{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T14:49:49Z","timestamp":1787064589055,"version":"build-2736575974"},"reference-count":50,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2025,1,22]],"date-time":"2025-01-22T00:00:00Z","timestamp":1737504000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Eur J Clin Investigation"],"published-print":{"date-parts":[[2025,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>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\u2010analysis sought to assess the prognostic implications of imaging parameters in FD.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      PubMed,\n                      <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/clinicaltrials.gov\">ClinicalTrials.gov<\/jats:ext-link>\n                      , 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\u2010up were selected. Pre\u2010defined study outcomes were a cardiovascular endpoint and a composite clinical endpoint. The study protocol was registered in PROSPERO (ID CRD42022342394).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      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\u20137.02), left atrium volume indexed (HR: 1.02 per mL\/m\n                      <jats:sup>2<\/jats:sup>\n                      ; 95% CI: 1.01\u20131.03), E\/e\u2032 (HR: 1.14 per unit increase; 95% CI: 1.08\u20131.21), left ventricular (LV) mass indexed (HR: 1.01 per mg\/m\n                      <jats:sup>2<\/jats:sup>\n                      ; 95% CI: 1.00\u20131.02), maximum LV wall thickness (HR: 1.19 per mm, 95% CI: 1.04\u20131.36) and LV\u2010global longitudinal strain (HR: 1.20 per unit increase; 95% CI: 1.16\u20131.25) were significantly associated with the cardiovascular endpoint, whereas T1\u2010mapping and LV\u2010ejection fraction were not. T1\u2010mapping was associated with the composite endpoint (HR: 0.99 per msec increase; 95% CI: 0.98\u20131.00). Meta\u2010regression analysis did not show any significant interaction between each of the potential effect modifiers.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Several imaging parameters were significant predictors of adverse clinical outcomes in patients with FD. Late gadolinium enhancement showed the strongest association with adverse prognosis.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/eci.14388","type":"journal-article","created":{"date-parts":[[2025,1,22]],"date-time":"2025-01-22T06:30:58Z","timestamp":1737527458000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Imaging predictors of adverse prognosis in Fabry disease cardiomyopathy: A systematic review and meta\u2010analysis"],"prefix":"10.1111","volume":"55","author":[{"given":"Kamil","family":"Stankowski","sequence":"first","affiliation":[{"name":"IRCCS Humanitas Research Hospital  Rozzano, Milan Italy"},{"name":"Department of Biomedical Sciences Humanitas University  Pieve Emanuele, Milan 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