{"title":"Imaging predictors of adverse prognosis in Fabry disease cardiomyopathy: A systematic review and meta-analysis.","authors":"Kamil Stankowski, Stefano Figliozzi, Thanakorn Rojanathagoon, Dimitrios Bampatsias, Dimitrios Klettas, Lorenzo Monti, Renato Bragato, Pier-Giorgio Masci, Marco Francone, Gianluigi Condorelli, Massimo Imbriaco, Maurizio Pieroni, Antonia Camporeale, Georgios Georgiopoulos","doi":"10.1111/eci.14388","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Cardiac involvement represents the main cause of death in patients with Fabry disease (FD). Echocardiography and cardiovascular magnetic resonance (CMR) have an established diagnostic role, but their prognostic value remains unresolved. This systematic review and meta-analysis sought to assess the prognostic implications of imaging parameters in FD.</p><p><strong>Methods: </strong>PubMed, ClinicalTrials.gov, Embase, Cochrane Library and Web of Science databases were searched for studies from inception through 1 May 2024. Studies including FD patients undergoing baseline imaging assessment and clinical follow-up were selected. Pre-defined study outcomes were a cardiovascular endpoint and a composite clinical endpoint. The study protocol was registered in PROSPERO (ID CRD42022342394).</p><p><strong>Results: </strong>Fourteen studies, including 1713 FD patients (44.7% males), were selected. At pooled analysis, late gadolinium enhancement (hazard ratio [HR]: 4.45; 95% CI: 2.82-7.02), left atrium volume indexed (HR: 1.02 per mL/m<sup>2</sup>; 95% CI: 1.01-1.03), E/e' (HR: 1.14 per unit increase; 95% CI: 1.08-1.21), left ventricular (LV) mass indexed (HR: 1.01 per mg/m<sup>2</sup>; 95% CI: 1.00-1.02), maximum LV wall thickness (HR: 1.19 per mm, 95% CI: 1.04-1.36) and LV-global longitudinal strain (HR: 1.20 per unit increase; 95% CI: 1.16-1.25) were significantly associated with the cardiovascular endpoint, whereas T1-mapping and LV-ejection fraction were not. T1-mapping was associated with the composite endpoint (HR: 0.99 per msec increase; 95% CI: 0.98-1.00). Meta-regression analysis did not show any significant interaction between each of the potential effect modifiers.</p><p><strong>Conclusions: </strong>Several imaging parameters were significant predictors of adverse clinical outcomes in patients with FD. Late gadolinium enhancement showed the strongest association with adverse prognosis.</p>","PeriodicalId":12013,"journal":{"name":"European Journal of Clinical Investigation","volume":" ","pages":"e14388"},"PeriodicalIF":4.4000,"publicationDate":"2025-01-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"European Journal of Clinical Investigation","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1111/eci.14388","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"MEDICINE, GENERAL & INTERNAL","Score":null,"Total":0}
引用次数: 0
Abstract
Background: Cardiac involvement represents the main cause of death in patients with Fabry disease (FD). Echocardiography and cardiovascular magnetic resonance (CMR) have an established diagnostic role, but their prognostic value remains unresolved. This systematic review and meta-analysis sought to assess the prognostic implications of imaging parameters in FD.
Methods: PubMed, ClinicalTrials.gov, Embase, Cochrane Library and Web of Science databases were searched for studies from inception through 1 May 2024. Studies including FD patients undergoing baseline imaging assessment and clinical follow-up were selected. Pre-defined study outcomes were a cardiovascular endpoint and a composite clinical endpoint. The study protocol was registered in PROSPERO (ID CRD42022342394).
Results: Fourteen studies, including 1713 FD patients (44.7% males), were selected. At pooled analysis, late gadolinium enhancement (hazard ratio [HR]: 4.45; 95% CI: 2.82-7.02), left atrium volume indexed (HR: 1.02 per mL/m2; 95% CI: 1.01-1.03), E/e' (HR: 1.14 per unit increase; 95% CI: 1.08-1.21), left ventricular (LV) mass indexed (HR: 1.01 per mg/m2; 95% CI: 1.00-1.02), maximum LV wall thickness (HR: 1.19 per mm, 95% CI: 1.04-1.36) and LV-global longitudinal strain (HR: 1.20 per unit increase; 95% CI: 1.16-1.25) were significantly associated with the cardiovascular endpoint, whereas T1-mapping and LV-ejection fraction were not. T1-mapping was associated with the composite endpoint (HR: 0.99 per msec increase; 95% CI: 0.98-1.00). Meta-regression analysis did not show any significant interaction between each of the potential effect modifiers.
Conclusions: Several imaging parameters were significant predictors of adverse clinical outcomes in patients with FD. Late gadolinium enhancement showed the strongest association with adverse prognosis.
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EJCI considers any original contribution from the most sophisticated basic molecular sciences to applied clinical and translational research and evidence-based medicine across a broad range of subspecialties. The EJCI publishes reports of high-quality research that pertain to the genetic, molecular, cellular, or physiological basis of human biology and disease, as well as research that addresses prevalence, diagnosis, course, treatment, and prevention of disease. We are primarily interested in studies directly pertinent to humans, but submission of robust in vitro and animal work is also encouraged. Interdisciplinary work and research using innovative methods and combinations of laboratory, clinical, and epidemiological methodologies and techniques is of great interest to the journal. Several categories of manuscripts (for detailed description see below) are considered: editorials, original articles (also including randomized clinical trials, systematic reviews and meta-analyses), reviews (narrative reviews), opinion articles (including debates, perspectives and commentaries); and letters to the Editor.
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