省法布瑞氏病患者的回顾性病例系列研究涉及 32 例患者的横断面分析和其中 14 例患者的纵向分析，这些患者至少有 4 次连续 CMR 测量。
横断面分析显示，LVMI-ECHO 的平均值为 97.8 ± 26.0 g/m，高于 LVMI-CMR 的 81.1 ± 26.9 g/m，平均偏差为 16.7 g/m（P < 0.001）。在纵向分析中，LVMI-ECHO 更高，估计边缘均值为 96.21 ± 6.13（平均值 ± 均数的标准误差 [SEM]），而 LVMI-CMR 为 71.18 ± 5.99（P < 0.01；广义估计方程）。LVMI-CMR 随时间的增加与心脏纤维化的存在有关，接受酶替代治疗（ERT）的患者比未接受治疗的患者增加速度较慢。LVMI-ECHO 由于其较高的变异性和高估 LVMI 的趋势，未能检测到这些关联。
我们建议在法布瑞氏病患者中，首选测量 LVMI 的方法是 CMR。
