{"title":"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》。","authors":"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","doi":"10.1148/ryct.230154","DOIUrl":null,"url":null,"abstract":"<p><p>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: <i>(a)</i> left ventricular wall mass (LVM), measured in grams; <i>(b)</i> LVM indexed to body mass index, measured in grams per meters squared; <i>(c)</i> maximum left ventricular wall thickness (MLVWT), measured in millimeters; <i>(d)</i> late gadolinium enhancement (LGE) extent, measured in percentage of LVM; and <i>(e)</i> 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, <i>I<sup>2</sup></i> = 88%) and LVM indexed (mean difference, -1 g/m<sup>2</sup> [95% CI: -6, 3]; four studies, 290 patients, <i>I<sup>2</sup></i> = 81%). The following measures minimally decreased: LVM (mean difference, -18 g [95% CI: -33, -3]; seven studies, 107 patients, <i>I<sup>2</sup></i> = 96%) and MLVWT (mean difference, -1 mm [95% CI: -2, -0.02]; six studies, 151 patients, <i>I<sup>2</sup></i> = 90%). LGE extent increased (mean difference, 1% [95% CI: 1, 1]; three studies, 114 patients, <i>I<sup>2</sup></i> = 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. <b>Keywords:</b> Fabry Disease, Enzyme Replacement Therapy (ERT), Cardiac MRI, Late Gadolinium Enhancement (LGE) <i>Supplemental material is available for this article.</i> © RSNA, 2024.</p>","PeriodicalId":21168,"journal":{"name":"Radiology. Cardiothoracic imaging","volume":null,"pages":null},"PeriodicalIF":3.8000,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11211942/pdf/","citationCount":"0","resultStr":"{\"title\":\"Effects of Enzyme Replacement Therapy on Cardiac MRI Findings in Fabry Disease: A Systematic Review and Meta-Analysis.\",\"authors\":\"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\",\"doi\":\"10.1148/ryct.230154\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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: <i>(a)</i> left ventricular wall mass (LVM), measured in grams; <i>(b)</i> LVM indexed to body mass index, measured in grams per meters squared; <i>(c)</i> maximum left ventricular wall thickness (MLVWT), measured in millimeters; <i>(d)</i> late gadolinium enhancement (LGE) extent, measured in percentage of LVM; and <i>(e)</i> 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, <i>I<sup>2</sup></i> = 88%) and LVM indexed (mean difference, -1 g/m<sup>2</sup> [95% CI: -6, 3]; four studies, 290 patients, <i>I<sup>2</sup></i> = 81%). The following measures minimally decreased: LVM (mean difference, -18 g [95% CI: -33, -3]; seven studies, 107 patients, <i>I<sup>2</sup></i> = 96%) and MLVWT (mean difference, -1 mm [95% CI: -2, -0.02]; six studies, 151 patients, <i>I<sup>2</sup></i> = 90%). LGE extent increased (mean difference, 1% [95% CI: 1, 1]; three studies, 114 patients, <i>I<sup>2</sup></i> = 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. <b>Keywords:</b> Fabry Disease, Enzyme Replacement Therapy (ERT), Cardiac MRI, Late Gadolinium Enhancement (LGE) <i>Supplemental material is available for this article.</i> © RSNA, 2024.</p>\",\"PeriodicalId\":21168,\"journal\":{\"name\":\"Radiology. Cardiothoracic imaging\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":3.8000,\"publicationDate\":\"2024-06-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11211942/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Radiology. Cardiothoracic imaging\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1148/ryct.230154\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Radiology. Cardiothoracic imaging","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1148/ryct.230154","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING","Score":null,"Total":0}
引用次数: 0