{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,29]],"date-time":"2026-05-29T23:59:10Z","timestamp":1780099150986,"version":"3.54.0"},"reference-count":23,"publisher":"BMJ","issue":"2","license":[{"start":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T00:00:00Z","timestamp":1761955200000},"content-version":"unspecified","delay-in-days":123,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100004339","name":"Sanofi","doi-asserted-by":"publisher","award":["NCT03199001"],"award-info":[{"award-number":["NCT03199001"]}],"id":[{"id":"10.13039\/100004339","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["Open Heart"],"accepted":{"date-parts":[[2025,10,2]]},"published-print":{"date-parts":[[2025,7]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Fabry disease (FD) is an X linked lysosomal disorder with ventricular myocardial involvement that drives morbidity and mortality. Early diagnosis of cardiac involvement can be difficult. This study explored whether abnormal left atrial (LA) strain by cardiovascular magnetic resonance (CMR) may be an early sign of ventricular involvement in FD.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A multicentre, multinational cohort of patients with FD was assembled with images centralised for core lab analysis. Adult patients with gene-positive FD and healthy volunteers (HV) underwent CMR. LA strain analyses included manually contouring the left atrium in end-diastole and end-systole to calculate LA volumes and ejection fraction, then semiautomatic analysis for LA reservoir strain.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      There were n=214\u2009patients with FD (mean age 45\u00b115 years, 39% males) and n=76\u2009HV (49\u00b115 years, 53% males). CMR results in FD: left ventricular ejection fraction 73% (IQR=9), left ventricular mass index (LVMi) 89\u00b139\u2009g\/m\n                      <jats:sup>2<\/jats:sup>\n                      , 99 (46%) had left ventricular hypertrophy (LVH), 36% had late gadolinium enhancement. In FD, LA strain correlated with LVMi (r=\u22120.52, p&lt;0.01), left ventricular (LV) global longitudinal strain (GLS) (r=\u22120.61, p&lt;0.01) and native myocardial T1 (r=0.34, p&lt;0.01). FD had abnormal LA strain in overt disease (LVH positive) compared with HV (p&lt;0.01). LVH-negative FD did not differ in LA strain compared with HV (p&gt;0.5). FD with low T1+LVH negative did not differ in LA strain compared with normal T1\/LVH-negative FD or HV (p&gt;0.3).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>LA strain is abnormal in FD with LVH (overt disease) and correlates with LVMi, native T1 and GLS. LA strain is normal in FD with early disease (LVH negative+low\u2009T1) and normal in FD with no myocardial disease (LVH negative+normal\u2009T1). These findings indicate that LA strain is a consequence of abnormal LV mechanics such as LVH and abnormal GLS, rather than isolated myocardial sphingolipid deposition.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Trial registration number<\/jats:title>\n                    <jats:p>\n                      <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" ext-link-type=\"clintrialgov\" xlink:href=\"NCT03199001\">NCT03199001<\/jats:ext-link>\n                      .\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1136\/openhrt-2025-003385","type":"journal-article","created":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T08:20:11Z","timestamp":1761985211000},"page":"e003385","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":1,"title":["Left atrial strain tracks abnormal ventricular mechanics in Fabry disease"],"prefix":"10.1136","volume":"12","clinical-trial-number":[{"clinical-trial-number":"nct03199001","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct03199001","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct03199001","registry":"10.18810\/clinical-trials-gov"}],"author":[{"ORCID":"https:\/\/orcid.org\/0009-0003-6277-3199","authenticated-orcid":false,"given":"Chosita","family":"Cheepvasarach","sequence":"first","affiliation":[{"name":"Royal North Shore Hospital, St Leonards, New South Wales, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Gribble","sequence":"additional","affiliation":[{"name":"The University of Sydney School of Medicine, Sydney, New South Wales, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Ugander","sequence":"additional","affiliation":[{"name":"The University of Sydney School of Medicine, Sydney, New South Wales, Australia"},{"name":"Department of Clinical Physiology, Karolinska Institutet, Stockholm, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ravi","family":"Vijapurapu","sequence":"additional","affiliation":[{"name":"Cardiology, Queen Elizabeth Hospital Birmingham, Birmingham, UK"},{"name":"Institute of Cardiovascular Sciences, University of Birmingham, Birmingham, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabrina","family":"Nordin","sequence":"additional","affiliation":[{"name":"Cardiology, Barts Health NHS Trust, London, UK"},{"name":"School of Cardiovascular & Metabolic Health, University of Glasgow, Glasgow, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joao","family":"Augusto","sequence":"additional","affiliation":[{"name":"Hospital Prof Doutor Fernando Fonseca, Amadora, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5687-2535","authenticated-orcid":false,"given":"Richard Paul","family":"Steeds","sequence":"additional","affiliation":[{"name":"Cardiology, Queen Elizabeth Hospital Birmingham, Birmingham, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michel","family":"Tchan","sequence":"additional","affiliation":[{"name":"The University of Sydney Faculty of Medicine and Health, Sydney, New South Wales, Australia"},{"name":"Westmead Hospital, Westmead, NSW, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James C","family":"Moon","sequence":"additional","affiliation":[{"name":"Cardiovascular Sciences, Barts Health NHS Trust, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Faraz","family":"Pathan","sequence":"additional","affiliation":[{"name":"The University of Sydney, Sydney, New South Wales, Australia"},{"name":"Department 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