Effects of Enzyme Replacement Therapy on Cardiac MRI Findings in Fabry Disease: A Systematic Review and Meta-Analysis.

IF 3.8 Q1 RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING
Stefano Figliozzi, Eleni Kollia, Alexandros Simistiras, Antonia Camporeale, Kamil Stankowski, Pier Giorgio Masci, George Mavraganis, Massimo Lombardi, Gianluigi Condorelli, Marco Francone, Maurizio Pieroni, Georgios Georgiopoulos
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Abstract

Purpose To perform a systematic review and meta-analysis to assess the effect of enzyme replacement therapy on cardiac MRI parameters in patients with Fabry disease. Materials and Methods A systematic literature search was conducted from January 1, 2000, through January 1, 2024, in PubMed, ClinicalTrials.gov, Embase, and Cochrane Library databases. Study outcomes were changes in the following parameters: (a) left ventricular wall mass (LVM), measured in grams; (b) LVM indexed to body mass index, measured in grams per meters squared; (c) maximum left ventricular wall thickness (MLVWT), measured in millimeters; (d) late gadolinium enhancement (LGE) extent, measured in percentage of LVM; and (e) native T1 mapping, measured in milliseconds. A random-effects meta-analysis of the pooled mean differences between baseline and follow-up parameters was conducted. The study protocol was registered in PROSPERO (CRD42022336223). Results The final analysis included 11 studies of a total of 445 patients with Fabry disease (mean age ± SD, 41 years ± 11; 277 male, 168 female). Between baseline and follow-up cardiac MRI, the following did not change: T1 mapping (mean difference, 6 msec [95% CI: -2, 15]; two studies, 70 patients, I2 = 88%) and LVM indexed (mean difference, -1 g/m2 [95% CI: -6, 3]; four studies, 290 patients, I2 = 81%). The following measures minimally decreased: LVM (mean difference, -18 g [95% CI: -33, -3]; seven studies, 107 patients, I2 = 96%) and MLVWT (mean difference, -1 mm [95% CI: -2, -0.02]; six studies, 151 patients, I2 = 90%). LGE extent increased (mean difference, 1% [95% CI: 1, 1]; three studies, 114 patients, I2 = 85%). Conclusion In patients with Fabry disease, enzyme replacement therapy was associated with stabilization of LVM, MLVWT, and T1 mapping values, whereas LGE extent mildly increased. Keywords: Fabry Disease, Enzyme Replacement Therapy (ERT), Cardiac MRI, Late Gadolinium Enhancement (LGE) Supplemental material is available for this article. © RSNA, 2024.

酶替代疗法对法布里病心脏磁共振成像结果的影响:系统回顾与元分析》。
目的 进行系统性回顾和荟萃分析,评估酶替代疗法对法布里病患者心脏磁共振成像参数的影响。材料和方法 在 PubMed、ClinicalTrials.gov、Embase 和 Cochrane Library 数据库中对 2000 年 1 月 1 日至 2024 年 1 月 1 日期间的文献进行了系统性检索。研究结果为以下参数的变化:(a) 左心室壁质量(LVM),以克为单位;(b) 与体重指数相关的左心室壁质量,以克/米平方为单位;(c) 最大左心室壁厚度(MLVWT),以毫米为单位;(d) 迟发性钆增强(LGE)程度,以左心室壁质量的百分比为单位;(e) 本地 T1 映射,以毫秒为单位。对基线和随访参数之间的汇总平均差异进行了随机效应荟萃分析。研究方案已在 PROSPERO 注册(CRD42022336223)。结果 最终分析包括 11 项研究,共涉及 445 名法布里病患者(平均年龄 ± SD,41 岁 ± 11;277 名男性,168 名女性)。在基线和随访心脏磁共振成像之间,以下指标没有发生变化:T1 映像(平均差异为 6 毫秒 [95% CI:-2,15];两项研究,70 名患者,I2 = 88%)和 LVM 指数(平均差异为-1 克/平方米 [95% CI:-6,3];四项研究,290 名患者,I2 = 81%)。以下指标的降幅最小:LVM(平均差异:-18 g [95% CI:-33,-3];7 项研究,107 名患者,I2 = 96%)和 MLVWT(平均差异:-1 mm [95% CI:-2,-0.02];6 项研究,151 名患者,I2 = 90%)。LGE 范围增加(平均差异为 1%[95% CI:1,1];3 项研究,114 名患者,I2 = 85%)。结论 在法布里病患者中,酶替代治疗与 LVM、MLVWT 和 T1 映射值的稳定有关,而 LGE 范围轻度增加。关键词法布里病、酶替代疗法(ERT)、心脏磁共振成像、晚期钆增强(LGE 本文有补充材料。© RSNA, 2024.
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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